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Gild ML, Do K, Tsang VHM, Tacon LJ, Clifton-Bligh RJ, Robinson BG. Pheochromocytoma in MEN2. Recent Results Cancer Res 2025; 223:211-235. [PMID: 40102259 DOI: 10.1007/978-3-031-80396-3_8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/20/2025]
Abstract
Pheochromocytomas (PCs) are rare neuroendocrine tumors found in 20-50% of MEN2 patients. MEN2-related PCs are more often bilateral, identified at a younger age and have a low metastatic potential. They secrete epinephrine as the predominant catecholamine, along with its metabolite metanephrine, and lesser amounts of norepinephrine and normetanephrine. The advent of molecular diagnostic tools has enhanced the identification and stratification of these tumors, revealing a strong genotype-phenotype correlation which is crucial for screening and managing patients. Evaluation involves a combination of structural (CT/MRI) and functional imaging. MIBG remains helpful for PC assessment but novel PET ligands (18F-DOPA, 68Ga-DOTATATE, 18F-FDG) aid in the detection of extra-adrenal paragangliomas, recurrence, and metastatic disease. The treatment paradigm has shifted toward personalized medicine, incorporating genetic insights to tailor interventions, particularly surgical approaches and novel therapeutics such as radiolabeling of somatostatin analogs with lutetium and tyrosine kinase inhibitors.
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Affiliation(s)
- Matti L Gild
- Department of Endocrinology and Diabetes, Royal North Shore Hospital, Sydney, Australia.
- Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia.
- Cancer Genetics Laboratory, Kolling Institute of Medical Research, St Leonards, NSW, Australia.
| | - Kimchi Do
- Department of Endocrinology, Nepean Hospital, Sydney, Australia
| | - Venessa H M Tsang
- Department of Endocrinology and Diabetes, Royal North Shore Hospital, Sydney, Australia
- Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
| | - Lyndal J Tacon
- Department of Endocrinology and Diabetes, Royal North Shore Hospital, Sydney, Australia
- Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
| | - Roderick J Clifton-Bligh
- Department of Endocrinology and Diabetes, Royal North Shore Hospital, Sydney, Australia
- Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
- Cancer Genetics Laboratory, Kolling Institute of Medical Research, St Leonards, NSW, Australia
| | - Bruce G Robinson
- Department of Endocrinology and Diabetes, Royal North Shore Hospital, Sydney, Australia
- Faculty of Medicine and Health, University of Sydney, Camperdown, NSW, Australia
- Cancer Genetics Laboratory, Kolling Institute of Medical Research, St Leonards, NSW, Australia
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2
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Echeverria Ortegon E, Millet-Herrera JL, Casillas J. Case Report: Unveiling an anomalous diaphragmatic paraganglioma mimicking a hepatic tumor. F1000Res 2024; 13:1023. [PMID: 39649836 PMCID: PMC11621607 DOI: 10.12688/f1000research.155205.2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/21/2024] [Indexed: 12/11/2024] Open
Abstract
Paragangliomas are rare neuroendocrine tumors, often associated with catecholamine secretion. These tumors can arise in various locations, with the majority found in the abdomen and pelvis, while a smaller percentage occurs in the thorax and head and neck regions. Diaphragmatic paragangliomas are exceedingly rare, with only two documented cases in the literature. This report details a case of a primary diaphragmatic paraganglioma in a 59-year-old patient presenting with unexplained weight loss, tremors, and diaphoresis. Imaging studies revealed a mass in the right lobe of the liver, later identified as a diaphragmatic paraganglioma during surgery. The case underscores the importance of preoperative catecholamine assessment and careful surgical planning due to the risks associated with tumor manipulation. Complete surgical resection, although challenging, remains the definitive treatment, especially in hypervascular tumors located near major vascular structures.
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Affiliation(s)
| | | | - Javier Casillas
- Division of Radiology, Jackson Memorial Hospital, Miami, Florida, USA
- Department of Radiology, University of Miami Health System, Miami, Florida, USA
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3
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Jawanjal AB, Sadananda S, Sriramaneni N, Ganavi YP, Kalra P, Selvan C, Manjunath PR, Shetty PK. Demographic and Clinical Characteristics of Patients with Adrenal Incidentaloma from a Single Center in South India: A Retrospective Study. Ann Afr Med 2024; 23:567-574. [PMID: 39164941 PMCID: PMC11556502 DOI: 10.4103/aam.aam_212_23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/23/2023] [Revised: 01/23/2024] [Accepted: 01/31/2024] [Indexed: 08/22/2024] Open
Abstract
CONTEXT Adrenal incidentalomas (AIs) are relatively uncommon neoplasms in 2% of apparently healthy individuals requiring evaluation for functionality and malignancy. AIM We aimed to study the clinical, biochemical, and radiological profiles of patients presenting with AI and histopathological outcomes of those undergoing adrenalectomy. Materials and Methods: This retrospective study enrolled 62 AI patients attending a tertiary care center in South India between January 2016 and October 2023. Demographic details, radiological features, functionality, and histopathological data were analyzed. RESULTS Out of 62 patients, total masses evaluated were 65 indicating bilaterality in 3 patients. The female: male ratio was 1.69, with a median age of 55 years (interquartile range: 44-64 years). 45.1% of individuals were >60 years. The most common indication for imaging was pain abdomen in 43 (69.4%). The median size was 3.2 cm. Fifty-five (88.7%) were assessed for functionality and 27 (49.1%) were functional. Among the 62 individuals, 14 (20.2%) had hypercortisolism, 11 (15.9%) had pheochromocytoma, 5 (7.24%) had primary hyperaldosteronism (PA), and 4 (5.7%) had hyperandrogenism including plurihormonal in 7. A mass size of 3.2 cm was of great value in distinguishing functional tumors with a sensitivity of 72% and specificity of 66% with an area under the curve of 0.682. A total of 34 (54.8%) patients underwent adrenalectomy. On histopathological examination, Adenoma (44.1%) was the most common followed pheochromocytoma (26.5%), adrenal cysts (8.8%), and Myelolipoma (5.9%). Two (5.9%) incidentalomas were adrenocortical carcinoma (ACC). Eight (53.3%) adenomas were functional with 6 having hypercortisolism (including 1 with hyperandrogenism) and 2 with PA. CONCLUSION In our experience, the incidence of pheochromocytoma was second most common after adenoma. Since most functional tumors (60%) and all ACCs were ≥4 cm, a thorough biochemical evaluation for hormonal excess and evaluation for malignancy followed by surgery should be considered for lesions, especially ≥4 cm. Thus, we report the baseline demographic and clinical characteristics of patients with AI from a single center in South India.
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Affiliation(s)
- Abhijeet B Jawanjal
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Siddarth Sadananda
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Nikitha Sriramaneni
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Y. P. Ganavi
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Pramila Kalra
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Chitra Selvan
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - P. R. Manjunath
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
| | - Praneethi K. Shetty
- Department of Endocrinology, Ramaiah Medical College, Bengaluru, Karnataka, India
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4
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Chung SM. Screening and treatment of endocrine hypertension focusing on adrenal gland disorders: a narrative review. JOURNAL OF YEUNGNAM MEDICAL SCIENCE 2024; 41:269-278. [PMID: 39295528 PMCID: PMC11534415 DOI: 10.12701/jyms.2024.00752] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/19/2024] [Revised: 08/27/2024] [Accepted: 09/03/2024] [Indexed: 09/21/2024]
Abstract
Most cases of high blood pressure have no identifiable cause, termed essential hypertension; however, in approximately 15% of cases, hypertension occurs due to secondary causes. Primary aldosteronism (PA) and pheochromocytoma and paraganglioma (PPGL) are representative endocrine hypertensive diseases. The differentiation of endocrine hypertension provides an opportunity to cure and prevent target organ damage. PA is the most common cause of secondary hypertension, which significantly increases the risk of cardiovascular disease compared to essential hypertension; thus, patients with clinical manifestations suggestive of secondary hypertension should be screened for PA. PPGL are rare but can be fatal when misdiagnosed. PPGL are the most common hereditary endocrine tumors; therefore, genetic testing using next-generation sequencing panels is recommended. Herein, we aimed to summarize the characteristic clinical symptoms of PA and PPGL and when and how diagnostic tests and treatment strategies should be performed.
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Affiliation(s)
- Seung Min Chung
- Division of Endocrinology and Metabolism, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
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5
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Hu J, Kassu R, Titanji B, Kebebew E. Evaluation of Adrenal Incidentaloma. Surg Clin North Am 2024; 104:837-849. [PMID: 38944503 DOI: 10.1016/j.suc.2024.02.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/01/2024]
Abstract
Adrenal incidentalomas have increased due to advanced and more sensitive imaging modalities, their increased utilization, and the aging population. Most adrenal incidentalomas are nonfunctional and benign, but a subset of patients has functional and/or malignant tumors. The evaluation of patients with an adrenal incidentaloma involves addressing 2 clinical questions: (1) Is the tumor functional? (2) Is the tumor malignant? A careful history and physical examination focused on signs and symptoms of adrenal functional tumors, biochemical testing, and imaging features are the cornerstone in the evaluation of patients with an adrenal incidentaloma.
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Affiliation(s)
- Jiangnan Hu
- Division of General Surgery, Department of Surgery, Stanford University, School of Medicine, 269 Campus Drive, CCSR-3100, Stanford, CA 94305, USA
| | - Rodas Kassu
- Division of General Surgery, Department of Surgery, Stanford University, School of Medicine, 269 Campus Drive, CCSR-3100, Stanford, CA 94305, USA
| | - Bosung Titanji
- Meharry Medical College, 1005 Dr DB Todd Jr Boulevard, Nashville, TN 37208, USA
| | - Electron Kebebew
- Division of General Surgery, Department of Surgery, Stanford University, School of Medicine, 300 Pasteur Drive, H3642, Stanford, CA 94305-2200, USA.
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6
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Zhou Y, Zhan Y, Zhao J, Zhong L, Tan Y, Zeng W, Zeng Q, Gong M, Li A, Gong L, Liu L. CT-Based Radiomics Analysis of Different Machine Learning Models for Discriminating the Risk Stratification of Pheochromocytoma and Paraganglioma: A Multicenter Study. Acad Radiol 2024; 31:2859-2871. [PMID: 38302388 DOI: 10.1016/j.acra.2024.01.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2023] [Revised: 01/01/2024] [Accepted: 01/04/2024] [Indexed: 02/03/2024]
Abstract
RATIONALE AND OBJECTIVES Using different machine learning models CT-based radiomics to integrate clinical radiological features to discriminating the risk stratification of pheochromocytoma/paragangliomas (PPGLs). MATERIALS AND METHODS The present study included 201 patients with PPGLs from three hospitals (training set: n = 125; external validation set: n = 45; external test set: n = 31). Patients were divided into low-risk and high-risk groups using a staging system for adrenal pheochromocytoma and paraganglioma (GAPP). We extracted and selected CT radiomics features, and built radiomics models using support vector machines (SVM), k-nearest neighbors, random forests, and multilayer perceptrons. Using receiver operating characteristic curve analysis to select the optimal radiomics model, a combined model was built using the output of the optimal radiomics model and clinical radiological features, and its accuracy and clinical applicability were evaluated using calibration curves and clinical decision curve analysis (DCA). RESULTS Finally, 13 radiomics features were selected to construct machine learning models. In the radiomics model, the SVM model demonstrated higher accuracy and stability, with an AUC value of 0.915 in the training set, 0.846 in external validation set, and 0.857 in external test set. Combining the outputs of SVM models with two clinical radiological features, a combined model constructed has demonstrated optimal risk stratification ability for PPGLs with an AUC of 0.926 for the training set, 0.883 for the external validation set, and 0.899 for the external test set. The calibration curve and DCA show good calibration accuracy and clinical effectiveness for the combined model. CONCLUSION Combined model that integrates radiomics and clinical radiological features can discriminate the risk stratification of PPGLs.
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Affiliation(s)
- Yongjie Zhou
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China; The Second Affiliated Hospital of Nanchang Medical College, Nanchang, China; Jiangxi Clinical Research Center for Cancer, Nanchang, China
| | - Yuan Zhan
- Department of Pathology, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Jinhong Zhao
- Department of Radiology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Linhua Zhong
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China
| | - Yongming Tan
- Department of Radiology, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Wei Zeng
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China
| | - Qiao Zeng
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China
| | - Mingxian Gong
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China
| | - Aihua Li
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China
| | - Lianggeng Gong
- Department of Radiology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China
| | - Lan Liu
- Department of Radiology, Jiangxi Cancer Hospital, Nanchang, China; The Second Affiliated Hospital of Nanchang Medical College, Nanchang, China; Jiangxi Clinical Research Center for Cancer, Nanchang, China.
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7
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Nasiroglu Imga N, Deniz MS, Tural Balsak BO, Aslan Y, Tuncel A, Berker D. A comparative analysis of surgically excised hereditary and sporadic pheochromocytomas: Insights from a single-center experience. Kaohsiung J Med Sci 2024; 40:583-588. [PMID: 38747189 DOI: 10.1002/kjm2.12836] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2024] [Revised: 04/05/2024] [Accepted: 04/09/2024] [Indexed: 06/06/2024] Open
Abstract
Pheochromocytoma is a tumor that usually originating from adrenal medullary chromaffin cells and producing one or more catecholamines, can manifest as hereditary or sporadic. While the majority pheochromocytomas are sporadic, hereditary forms are often associated with genetic syndromes such as von Hippel-Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. This study aims to analyze data from our series of surgically excited pheochromocytoma patients and compare the characteristics between hereditary and sporadic cases. We retrospectively evaluated 33 diagnosed pheochromocytoma patients, documenting clinical features, surgical complications, and tumor characteristics in both hereditary and sporadic cases. Among the patients, 21% (7 individuals) had hereditary pheochromocytoma, while 79% (26 individuals) had sporadic cases. During diagnosis, hereditary pheochromocytoma patients exhibited a significantly lower mean age compared to the sporadic group (26.4 ± 9.9 years vs. 50.4 ± 14.0 years; p < 0.001). The maximum tumor size was also lower in hereditary cases compared to sporadic cases (p = 0.004). Adrenal tumor localization analysis showed that 63.6% were right-sided, 24.2% were left-sided, and 12.1% were bilateral. Laboratory analysis revealed significantly higher urinary norepinephrine levels in hereditary pheochromocytoma patients (p = 0.021). Our findings suggest that hereditary pheochromocytoma cases are characterized by a younger age at diagnosis, smaller tumor size, and a higher prevalence of multiple bilateral adrenal adenomas. We recommend genetic testing for all pheochromocytoma patients, particularly those with early-onset disease and bilateral adrenal tumors.
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Affiliation(s)
- Narin Nasiroglu Imga
- Department of Endocrinology and Metabolism, University of Health Science, Ankara City Hospital, Ankara, Turkey
| | - Muzaffer Serdar Deniz
- Department of Endocrinology and Metabolism, University of Health Science, Ankara City Hospital, Ankara, Turkey
| | - Belma Ozlem Tural Balsak
- Department of Endocrinology and Metabolism, University of Health Science, Ankara City Hospital, Ankara, Turkey
| | - Yilmaz Aslan
- Department of Urology, University of Health Science, Ankara City Hospital, Ankara, Turkey
| | - Altug Tuncel
- Department of Urology, University of Health Science, Ankara City Hospital, Ankara, Turkey
| | - Dilek Berker
- Department of Endocrinology and Metabolism, University of Health Science, Ankara City Hospital, Ankara, Turkey
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8
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Timmers HJLM, Taïeb D, Pacak K, Lenders JWM. Imaging of Pheochromocytomas and Paragangliomas. Endocr Rev 2024; 45:414-434. [PMID: 38206185 PMCID: PMC11074798 DOI: 10.1210/endrev/bnae001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2023] [Revised: 12/11/2023] [Accepted: 01/10/2024] [Indexed: 01/12/2024]
Abstract
Pheochromocytomas/paragangliomas are unique in their highly variable molecular landscape driven by genetic alterations, either germline or somatic. These mutations translate into different clusters with distinct tumor locations, biochemical/metabolomic features, tumor cell characteristics (eg, receptors, transporters), and disease course. Such tumor heterogeneity calls for different imaging strategies in order to provide proper diagnosis and follow-up. This also warrants selection of the most appropriate and locally available imaging modalities tailored to an individual patient based on consideration of many relevant factors including age, (anticipated) tumor location(s), size, and multifocality, underlying genotype, biochemical phenotype, chance of metastases, as well as the patient's personal preference and treatment goals. Anatomical imaging using computed tomography and magnetic resonance imaging and functional imaging using positron emission tomography and single photon emission computed tomography are currently a cornerstone in the evaluation of patients with pheochromocytomas/paragangliomas. In modern nuclear medicine practice, a multitude of radionuclides with relevance to diagnostic work-up and treatment planning (theranostics) is available, including radiolabeled metaiodobenzylguanidine, fluorodeoxyglucose, fluorodihydroxyphenylalanine, and somatostatin analogues. This review amalgamates up-to-date imaging guidelines, expert opinions, and recent discoveries. Based on the rich toolbox for anatomical and functional imaging that is currently available, we aim to define a customized approach in patients with (suspected) pheochromocytomas/paragangliomas from a practical clinical perspective. We provide imaging algorithms for different starting points for initial diagnostic work-up and course of the disease, including adrenal incidentaloma, established biochemical diagnosis, postsurgical follow-up, tumor screening in pathogenic variant carriers, staging and restaging of metastatic disease, theranostics, and response monitoring.
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Affiliation(s)
- Henri J L M Timmers
- Department of Internal Medicine, Radboud University Medical Centre, 6525 GA Nijmegen, The Netherlands
| | - David Taïeb
- Department of Nuclear Medicine, La Timone University Hospital, Aix-Marseille University, Marseille, France and European Center for Research in Medical Imaging, Aix-Marseille University, 13005 Marseille, France
| | - Karel Pacak
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892-1583, USA
| | - Jacques W M Lenders
- Department of Internal Medicine, Radboud University Medical Centre, 6525 GA Nijmegen, The Netherlands
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9
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Fagundes GFC, Almeida MQ. Pitfalls in the Diagnostic Evaluation of Pheochromocytomas. J Endocr Soc 2024; 8:bvae078. [PMID: 38737592 PMCID: PMC11087876 DOI: 10.1210/jendso/bvae078] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2024] [Indexed: 05/14/2024] Open
Abstract
Pheochromocytomas and paragangliomas (PPGLs), rare neuroendocrine tumors arising from chromaffin cells, present a significant diagnostic challenge due to their clinical rarity and polymorphic symptomatology. The clinical cases demonstrate the importance of an integrated approach that combines clinical assessment, biochemical testing, and imaging to distinguish PPGLs from mimicking conditions, such as obstructive sleep apnea and interfering medication effects, which can lead to false-positive biochemical results. Although a rare condition, false-negative metanephrine levels can occur in pheochromocytomas, but imaging findings can give some clues and increase suspicion for a pheochromocytoma diagnosis. This expert endocrine consult underscores the critical role of evaluating preanalytical conditions and pretest probability in the biochemical diagnosis of PPGLs. Moreover, a careful differentiation of PPGLs from similar conditions and careful selection and interpretation of diagnostic tests, with focus on understanding and reducing false positives to enhance diagnostic accuracy and patient outcomes, is crucial.
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Affiliation(s)
- Gustavo F C Fagundes
- Unidade de Adrenal, Laboratório de Endocrinologia Molecular e Celular LIM/25, Disciplina de Endocrinologia e Metabologia, Departamento de Medicina Interna, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, 01246-903, São Paulo, SP, Brazil
| | - Madson Q Almeida
- Unidade de Adrenal, Laboratório de Endocrinologia Molecular e Celular LIM/25, Disciplina de Endocrinologia e Metabologia, Departamento de Medicina Interna, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, 01246-903, São Paulo, SP, Brazil
- Unidade de Oncologia Endócrina, Instituto do Câncer do Estado de São Paulo (ICESP), Faculdade de Medicina da Universidade de São Paulo, 01246-903, São Paulo, SP, Brazil
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10
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Kwek KJH, Yeo CP, Ho BCS, Kon YC. Small Incidental Pheochromocytoma Presenting With Normal or Borderline High 24-hour Urine Fractionated Metanephrines. JCEM CASE REPORTS 2024; 2:luae035. [PMID: 38487512 PMCID: PMC10939167 DOI: 10.1210/jcemcr/luae035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 12/12/2023] [Indexed: 03/17/2024]
Abstract
A 73-year-old man was found to have a 2-cm lipid-poor right adrenal incidentaloma on computed tomography imaging for hematuria. Twenty-four-hour urine metanephrine was 1.1-fold elevated, then normal on repeat measurement. Paired with the second urine collection, plasma metanephrine measured by liquid chromatography tandem mass spectrometry after a 30-minute supine rest was 3.3-fold elevated. Plasma normetanephrine was 1.2-fold elevated. The 24-hour urine catecholamines and normetanephrine, measured twice, were normal. He received low-dose phenoxybenzamine and underwent successful resection of right pheochromocytoma. Postoperatively, both plasma metanephrine and normetanephrine levels normalized, using an age-appropriate upper reference limit for plasma normetanephrine. Patients who harbor small lipid-poor adrenal incidentalomas have a relatively high risk (>5%) of having pheochromocytoma, indistinguishable from adenomas or carcinomas on computed tomography scan. In such cases when 24-hour urine fractionated metanephrines are normal, plasma free metanephrines measured by liquid chromatography tandem mass spectrometry under optimal sampling conditions that are 2-fold or more elevated confirm the diagnosis of pheochromocytoma. Preoperative alpha blockade followed by surgical resection is then appropriate, rather than continued monitoring with repeat urine measurements.
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Affiliation(s)
| | - Chin Pin Yeo
- Department of Clinical Pathology, Singapore General Hospital, 169608, Singapore
| | | | - Yin Chian Kon
- Department of Endocrinology, Tan Tock Seng Hospital, 308433, Singapore
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11
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Kocic S, Vukomanovic V, Djukic A, Saponjski J, Saponjski D, Aleksic V, Ignjatovic V, Vuleta Nedic K, Markovic V, Vojinovic R. Can MDCT Enhancement Patterns Be Helpful in Differentiating Secretory from Non-Functional Adrenal Adenoma? MEDICINA (KAUNAS, LITHUANIA) 2023; 60:72. [PMID: 38256333 PMCID: PMC10819253 DOI: 10.3390/medicina60010072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Revised: 12/14/2023] [Accepted: 12/19/2023] [Indexed: 01/24/2024]
Abstract
Background and Objectives: Primary adrenal tumors (AT) are a heterogeneous group of neoplasms due to their functional heterogeneity, which results in the diverse clinical presentation of these tumors. The purpose of this study was to examine cross-sectional imaging characteristics using multi-detector computed tomography (MDCT) to provide insight into the lesion characterization and functional status of these tumors. The radionuclide imaging using Technetium-99m radiolabeled hydrazinonicotinylacid-d-phenylalanyl1-tyrosine3-octreotide (99mTc-HYNIC-TOC), was also used in the diagnostic evaluation of these tumors. Materials and Methods: This cross-sectional study included 50 patients with confirmed diagnoses of AT (21 hormone-secreting and 29 non-functional) at the University Clinical Center, Kragujevac, Serbia, during the 2019-2022 year period. The morphological and dynamic characteristics using MDCT were performed, using qualitative, semi-quantitative, and quantitative analysis. Absolute washout (APW) and relative washout (RPW) values were also calculated. A semi-quantitative analysis of all visual findings with 99mTc-HYNIC-TOC was performed to compare the tumor to non-tumor tracer uptake. Results: A statistically significant difference was found in the MDCT values in the native phase (p < 0.05), the venous phase (p < 0.05), and the delayed phase (p < 0.001) to detect the existence of adrenal tumors. Most of these functional adrenocortical lesions (n = 44) can be differentiated using the delayed phase (p < 0.05), absolute percentage washout (APW) (p < 0.05), and relative percentage washout (RPW) (p < 0.001). Furthermore, 99mTc-HYNIC-TOC could have a high diagnostic yield to detect adrenal tumor existence (p < 0.001). There is a positive correlation between radionuclide imaging scan and APW to detect all AT (p < 0.01) and adrenocortical adenomas as well (p < 0.01). Conclusions: The results can be very helpful in a diagnostic algorithm to quickly and precisely diagnose the expansive processes of the adrenal glands, as well as to learn about the advantages and limitations of the mentioned imaging modalities.
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Affiliation(s)
- Svetlana Kocic
- Department of Radiology, Clinical Hospital Center Zemun, 11070 Belgrade, Serbia;
| | - Vladimir Vukomanovic
- Department of Nuclear Medicine, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia; (V.I.); (K.V.N.)
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
| | - Aleksandar Djukic
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
- Department of Pathophysiology, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia
| | - Jovica Saponjski
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia; (J.S.); (D.S.)
- University Clinical Center of Serbia, 11000 Belgrade, Serbia
| | - Dusan Saponjski
- Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia; (J.S.); (D.S.)
- University Clinical Center of Serbia, 11000 Belgrade, Serbia
| | - Vuk Aleksic
- Department of Neurosurgery, Clinical Hospital Center Zemun, 11070 Belgrade, Serbia;
| | - Vesna Ignjatovic
- Department of Nuclear Medicine, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia; (V.I.); (K.V.N.)
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
| | - Katarina Vuleta Nedic
- Department of Nuclear Medicine, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia; (V.I.); (K.V.N.)
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
| | - Vladan Markovic
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
- Department of Radiology, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia
| | - Radisa Vojinovic
- University Clinical Center Kragujevac, 34000 Kragujevac, Serbia; (A.D.); (V.M.); (R.V.)
- Department of Radiology, Faculty of Medical Science, University of Kragujevac, 34000 Kragujevac, Serbia
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12
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Aggarwal S, Prete A, Chortis V, Asia M, Sutcliffe RP, Arlt W, Ronchi CL, Karavitaki N, Ayuk J, Elhassan YS. Pheochromocytomas Most Commonly Present As Adrenal Incidentalomas: A Large Tertiary Center Experience. J Clin Endocrinol Metab 2023; 109:e389-e396. [PMID: 37417693 PMCID: PMC10735286 DOI: 10.1210/clinem/dgad401] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/20/2023] [Revised: 06/12/2023] [Accepted: 07/05/2023] [Indexed: 07/08/2023]
Abstract
CONTEXT Pheochromocytomas are increasingly diagnosed in incidentally detected adrenal masses. However, the characteristics of incidental pheochromocytomas are unclear. OBJECTIVE We aimed to assess the proportion and clinical, biochemical, radiological, genetic, histopathological, and follow-up characteristics of incidental pheochromocytomas. METHODS A retrospective review was conducted of patients with pheochromocytoma seen between January 2010 and October 2022 at a large UK tertiary care center. The diagnosis was confirmed histologically or by the combined presence of increased plasma and/or urinary metanephrines (MN), indeterminate adrenal mass on cross-sectional imaging, and metaiodobenzylguanidine avidity. RESULTS We identified 167 patients with pheochromocytoma; 144 (86.2%) underwent adrenalectomy, for 23 (13.8%) surgery was either awaited, deemed unsuitable due to frailty or other metastatic malignancy, or declined by the patients. Excluding pheochromocytomas diagnosed via screening genetically predisposed individuals (N = 20), 37 of 132 (28.0%) presented with adrenergic symptoms and/or uncontrolled hypertension, while 91 of 132 (69.0%) patients presented with an incidentally detected adrenal mass. Incidentally detected patients were older (median age 62 years) than those detected due to clinical suspicion (aged 42 years) or after genetic screening (aged 33 years) (all P < .05). Incidentally detected pheochromocytomas were smaller (median 42 mm) than tumors detected due to adrenergic symptoms/uncontrolled hypertension (60 mm), but larger than tumors identified by genetic screening (30 mm) (all P < .05). Increased MN excretion showed a similar pattern (symptomatic/uncontrolled hypertension > incidental > genetic screening) (all P < .05). Hereditary predisposition was detected in 20.4% of patients (incidental, 15.3%; symptomatic/uncontrolled hypertension, 42.9%). CONCLUSION The majority of pheochromocytomas are diagnosed incidentally and have distinct clinical, radiological, biochemical, and genetic features. Their detection at older age but smaller size may point to a different underlying tumor biology.
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Affiliation(s)
- Sunil Aggarwal
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Alessandro Prete
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
- NIHR Birmingham Biomedical Research Centre, University of Birmingham and University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TT, UK
| | - Vasileios Chortis
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Miriam Asia
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Robert P Sutcliffe
- Department of Hepatobiliary Surgery, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Wiebke Arlt
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Cristina L Ronchi
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Niki Karavitaki
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - John Ayuk
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
| | - Yasir S Elhassan
- Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK
- Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK
- Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK
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13
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Sharma S, Fishbein L. Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician. Endocr Pract 2023; 29:999-1006. [PMID: 37586639 DOI: 10.1016/j.eprac.2023.07.027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/08/2023] [Revised: 07/10/2023] [Accepted: 07/24/2023] [Indexed: 08/18/2023]
Abstract
OBJECTIVE The aim of this review was to provide a practical approach for clinicians regarding the diagnosis and management of pheochromocytomas and paragangliomas (PPGLs). METHODS A literature search of PubMed was carried out using key words, including pheochromocytoma, paraganglioma, treatment, diagnosis, screening, and management. The discussion of diagnosis and management of PPGL is based on the evidence available from prospective studies when available and mostly from cohort studies, cross-sectional studies, and expert consensus. RESULTS PPGL are neuroendocrine tumors arising from the chromaffin cells of adrenal medulla and sympathetic and parasympathetic ganglia, respectively. PPGL can be localized or metastatic, and they may secrete catecholamines, causing a variety of symptoms and potentially catastrophic and lethal complications if left untreated. The rarity of these tumors along with heterogeneous clinical presentation often poses challenges for the diagnosis and management. PPGL can be associated with several familial syndromes which are important to recognize. CONCLUSION The last few years have witnessed an exponential growth in the knowledge around PPGL. This review aims at providing a comprehensive discussion of current concepts for clinicians regarding clinical presentation, diagnostic tools, and management strategies for PPGL.
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Affiliation(s)
- Sona Sharma
- Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, Duke University, Durham, North Carolina
| | - Lauren Fishbein
- Department of Medicine, Division of Endocrinology, Diabetes and Metabolism and Rocky Mountain Regional Veteran's Administration Medical Center, Endocrinology Section, University of Colorado School of Medicine, Aurora, Colorado.
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14
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Nazari MA, Hasan R, Haigney M, Maghsoudi A, Lenders JWM, Carey RM, Pacak K. Catecholamine-induced hypertensive crises: current insights and management. Lancet Diabetes Endocrinol 2023; 11:942-954. [PMID: 37944546 DOI: 10.1016/s2213-8587(23)00256-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2023] [Revised: 08/03/2023] [Accepted: 08/15/2023] [Indexed: 11/12/2023]
Abstract
Phaeochromocytomas and paragangliomas (PPGLs) release catecholamines leading to catecholamine-induced hypertensive (CIH) crises, with blood pressure greater than or equal to 180/120 mm Hg. CIH crises can be complicated by tachyarrhythmias, hypotension, or life-threatening target organ damage while treatment remains undefined, often requiring co-management between endocrinologists and cardiologists. Furthermore, biochemical diagnosis of a PPGL as a cause of a CIH crisis can be difficult to identify or confounded by comorbid conditions, potentially resulting in misdiagnosis. Here, we combine relevant evidence, 60 years of collective clinical experience, insights derived from assessing over 2600 patients with PPGL, and supplementary outcomes from 100 patients (treated at the National Institutes of Health) with a CIH crisis to inform diagnosis and treatment of CIH crises. Recognising that disparities exist between availability, cost, and familiarity of various agents, flexible approaches are delineated allowing for customisation, given institutional availability and provider preference. A CIH crisis and its complications are readily treatable with available drugs, with effective intervention defining an avenue for mitigating consequent morbidity and mortality in patients with PPGL.
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Affiliation(s)
- Matthew A Nazari
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
| | - Rockyb Hasan
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
| | - Mark Haigney
- Division of Cardiology, Department of Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD, USA; Division of Cardiology, Department of Medicine, Walter Reed National Military Medical Center and Herbert School of Medicine, Bethesda, MD, USA
| | - Alireza Maghsoudi
- Department of Cardiovascular Disease, INOVA Heart and Vascular Institute, Fairfax, VA, USA
| | - Jacques W M Lenders
- Department of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands; Department of Medicine, University Hospital Carl Gustav Carus at the TU Dresden, Dresden, Germany
| | - Robert M Carey
- Department of Medicine, University of Virginia Health System, Charlottesville, VA, USA
| | - Karel Pacak
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
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15
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Kiriakopoulos A, Giannakis P, Menenakos E. Pheochromocytoma: a changing perspective and current concepts. Ther Adv Endocrinol Metab 2023; 14:20420188231207544. [PMID: 37916027 PMCID: PMC10617285 DOI: 10.1177/20420188231207544] [Citation(s) in RCA: 9] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2023] [Accepted: 09/26/2023] [Indexed: 11/03/2023] Open
Abstract
This article aims to review current concepts in diagnosing and managing pheochromocytoma and paraganglioma (PPGL). Personalized genetic testing is vital, as 40-60% of tumors are linked to a known mutation. Tumor DNA should be sampled first. Next-generation sequencing is the best and most cost-effective choice and also helps with the expansion of current knowledge. Recent advancements have also led to the increased incorporation of regulatory RNA, metabolome markers, and the NETest in PPGL workup. PPGL presentation is highly volatile and nonspecific due to its multifactorial etiology. Symptoms mainly derive from catecholamine (CMN) excess or mass effect, primarily affecting the cardiovascular system. However, paroxysmal nature, hypertension, and the classic triad are no longer perceived as telltale signs. Identifying high-risk subjects and diagnosing patients at the correct time by using appropriate personalized methods are essential. Free plasma/urine catecholamine metabolites must be first-line examinations using liquid chromatography with tandem mass spectrometry as the gold standard analytical method. Reference intervals should be personalized according to demographics and comorbidity. The same applies to result interpretation. Threefold increase from the upper limit is highly suggestive of PPGL. Computed tomography (CT) is preferred for pheochromocytoma due to better cost-effectiveness and spatial resolution. Unenhanced attenuation of >10HU in non-contrast CT is indicative. The choice of extra-adrenal tumor imaging is based on location. Functional imaging with positron emission tomography/computed tomography and radionuclide administration improves diagnostic accuracy, especially in extra-adrenal/malignant or familial cases. Surgery is the mainstay treatment when feasible. Preoperative α-adrenergic blockade reduces surgical morbidity. Aggressive metastatic PPGL benefits from systemic chemotherapy, while milder cases can be managed with radionuclides. Short-term postoperative follow-up evaluates the adequacy of resection. Long-term follow-up assesses the risk of recurrence or metastasis. Asymptomatic carriers and their families can benefit from surveillance, with intervals depending on the specific gene mutation. Trials primarily focusing on targeted therapy and radionuclides are currently active. A multidisciplinary approach, correct timing, and personalization are key for successful PPGL management.
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Affiliation(s)
- Andreas Kiriakopoulos
- Department of Surgery, ‘Evgenidion Hospital’, National and Kapodistrian University of Athens School of Medicine, 5th Surgical Clinic, Papadiamantopoulou 20 Str, PO: 11528, Athens 11528, Greece
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16
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Boot CS. A Laboratory Medicine Perspective on the Investigation of Phaeochromocytoma and Paraganglioma. Diagnostics (Basel) 2023; 13:2940. [PMID: 37761307 PMCID: PMC10529273 DOI: 10.3390/diagnostics13182940] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2023] [Revised: 09/04/2023] [Accepted: 09/07/2023] [Indexed: 09/29/2023] Open
Abstract
Phaeochromocytomas (PC) and sympathetic paragangliomas (PGL) are potentially malignant tumours arising from the adrenal medulla (PC) or elsewhere in the sympathetic nervous system (PGL). These tumours usually secrete catecholamines and are associated with significant morbidity and mortality, so accurate and timely diagnosis is essential. The initial diagnosis of phaeochromocytoma/paraganglioma (PPGL) is often dependent on biochemical testing. There is a range of pre-analytical, analytical and post-analytical factors influencing the analytical and diagnostic performance of biochemical tests for PPGL. Pre-analytical factors include patient preparation, sample handling and choice of test. Analytical factors include choice of methodology and the potential for analytical interference from medications and other compounds. Important factors in the post-analytical phase include provision of appropriate reference ranges, an understanding of the potential effects of various medications on metanephrine concentrations in urine and plasma and a consideration of PPGL prevalence in the patient population being tested. This article reviews these pre-analytical, analytical and post-analytical factors that must be understood in order to provide effective laboratory services for biochemical testing in the diagnosis of PPGL.
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Affiliation(s)
- Christopher S Boot
- Department of Blood Sciences, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 4LP, UK
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17
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Park SS, Kim JH. Recent Updates on the Management of Adrenal Incidentalomas. Endocrinol Metab (Seoul) 2023; 38:373-380. [PMID: 37583083 PMCID: PMC10475962 DOI: 10.3803/enm.2023.1779] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2023] [Revised: 07/21/2023] [Accepted: 07/25/2023] [Indexed: 08/17/2023] Open
Abstract
Adrenal incidentalomas represent an increasingly common clinical conundrum with significant implications for patients. The revised 2023 European Society of Endocrinology (ESE) guideline incorporates cutting-edge evidence for managing adrenal incidentalomas. This paper provides a concise review of the updated contents of the revised guideline. In the 2023 guideline, in patients without signs and symptoms of overt Cushing's syndrome, a post-dexamethasone cortisol level above 50 nmol/L (>1.8 μg/dL) should be considered as mild autonomous cortisol secretion. Regarding the criteria of benign adrenal adenomas, a homogeneous adrenal mass with ≤10 Hounsfield units on non-contrast computed tomography requires no further follow-up, irrespective of its size. The updated guideline also discusses steroid metabolomics using tandem mass spectrometry to discriminate malignancy. It underscores the importance of high-volume surgeons performing adrenalectomy and emphasizes the pivotal role of a multidisciplinary team approach in deciding the treatment plan for indeterminate adrenal masses. The guideline advocates for more proactive surgical treatment for indeterminate adrenal masses in young patients (<40 years) and pregnant women. This review of the 2023 ESE guideline underscores the ongoing evolution of the adrenal incidentaloma management landscape, emphasizing the need for further research and adaptation of diagnostic and therapeutic strategies.
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Affiliation(s)
- Seung Shin Park
- Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
| | - Jung Hee Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
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18
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Ceccato F, Correa R, Livhits M, Falhammar H. Editorial: Predictive tools in pheochromocytoma and paraganglioma. Front Endocrinol (Lausanne) 2023; 14:1227543. [PMID: 37383393 PMCID: PMC10298152 DOI: 10.3389/fendo.2023.1227543] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2023] [Accepted: 06/02/2023] [Indexed: 06/30/2023] Open
Affiliation(s)
- Filippo Ceccato
- Department of Medicine DIMED, University of Padua, Padua, Italy
- Endocrine Disease Unit, University-Hospital of Padua, Padua, Italy
| | - Ricardo Correa
- Endocrinology, Diabetes and Metabolism, The University of Arizona College of Medicine Phoenix, Phoenix, AZ, United States
| | - Masha Livhits
- Department of General Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA, United States
| | - Henrik Falhammar
- Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden
- Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
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19
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Grigoryan S, Nhan W, Zhang L, Urban C, Zhao L, Turcu AF. Rates of Pheochromocytoma/Paraganglioma Screening in At-Risk Populations. J Clin Endocrinol Metab 2023; 108:e343-e349. [PMID: 36469797 PMCID: PMC10188311 DOI: 10.1210/clinem/dgac701] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2022] [Revised: 11/11/2022] [Accepted: 11/30/2022] [Indexed: 12/12/2022]
Abstract
CONTEXT Pheochromocytomas and paragangliomas (PPGL) are rare causes of secondary hypertension, but when unrecognized, they can lead to serious complications. Data regarding PPGL screening are lacking. OBJECTIVE This study aimed to assess the rates and patterns of PPGL screening among eligible patients. METHODS We conducted a retrospective review of adults with hypertension seen in outpatient clinics of a large academic center between January 1, 2017, and June 30, 2020. We included patients with treatment-resistant hypertension, hypertension at age < 35 years, and/or adrenal mass(es). RESULTS Of 203 535 patients with hypertension identified, 71 088 (35%) met ≥ 1 inclusion criteria, and 2013 (2.83%) were screened for PPGL. Patients screened were younger (56.2 ± 17.4 vs 64.0 ± 17.1 years), more often women (54.1% vs 44.2%), and never-smokers (54.6% vs 47.5%, P < 0.001 for all). The rate of screening was highest in patients with hypertension and adrenal mass(es) (51.7%, vs 3.9% in patients with early-onset hypertension, and 2.4% in those with treatment-resistant hypertension). Multivariable logistic regression showed higher odds ratio (OR) of PPGL screening in women (OR [95% CI]: 1.48 [1.34-1.63]); Black vs White patients (1.35 [1.19-1.53]); patients with adrenal mass(es) (55.1 [44.53-68.15]), stroke (1.34 [1.16-1.54]), dyslipidemia (1.41 [1.26-1.58]), chronic kidney disease (1.40 [1.26-1.56]), and obstructive sleep apnea (1.96 [1.76-2.19]). CONCLUSION PPGL screening is pursued in roughly half of patients with adrenal nodules and hypertension, but rarely in patients with treatment-resistant or early-onset hypertension. Similar to screening for other forms of secondary hypertension, PPGL screening occurs more often after serious complications develop.
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Affiliation(s)
- Seda Grigoryan
- Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor, MI 48109, USA
| | - Winnie Nhan
- Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor, MI 48109, USA
| | - Lei Zhang
- School of Public Health, University of Michigan, Ann Arbor, MI 48109, USA
| | - Caitlin Urban
- Michigan State University College of Human Medicine, Grand Rapids, MI 49503, USA
| | - Lili Zhao
- School of Public Health, University of Michigan, Ann Arbor, MI 48109, USA
| | - Adina F Turcu
- Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor, MI 48109, USA
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20
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Herndon J, Bancos I. Diagnosing and managing adrenal incidentalomas. JAAPA 2023; 36:12-18. [PMID: 37043721 DOI: 10.1097/01.jaa.0000923528.75127.88] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/14/2023]
Abstract
ABSTRACT Adrenal incidentalomas are commonly encountered because of the widespread use of high-resolution cross-sectional imaging. Adrenal incidentalomas may be benign or malignant, and also may demonstrate hormonal hypersecretion, so all patients with adrenal masses should undergo further assessment. Clinicians should have a basic understanding of adrenal incidentalomas, their workup, and when follow-up and referral are warranted.
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Affiliation(s)
- Justine Herndon
- At the Mayo Clinic in Rochester, Minn., Justine Herndon practices in the Division of Endocrinology, Diabetes, and Nutrition, and Irina Bancos practices in the Division of Endocrinology, Diabetes, and Nutrition and the Department of Laboratory Medicine and Pathology. Dr. Bancos reports advisory board participation and/or consulting with Lantheus, Sparrow Pharmaceuticals, Spruce Biosciences, Recordati Rare Disease, Corcept Therapeutics, Adrenas Therapeutics, and HRA Pharma. She also is partly supported by the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) of the National Institutes of Health (NIH) under awards K23DK121888 and R03DK132121. The views expressed are those of the authors and not necessarily those of the NIH. The authors have disclosed no other potential conflicts of interest, financial or otherwise
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21
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Teixeira AP, Haddad Jr. W, Barreto LO, Secaf A, Mermejo LM, Lucchesi FR, Tucci Jr. S, Elias Junior J, Molina CAF, Muglia VF. Histogram analysis in the differentiation between adrenal adenomas and pheochromocytomas: the value of a single measurement. Radiol Bras 2023; 56:59-66. [PMID: 37168042 PMCID: PMC10165976 DOI: 10.1590/0100-3984.2022.0067] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2022] [Accepted: 09/12/2022] [Indexed: 05/13/2023] Open
Abstract
Objective To assess the diagnostic accuracy of histogram analysis on unenhanced computed tomography (CT) for differentiating between adrenal adenomas and pheochromocytomas (PCCs). Materials and Methods We retrospectively identified patients with proven PCCs who had undergone CT examinations between January 2009 and July 2019 at one of two institutions. For each PCC, we selected one or two adenomas diagnosed within two weeks of the date of diagnosis of the PCC. For each lesion, two readers scored the size, determined the mean attenuation, and generated a voxel histogram. The 10th percentile (P10) was obtained from the conventional histogram analysis, as well as being calculated with the following formula: P10 = mean attenuation - (1.282 × standard deviation). The mean attenuation threshold, histogram analysis (observed) P10, and calculated P10 (calcP10) were compared in terms of their diagnostic accuracy. Results We included 52 adenomas and 29 PCCs. The sensitivity, specificity, and accuracy of the mean attenuation threshold were 75.0%, 100.0%, and 82.5%, respectively, for reader 1, whereas they were 71.5%, 100.0%, and 81.5%, respectively, for reader 2. The sensitivity, specificity, and accuracy of the observed P10 and calcP10 were equal for both readers: 90.4%, 96.5%, and 92.6%, respectively, for reader 1; and 92.3%, 93.1%, and 92.6%, respectively, for reader 2. The increase in sensitivity was significant for both readers (p = 0.009 and p = 0.005, respectively). Conclusion For differentiating between adenomas and PCCs, the histogram analysis (observed P10 and calcP10) appears to outperform the mean attenuation threshold as a diagnostic criterion.
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Affiliation(s)
- Ana P. Teixeira
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | - William Haddad Jr.
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | - Luan Oliveira Barreto
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | | | - Livia M. Mermejo
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | | | - Silvio Tucci Jr.
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | - Jorge Elias Junior
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | - Carlos A. F. Molina
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
| | - Valdair F. Muglia
- Faculdade de Medicina de Ribeirão Preto da Universidade de São
Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil
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22
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Crimì F, Agostini E, Toniolo A, Torresan F, Iacobone M, Tizianel I, Scaroni C, Quaia E, Campi C, Ceccato F. CT Texture Analysis of Adrenal Pheochromocytomas: A Pilot Study. Curr Oncol 2023; 30:2169-2177. [PMID: 36826128 PMCID: PMC9955696 DOI: 10.3390/curroncol30020167] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Revised: 02/02/2023] [Accepted: 02/07/2023] [Indexed: 02/12/2023] Open
Abstract
Radiomics is a promising research field that combines big data analysis (from tissue texture analysis) with clinical questions. We studied the application of CT texture analysis in adrenal pheochromocytomas (PCCs) to define the correlation between the extracted features and the secretory pattern, the histopathological data, and the natural history of the disease. A total of 17 patients affected by surgically removed PCCs were retrospectively enrolled. Before surgery, all patients underwent contrast-enhanced CT and complete endocrine evaluation (catecholamine secretion and genetic evaluation). The pheochromocytoma adrenal gland scaled score (PASS) was determined upon histopathological examination. After a resampling of all CT images, the PCCs were delineated using LifeX software in all three phases (unenhanced, arterial, and venous), and 58 texture parameters were extracted for each volume of interest. Using the Mann-Whitney test, the correlations between the hormonal hypersecretion, the malignancy score of the lesion (PASS > 4), and texture parameters were studied. The parameters DISCRETIZED_HUpeak and GLZLM_GLNU in the unenhanced phase and GLZLM_SZE, CONVENTIONAL_HUmean, CONVENTIONAL_HUQ3, DISCRETIZED_HUmean, DISCRETIZED_AUC_CSH, GLRLM_HGRE, and GLZLM_SZHGE in the venous phase were able to differentiate secreting PCCs (p < 0.01), and the parameters GLZLM_GLNU in the unenhanced phase and GLRLM_GLNU and GLRLM_RLNU in the venous differentiated tumors with low and high PASS. CT texture analysis of adrenal PCCs can be a useful tool for the early identification of secreting or malignant tumors.
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Affiliation(s)
- Filippo Crimì
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Institute of Radiology, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Elena Agostini
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Institute of Radiology, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Alessandro Toniolo
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Institute of Radiology, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Francesca Torresan
- Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Endocrine Surgery Unit, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Maurizio Iacobone
- Department of Surgical, Oncological and Gastroenterological Sciences, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Endocrine Surgery Unit, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Irene Tizianel
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Endocrinology Unit, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Carla Scaroni
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Endocrinology Unit, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Emilio Quaia
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Institute of Radiology, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
| | - Cristina Campi
- Department of Mathematics (DIMA), University of Genova, Via Dodecaneso 33, 16146 Genova, Italy
| | - Filippo Ceccato
- Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy
- Endocrinology Unit, Padova University Hospital, Via Giustiniani 2, 35128 Padova, Italy
- Correspondence:
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Igaz P. Pheochromocytoma/paraganglioma screening: low rates in at-risk populations. J Clin Endocrinol Metab 2023; 108:e350-e351. [PMID: 36652425 DOI: 10.1210/clinem/dgad029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Revised: 01/08/2023] [Accepted: 01/13/2023] [Indexed: 01/19/2023]
Affiliation(s)
- Peter Igaz
- Department of Endocrinology, Faculty of Medicine, Semmelweis University, 1083 Korányi Str. 2/a Budapest, Hungary
- Department of Internal Medicine and Oncology, Faculty of Medicine, Semmelweis University, 1083 Korányi Str. 2/a, Budapest, Hungary
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Garstka N, Remzi M. Diagnostic evaluation for adrenal tumors - What does the urologist need to know about endocrine metabolic work up? Curr Opin Urol 2023; 33:59-63. [PMID: 36239402 DOI: 10.1097/mou.0000000000001050] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
PURPOSE OF REVIEW Adrenal lesions are commonly discovered on abdominal imaging studies, whereas some patients with adrenal tumors present with symptoms of homonal excess. They are categorized as either hormone active or inactive and either as benign or malignant. This review is focused on the endocrine work up in patients with adrenal tumors. Knowledge of the use and limitations of hormonal assessment is essential for propper interpretation of the obtained test results. This article reviews which diagnostics are necessary to identify adrenal masses requiring treatment. RECENT FINDINGS Delayed hormonal assessment is not uncommon and associated with delayed treatment. The endocrine work up is guided by data from prospective and retrospective observational studies. Adrenal tumors include a wide spectrum of diseases and as a principle, most patients require biochemical testing to select the appropriate treatment. SUMMARY The most important factor for the outcome in the management of adrenal masses is, beside the exclusion of malignancy, a structured evaluation of the patients endocrine status.
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Affiliation(s)
- Nathalie Garstka
- Vienna General Hospital, Department of Urology, Medical University of Vienna, Austria
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25
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Dogra P, Navin PJ, McKenzie TJ, Foster T, Dy B, Lyden M, Young WF, Bancos I. Clinical, imaging and biochemical presentation of cystic pheochromocytomas. Clin Endocrinol (Oxf) 2023; 98:32-40. [PMID: 35445428 PMCID: PMC9585148 DOI: 10.1111/cen.14743] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/03/2022] [Revised: 04/03/2022] [Accepted: 04/10/2022] [Indexed: 12/16/2022]
Abstract
OBJECTIVE Cystic adrenal mass is a rare imaging presentation of pheochromocytoma. We aimed to describe the clinical, biochemical and imaging characteristics of patients with cystic pheochromocytoma. DESIGN Single-centre, retrospective study, 2000-2020. PATIENTS Consecutive patients with cystic pheochromocytoma were identified from our institutional pathology and adrenal tumour database. RESULTS Of the 638 patients with pheochromocytomas, 21 (3.2%) had cystic pheochromocytomas (median age: 57 years, 57% women). Most pheochromocytomas were discovered incidentally (57%) or due to symptoms of catecholamine excess (24%). The median tumour size was 6.4 cm. On imaging, cystic pheochromocytomas were round or oval (90%), heterogeneous lesions (86%) with a thick solid rim (median rim thickness 13.9 mm, unenhanced computed tomography (CT) attenuation 40 Hounsfield units (HU), venous-phase CT attenuation 83 HU), and a median cystic component of 40% (unenhanced CT attenuation 17.6 HU, venous-phase CT attenuation 20.4 HU), and rarely with calcifications (15%). All 20 patients with biochemical testing had functioning tumours (adrenergic in 80%, noradrenergic in 20%). Total urinary metanephrine excretion correlated with the volume of the solid component (R2 = .75, p < .0001) but not the cystic component (R2 = .04, p = .4386). All patients underwent adrenalectomy (48% laparoscopic, 52% open), and the median duration of hospital stay was 4 days. CONCLUSIONS Cystic pheochromocytomas are rare, large tumours with a phenotypic appearance that can masquerade as other adrenal cystic lesions. The degree of biochemical abnormality in cystic pheochromocytomas is associated with the volume of the solid component. All patients with adrenal cysts that have a solid component or an unenhanced attenuation >10 HU should undergo biochemical testing for pheochromocytoma.
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Affiliation(s)
- Prerna Dogra
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, USA
| | | | | | | | - Benzon Dy
- Department of Surgery, Mayo Clinic, Rochester, MN, USA
| | - Melanie Lyden
- Department of Surgery, Mayo Clinic, Rochester, MN, USA
| | - William F. Young
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, USA
| | - Irina Bancos
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, USA
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27
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Muacevic A, Adler JR, Aynaou H, Salhi H, El Ouahabi H. The Etiological Profile of Adrenal Incidentalomas. Cureus 2022; 14:e32564. [PMID: 36654569 PMCID: PMC9840518 DOI: 10.7759/cureus.32564] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/04/2022] [Indexed: 12/23/2022] Open
Abstract
Introduction An adrenal incidentaloma (AI) is an unsuspected tumor in one or both adrenal glands, which is discovered incidentally on an imaging exam not prompted by adrenal exploration. The etiologies can be multiple; they condition therapeutic management. The objective of our study is to describe the etiological and therapeutic profiles of AI in our department. Materials and methods A retrospective study was carried out in the Endocrinology, Diabetology, and Nutrition Department of the Hassan II University Hospital of Fez on patients managed for AI from September 2009 until March 2022. We included all the patients who were followed and/or hospitalized for adrenal incidentalomas. Results There were 86, predominantly female, patients (67.85%). The mean age was 58.91+/-14.40 years. The clinical findings were a unilateral adrenal mass in 73.25% of patients, localized on the left in 39.53%, on the right in 33.72%, and a bilateral one in 26.75%. Its size varied from 12 to 196 mm, with an average of 35.5 mm. The most common etiologies found in our series were a non-functional adrenal adenoma in 54.56%, a subclinical cortisolic adenoma in 19.76%, an adrenocortical carcinoma in 5.81%, and a pheochromocytoma in 5.81%. Adrenalectomy was indicated in 19.76% of our patients, 17.44% were monitored closely, 20.94% were monitored for comorbidities, and 41.86% had been advised to abstain from treatment. Conclusion An adrenal incidentaloma has become more and more frequent. It constitutes an entity with various etiologies, which can be serious. The main etiology in our series was non-functioning adrenal adenoma, for which therapeutic abstention was indicated in 48% of cases.
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28
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Yang M, Kang C, Zhu S. Effects of epidural anesthesia in pheochromocytoma and paraganglioma surgeries: A protocol for systematic review and meta-analysis. Medicine (Baltimore) 2022; 101:e31768. [PMID: 36451496 PMCID: PMC9704962 DOI: 10.1097/md.0000000000031768] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/25/2022] [Accepted: 10/21/2022] [Indexed: 12/02/2022] Open
Abstract
OBJECTIVE Anesthetic management is a great challenge during the surgical resection of pheochromocytomas and paragangliomas (PPGLs) due to potential hemodynamic fluctuations and/or postoperative complications. Although combined epidural-general anesthesia is commonly used in PPGLs surgeries, there is still no consensus whether combined epidural-general anesthesia was superior than general anesthesia alone for these populations. For the first time, we conducted this systematic review and meta-analysis to summarize the effects of combined epidural-general anesthesia versus general anesthesia alone on hemodynamic fluctuations as well as postoperative complications in patients undergone PPGLs surgeries. METHODS This systematic review and meta-analysis was performed according to the preferred reporting items for systematic reviews and meta-analyses statement. The primary outcome were hemodynamic fluctuations, including intraoperative hypotension, postoperative hypotension, and hypertensive crisis. Secondary outcome was the incidence of postoperative complications during hospital stay. RESULTS Finally, three retrospective cohort studies involving 347 patients met the inclusion criteria. A meta-analysis was not performed since outcomes from included studies were not available to be pooled. On the basis of the findings of non-randomized controlled trials (RCTs) literature, 2 studies suggested that combined epidural-general anesthesia was associated with intraoperative and postoperative hypotension, although one study reported that epidural anesthesia use reduced the incidence of postoperative complications in patients undergone surgical resection of PPGLs. CONCLUSIONS Currently, no published RCTs have yet assessed clinically relevant outcomes with respect to the application of epidural anesthesia during PPGLs surgeries. Well-designed RCTs should nonetheless be encouraged to properly assess the efficacy and safety of epidural anesthesia for PPGLs surgeries.
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Affiliation(s)
- Min Yang
- Department of Anesthesiology, Northwest Women’s and Children’s Hospital, Xi’an, China
| | - Chao Kang
- Department of Anesthesiology, Northwest Women’s and Children’s Hospital, Xi’an, China
| | - Shuai Zhu
- Department of Anesthesiology, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China
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Kang D, Kim BE, Hong M, Kim J, Jeong S, Lee S. Different intraoperative decisions for undiagnosed paraganglioma: Two case reports. World J Clin Cases 2022; 10:11059-11065. [PMID: 36338226 PMCID: PMC9631158 DOI: 10.12998/wjcc.v10.i30.11059] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2022] [Revised: 08/20/2022] [Accepted: 09/12/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Paragangliomas may be preoperatively misdiagnosed as non-functioning retroperitoneal tumors and are sometimes suspected only at the time of intraoperative manipulation. Without preoperative alpha blockade preparation, a hypertensive crisis during tumor manipulation and hypotension after tumor removal may result in critical consequences. Therefore, primary consideration should be given to the continuation or discontinuation of surgery on the basis of the possibility of gentle surgical manipulation and hemodynamic stabilization. We report two cases of paragangliomas detected intraoperatively.
CASE SUMMARY A 65-year-woman underwent laparoscopic small-bowel wedge resection. A hypertensive crisis occurred during manipulation of the mass, and an unrecognized catecholamine-producing paraganglioma was suspected. The surgeon and anesthesiologists believed that tumor excision could be performed with minimal manipulation of the tumor because the tumor was in a favorable location. Serious hemodynamic instability did not occur with aggressive use of vasoactive drugs. A week later, a 54-year-man underwent open resection of a 3-cm-sized retroperitoneal mass and showed the same findings during mass manipulation. For this patient, continuous manipulation of the mass seemed inevitable due to adhesion between the right adrenal gland and the mass in a narrow surgical field. The surgeon and anesthesiologists decided to cancel the surgical procedure and planned to perform a reoperation after alpha blockade therapy. Two weeks later, the tumor was uneventfully removed with small doses of vasoactive drugs.
CONCLUSION When an undiagnosed paraganglioma is suspected intraoperatively, reoperation after adequate preparation should be considered as an option to avoid fatal outcomes.
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Affiliation(s)
- Dongho Kang
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
| | - Bo-eun Kim
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
| | - Minjae Hong
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
| | - Joungmin Kim
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
| | - Seongtae Jeong
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
| | - Seongheon Lee
- Department of Anesthesiology and Pain Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju 61469, South Korea
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Dawud F, Takyi SA, Arko-Mensah J, Basu N, Egbi G, Ofori-Attah E, Bawuah SA, Fobil JN. Relationship between Metal Exposures, Dietary Macronutrient Intake, and Blood Glucose Levels of Informal Electronic Waste Recyclers in Ghana. INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH 2022; 19:12768. [PMID: 36232070 PMCID: PMC9564681 DOI: 10.3390/ijerph191912768] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/06/2022] [Revised: 09/29/2022] [Accepted: 10/01/2022] [Indexed: 06/16/2023]
Abstract
While metal exposures are generally high among informal electronic waste (e-waste) recyclers, the joint effect of metals and dietary macronutrients on their metabolic health is unknown. Therefore, we investigated the relationship between metal exposures, dietary macronutrients intake, and blood glucose levels of e-waste recyclers at Agbogbloshie using dietary information (48-h recall survey), blood metals (Pb & Cd), and HbA1C levels of 151 participants (100 e-waste recyclers and 51 controls from the Accra, Ghana) in March 2017. A linear regression model was used to estimate the joint relationship between metal exposures, dietary macronutrient intake, and blood glucose levels. Except for dietary proteins, both groups had macronutrient deficiencies. Diabetes prevalence was significantly higher among controls. Saturated fat, OMEGA-3, and cholesterol intake were associated with significant increases in blood glucose levels of recyclers. In a joint model, while 1 mg of cholesterol consumed was associated with a 0.7% increase in blood glucose, 1 g/L of Pb was found to significantly increase blood glucose levels by 0.9% among recyclers. Although the dietary consumption of cholesterol and fat was not high, it is still possible that exposure to Pb and Cd may still increase the risk of diabetes among both e-waste recyclers and the general population.
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Affiliation(s)
- Fayizatu Dawud
- School of Public Health, University of Ghana, Legon, Accra P.O. Box LG13, Ghana
| | - Sylvia Akpene Takyi
- School of Public Health, University of Ghana, Legon, Accra P.O. Box LG13, Ghana
| | - John Arko-Mensah
- School of Public Health, University of Ghana, Legon, Accra P.O. Box LG13, Ghana
| | | | - Godfred Egbi
- Noguchi Memorial Institute for Medical Research, University of Ghana, Legon, Accra P.O. Box LG 581, Ghana
| | - Ebenezer Ofori-Attah
- Noguchi Memorial Institute for Medical Research, University of Ghana, Legon, Accra P.O. Box LG 581, Ghana
| | - Serwaa Akoto Bawuah
- School of Public Health, University of Ghana, Legon, Accra P.O. Box LG13, Ghana
| | - Julius N. Fobil
- School of Public Health, University of Ghana, Legon, Accra P.O. Box LG13, Ghana
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De Leo A, Vara G, Paccapelo A, Balacchi C, Vicennati V, Tucci L, Pagotto U, Selva S, Ricci C, Alberici L, Minni F, Nanni C, Ambrosi F, Santini D, Golfieri R, Di Dalmazi G, Mosconi C. Computerized tomography texture analysis of pheochromocytoma: relationship with hormonal and histopathological data. J Endocrinol Invest 2022; 45:1935-1944. [PMID: 35680695 PMCID: PMC9463266 DOI: 10.1007/s40618-022-01826-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2021] [Accepted: 05/17/2022] [Indexed: 11/29/2022]
Abstract
OBJECTIVES Pheochromocytomas are rare tumors which can present with heterogeneous secretion profiles, clinical manifestations, and radiologic appearance. Under a histopathological point of view, they can be characterized as more or less aggressive with the Pheochromocytoma of the Adrenal gland Scaled Score (PASS) and the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) score. The aim of this study is to analyze the texture analysis characteristics of pheochromocytoma and identify whether the texture analysis can yield information aiding in the diagnosis and the characterization of those tumors. METHODS Radiological, biochemical, and histopathological data regarding 30 consecutive patients with histologically confirmed pheochromocytoma were analyzed. Images obtained in the unenhanced, late arterial, venous, and delayed phases were used for the texture analysis. RESULTS Urinary epinephrine and metanephrine levels showed a significant correlation (R2 = 0.946; R2 = 699) in the multivariate linear model with texture features, as well as Ki-67 (R2 = 0.397), PASS score (R2 = 0.182), GAPP score (R2 = 0.705), and cellularity showed a significant correlation (R2 = 0.389). The cluster analysis based on radiomic features resulted in 2 clusters, with significative differences in terms of systolic and diastolic blood pressure values at the time of diagnosis (p = 0.025), GAPP score (4 vs 6, p = 0.05), histological pattern (1-2, p = 0.039), and comedonecrosis (0% vs 50%, p = 0.013). CONCLUSION In conclusion, our study provides the proof of concept for the use of texture analysis on contrast-enhanced CT images as a noninvasive, quantitative tool for helping in the characterization of the clinical, biochemical, and histopathological features of pheochromocytoma.
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Affiliation(s)
- A De Leo
- Pathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum-University of Bologna, Bologna, Italy
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
| | - G Vara
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy.
| | - A Paccapelo
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
| | - C Balacchi
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
| | - V Vicennati
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Unit of Endocrinology and Diabetes Prevention and Care, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
| | - L Tucci
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Unit of Endocrinology and Diabetes Prevention and Care, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
| | - U Pagotto
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Unit of Endocrinology and Diabetes Prevention and Care, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
| | - S Selva
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Division of Pancreatic Surgery, Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - C Ricci
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Division of Pancreatic Surgery, Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - L Alberici
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Division of Pancreatic Surgery, Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - F Minni
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Division of Pancreatic Surgery, Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - C Nanni
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Nuclear Medicine Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - F Ambrosi
- Pathology Unit, Maggiore Hospital, Bologna, Italy
| | - D Santini
- Pathology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum-University of Bologna, Bologna, Italy
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
| | - R Golfieri
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
| | - G Di Dalmazi
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
- Unit of Endocrinology and Diabetes Prevention and Care, Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
| | - C Mosconi
- Diagnostic and Interventional Radiology Unit, Department of Diagnostic and Preventive Medicine, Via Albertoni 15, 40136, Bologna, Italy
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Hereditary and Sporadic Pheochromocytoma: Comparison of Imaging, Clinical, and Laboratory Features. AJR Am J Roentgenol 2022; 219:97-109. [PMID: 35080458 DOI: 10.2214/ajr.21.26918] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
BACKGROUND. A considerable fraction of pheochromocytomas initially suspected to be sporadic, whether or not symptomatic, are a result of germline mutations. OBJECTIVE. The purpose of this article is to compare imaging features between hereditary and sporadic pheochromocytomas. METHODS. This retrospective study included 71 patients (39 women, 32 men; median age, 48 years) who underwent adrenal pheochromocytoma resection from January 2002 to October 2021 after preoperative CT or MRI. Two radiologists independently reviewed examinations to assess features of the largest resected pheochromocytoma. Interreader agreement was assessed by prevalence-adjusted bias-adjusted kappa coefficients; a third radiologist resolved discrepancies for further analysis. Genetic testing was used to classify pheochromocytomas as hereditary or sporadic and to classify hereditary pheochromocytomas by germline mutation clusters. Symptoms associated with pheochromocytomas and preoperative biochemical laboratory values were recorded. Groups were compared using Kruskal-Wallis, Fisher exact, and chi-square tests, and false-discovery rate-adjusted p values were computed to account for multiple comparisons. RESULTS. Hereditary pheochromocytoma (n = 32), compared with sporadic pheochromocytoma (n = 39), was associated with younger median age (38 vs 52 years, p = .001) and smaller median size (24 vs 40 mm, p < .001). Interreader agreement for CT and MRI features, expressed as kappa, ranged from 0.44 to 1.00. Hereditary and sporadic pheochromocytoma showed no difference in frequency of calcifications, hemorrhage, cystic change/necrosis, or macroscopic fat on CT, or in frequency of hemorrhage, cystic change/necrosis, macroscopic fat, or microscopic fat on MRI (p > .05). When combining CT and MRI, cystic change/necrosis was observed in 35% of hereditary versus 67% of sporadic pheochromocytomas (p = .10). Hereditary pheochromocytoma, compared with sporadic, had lower frequency of symptoms (31% vs 74%; p = .004) and lower 24-hour urinary normetanephrines (1.1 vs 5.1 times upper limits of normal, p = .006). Among hereditary pheochromocytomas, cystic change/necrosis (when assessable on imaging) was present in 18% and 45% of those with cluster 1 (n = 11) and cluster 2 (n = 21) germ-line mutations, respectively. CONCLUSION. Hereditary pheochromocytomas, compared with sporadic, are detected at a younger age and smaller size, produce lower 24-hour urinary normetanephrines, are less often symptomatic, and may less frequently show cystic change/necrosis. CLINICAL IMPACT. Imaging findings may complement clinical and biochemical features in raising suspicion for a previously unsuspected germline mutation in patients with pheochromocytoma.
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Prinzi N, Corti F, Torchio M, Niger M, Antista M, Pagani F, Beninato T, Pulice I, Rossi RE, Coppa J, Cascella T, Giacomelli L, Di Bartolomeo M, Milione M, de Braud F, Pusceddu S. Metastatic pheochromocytomas and paragangliomas: where are we? TUMORI JOURNAL 2022; 108:526-540. [PMID: 35593402 DOI: 10.1177/03008916221078621] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Pheochromocytomas and paragangliomas (PPGLs) can metastasize in approximately 15-20% of cases. This review discusses the available evidence on the biology and treatment of metastatic PPGLs. Chemotherapy is the first-line treatment option for this evolving and symptomatic disease. In patients with high MIBG uptake and positive PETGa-68, radiometabolic treatment may be considered. The efficacy of sunitinib has been shown in observational studies, and pembrolizumab has been evaluated in phase II clinical studies, while other agents investigated in this setting are anti-angiogenic drugs cabozantinib, dovitinib, axitinib and lenvatinib. As these agents' efficacy and safety data, alone or in combination, are scant and based on few treated patients, enrollment in clinical trials is mandatory. Future therapeutic options may be represented by DNA repair system inhibitors (such as olaparib), HIF2 inhibitors and immunotherapy.
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Affiliation(s)
- Natalie Prinzi
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Francesca Corti
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Martina Torchio
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Monica Niger
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Maria Antista
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Filippo Pagani
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Teresa Beninato
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Iolanda Pulice
- Clinical Trial Center, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - Roberta Elisa Rossi
- Gastro-intestinal Surgery and Liver Transplantation Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.,Department of Pathophysiology and Organ Transplant, Università degli Studi di Milano, Milan, Italy
| | - Jorgelina Coppa
- Gastro-intestinal Surgery and Liver Transplantation Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Tommaso Cascella
- Radiology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | | | - Maria Di Bartolomeo
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
| | - Massimo Milione
- Diagnostic Pathology and Laboratory Medicine Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Filippo de Braud
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy.,Oncology and Hemato-Oncology Department, Università degli Studi di Milano, Milan, Italy
| | - Sara Pusceddu
- Department of Medical Oncology, Fondazione IRCCS Istituto Tumori Milano, Milan, Italy
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Olmos R, Mertens N, Vaidya A, Uslar T, Fernandez P, Guarda FJ, Zúñiga Á, San Francisco I, Huete A, Baudrand R. Discriminative Capacity of CT Volumetry to Identify Autonomous Cortisol Secretion in Incidental Adrenal Adenomas. J Clin Endocrinol Metab 2022; 107:e1946-e1953. [PMID: 35020922 PMCID: PMC9272424 DOI: 10.1210/clinem/dgac005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2021] [Indexed: 01/12/2023]
Abstract
CONTEXT Incidentally discovered adrenal adenomas are common. Assessment for possible autonomous cortisol excess (ACS) is warranted for all adrenal adenomas, given the association with increased cardiometabolic disease. OBJECTIVE To evaluate the discriminatory capacity of 3-dimensional volumetry on computed tomography (CT) to identify ACS. METHODS Two radiologists, blinded to hormonal levels, prospectively analyzed CT images of 149 adult patients with unilateral, incidentally discovered, adrenal adenomas. Diameter and volumetry of the adenoma, volumetry of the contralateral adrenal gland, and the adenoma volume-to-contralateral gland volume (AV/CV) ratio were measured. ACS was defined as cortisol ≥ 1.8 mcg/dL after 1-mg dexamethasone suppression test (DST) and a morning ACTH ≤ 15. pg/mL. RESULTS We observed that ACS was diagnosed in 35 (23.4%) patients. Cortisol post-DST was positively correlated with adenoma diameter and volume, and inversely correlated with contralateral adrenal gland volume. Cortisol post-DST was positively correlated with the AV/CV ratio (r = 0.46, P < 0.001) and ACTH was inversely correlated (r = -0.28, P < 0.001). The AV/CV ratio displayed the highest odds ratio (1.40; 95% CI, 1.18-1.65) and area under curve (0.91; 95% CI, 0.86-0.96) for predicting ACS. An AV/CV ratio ≥ 1 (48% of the cohort) had a sensitivity of 97% and a specificity of 70% to identify ACS. CONCLUSION CT volumetry of adrenal adenomas and contralateral adrenal glands has a high discriminatory capacity to identify ACS. The combination of this simple and low-cost radiological phenotyping can supplement biochemical testing to substantially improve the identification of ACS.
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Affiliation(s)
- Roberto Olmos
- Department of Endocrinology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
- Program for Adrenal Disorders, CETREN UC, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - Nicolás Mertens
- Department of Radiology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - Anand Vaidya
- Center for Adrenal Disorders, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA 02115, USA
| | - Thomas Uslar
- Department of Endocrinology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
- Program for Adrenal Disorders, CETREN UC, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - Paula Fernandez
- Program for Adrenal Disorders, CETREN UC, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - Francisco J Guarda
- Department of Endocrinology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - Álvaro Zúñiga
- Department of Urology, Pontificia Universidad Catolica de Chile, Santiago 8330077, Chile
| | - Ignacio San Francisco
- Department of Urology, Pontificia Universidad Catolica de Chile, Santiago 8330077, Chile
| | - Alvaro Huete
- Department of Radiology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
| | - René Baudrand
- Department of Endocrinology, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
- Program for Adrenal Disorders, CETREN UC, Pontificia Universidad Catolica de Chile, Santiago, 8330077, Chile
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Chee YJ, Teo CHY, Au RTM, Kon YC. Subclinical phaeochromocytoma: a diagnostic and management challenge. BMJ Case Rep 2022; 15:e248571. [PMID: 35428667 PMCID: PMC9014025 DOI: 10.1136/bcr-2021-248571] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/29/2022] [Indexed: 11/04/2022] Open
Abstract
There is a paradigm shift in the detection of phaeochromocytomas with more being identified as adrenal 'incidentalomas'. While majority of these individuals are asymptomatic, they are nevertheless at risk of subtle cardiovascular dysfunction and phaeochromocytoma crises. Therefore, early resection of phaeochromocytomas, even if subclinical, is recommended. However, the perioperative management can be challenging as the normotension can limit the initiation and titration of alpha-blockade. We present a man in his 60s with a subclinical phaeochromocytoma, discuss the evaluation of an incidentally discovered adrenal nodule, as well as the practical considerations in the perioperative management.
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Affiliation(s)
- Ying Jie Chee
- Department of Endocrinology, Tan Tock Seng Hospital, Singapore, Singapore
| | | | | | - Yin Chian Kon
- Department of Endocrinology, Tan Tock Seng Hospital, Singapore, Singapore
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Role of 18F-FDG PET/CT in management of adrenocortical carcinoma: a comprehensive review of the literature. Clin Transl Imaging 2022. [DOI: 10.1007/s40336-022-00485-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
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Araujo-Castro M, García Centeno R, Robles Lázaro C, Parra Ramírez P, Gracia Gimeno P, Rojas-Marcos PM, Fernández-Ladreda MT, Percovich Hualpa JC, Sampedro Núñez M, López-García MC, Lamas C, Álvarez Escolá C, Calatayud Gutiérrez M, Blanco Carrera C, de Miguel Novoa P, Valdés Gallego N, Hanzu F, Marazuela M, Mora Porta M, Mínguez Ojeda C, García Gómez Muriel I, Escobar-Morreale HF, Valderrabano P. Predictive model of pheochromocytoma based on the imaging features of the adrenal tumours. Sci Rep 2022; 12:2671. [PMID: 35177692 PMCID: PMC8854552 DOI: 10.1038/s41598-022-06655-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/28/2021] [Accepted: 02/03/2022] [Indexed: 12/18/2022] Open
Abstract
The purpose of our study was to develop a predictive model to rule out pheochromocytoma among adrenal tumours, based on unenhanced computed tomography (CT) and/or magnetic resonance imaging (MRI) features. We performed a retrospective multicentre study of 1131 patients presenting with adrenal lesions including 163 subjects with histological confirmation of pheochromocytoma (PHEO), and 968 patients showing no clinical suspicion of pheochromocytoma in whom plasma and/or urinary metanephrines and/or catecholamines were within reference ranges (non-PHEO). We found that tumour size was significantly larger in PHEO than non-PHEO lesions (44.3 ± 33.2 versus 20.6 ± 9.2 mm respectively; P < 0.001). Mean unenhanced CT attenuation was higher in PHEO (52.4 ± 43.1 versus 4.7 ± 17.9HU; P < 0.001). High lipid content in CT was more frequent among non-PHEO (83.6% versus 3.8% respectively; P < 0.001); and this feature alone had 83.6% sensitivity and 96.2% specificity to rule out pheochromocytoma with an area under the receiver operating characteristics curve (AUC-ROC) of 0.899. The combination of high lipid content and tumour size improved the diagnostic accuracy (AUC-ROC 0.961, sensitivity 88.1% and specificity 92.3%). The probability of having a pheochromocytoma was 0.1% for adrenal lesions smaller than 20 mm showing high lipid content in CT. Ninety percent of non-PHEO presented loss of signal in the “out of phase” MRI sequence compared to 39.0% of PHEO (P < 0.001), but the specificity of this feature for the diagnosis of non-PHEO lesions low. In conclusion, our study suggests that sparing biochemical screening for pheochromocytoma might be reasonable in patients with adrenal lesions smaller than 20 mm showing high lipid content in the CT scan, if there are no typical signs and symptoms of pheochromocytoma.
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Affiliation(s)
- Marta Araujo-Castro
- Department of Endocrinology & Nutrition, Hospital Universitario Ramón y Cajal, Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Universidad de Alcalá, Madrid, Spain.
| | - Rogelio García Centeno
- Department of Endocrinology & Nutrition, Hospital Universitario Gregorio Marañón, Madrid, Spain
| | - Cristina Robles Lázaro
- Department of Endocrinology & Nutrition, Hospital Universitario Virgen de La Concha, Zamora, Spain
| | - Paola Parra Ramírez
- Department of Endocrinology & Nutrition, Hospital Universitario La Paz, Madrid, Spain
| | - Paola Gracia Gimeno
- Department of Endocrinology & Nutrition, Hospital Universitario Rollo Villanova, Zaragoza, Spain
| | | | | | | | - Miguel Sampedro Núñez
- Department of Endocrinology & Nutrition, Hospital Universitario La Princesa, Madrid, Spain
| | - María-Carmen López-García
- Department of Endocrinology & Nutrition, Complejo Hospitalario Universitario de Albacete, Albacete, Spain
| | - Cristina Lamas
- Department of Endocrinology & Nutrition, Complejo Hospitalario Universitario de Albacete, Albacete, Spain
| | | | | | | | - Paz de Miguel Novoa
- Department of Endocrinology & Nutrition, Hospital Universitario Clínico San Carlos, Madrid, Spain
| | - Nuria Valdés Gallego
- Department of Endocrinology & Nutrition, Hospital Universitario de Cabueñes, Asturias, Spain
| | - Felicia Hanzu
- Department of Endocrinology & Nutrition, Hospital Clinic, Barcelona, Spain
| | - Mónica Marazuela
- Department of Endocrinology & Nutrition, Hospital Universitario La Princesa, Madrid, Spain
| | - Mireia Mora Porta
- Department of Endocrinology & Nutrition, Hospital Clinic, Barcelona, Spain
| | | | | | - Héctor F Escobar-Morreale
- Department of Endocrinology & Nutrition, Hospital Universitario Ramón y Cajal, Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Universidad de Alcalá, Madrid, Spain
| | - Pablo Valderrabano
- Department of Endocrinology & Nutrition, Hospital Universitario Ramón y Cajal, Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Universidad de Alcalá, Madrid, Spain.
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Berke K, Constantinescu G, Masjkur J, Kimpel O, Dischinger U, Peitzsch M, Kwapiszewska A, Dobrowolski P, Nölting S, Reincke M, Beuschlein F, Bornstein SR, Prejbisz A, Lenders JWM, Fassnacht M, Eisenhofer G. Plasma Steroid Profiling in Patients With Adrenal Incidentaloma. J Clin Endocrinol Metab 2022; 107:e1181-e1192. [PMID: 34665854 DOI: 10.1210/clinem/dgab751] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Indexed: 01/13/2023]
Abstract
CONTEXT Most patients with adrenal incidentaloma have nonfunctional lesions that do not require treatment, while others have functional or malignant tumors that require intervention. The plasma steroid metabolome may be useful to assess therapeutic need. OBJECTIVE This work aimed to establish the utility of plasma steroid profiling combined with metanephrines and adrenal tumor size for the differential diagnosis of patients with adrenal incidentaloma. METHODS This retrospective cross-sectional study, which took place at 7 European tertiary-care centers, comprised 577 patients with adrenal incidentaloma, including 19, 77, 65, 104 and 312 respective patients with adrenocortical carcinoma (ACC), pheochromocytoma (PHEO), primary aldosteronism (PA), autonomous cortisol secretion (ACS), and nonfunctional adrenal incidentaloma (NFAI). Mesaures of diagnostic performance were assessed (with [95% CIs]) for discriminating different subgroups of patients with adrenal incidentaloma. RESULTS Patients with ACC were characterized by elevated plasma concentrations of 11-deoxycortisol, 11-deoxycorticosterone, 17-hydroxyprogesterone, androstenedione, and dehydroepiandrosterone-sulfate, whereas patients with PA had elevations of aldosterone, 18-oxocortisol, and 18-hydroxycortisol. A selection of those 8 steroids, combined with 3 others (cortisol, corticosterone, and dehydroepiandrosterone) and plasma metanephrines, proved optimal for identifying patients with ACC, PA, and PHEO at respective sensitivities of 83.3% (66.1%-100%), 90.8% (83.7%-97.8%), and 94.8% (89.8%-99.8%); and specificities of 98.0% (96.9%-99.2%), 92.0% (89.6%-94.3%), and 98.6% (97.6%-99.6%). With the addition of tumor size, discrimination improved further, particularly for ACC (100% [100%-100%] sensitivity, 99.5% [98.9%-100%] specificity). In contrast, discrimination of ACS and NFAI remained suboptimal (70%-71% sensitivity, 89%-90% specificity). CONCLUSION Among patients with adrenal incidentaloma, the combination of plasma steroid metabolomics with routinely available plasma free metanephrines and data from imaging studies may facilitate the identification of almost all clinically relevant adrenal tumors.
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Affiliation(s)
- Kristina Berke
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
| | - Georgiana Constantinescu
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
| | - Jimmy Masjkur
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
| | - Otilia Kimpel
- Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, 97082 Würzburg, Germany
| | - Ulrich Dischinger
- Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, 97082 Würzburg, Germany
| | - Mirko Peitzsch
- Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
| | | | - Piotr Dobrowolski
- Department of Hypertension, National Institute of Cardiology, 04-828 Warsaw, Poland
| | - Svenja Nölting
- Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), 8057 Zurich, Switzerland
- Department of Medicine IV, University Hospital, Ludwig Maximilian University of Munich, 80539 Munich, Germany
| | - Martin Reincke
- Department of Medicine IV, University Hospital, Ludwig Maximilian University of Munich, 80539 Munich, Germany
| | - Felix Beuschlein
- Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), 8057 Zurich, Switzerland
- Department of Medicine IV, University Hospital, Ludwig Maximilian University of Munich, 80539 Munich, Germany
| | - Stefan R Bornstein
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
| | - Aleksander Prejbisz
- Department of Hypertension, National Institute of Cardiology, 04-828 Warsaw, Poland
| | - Jacques W M Lenders
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
- Department of Internal Medicine, Radboud University Medical Center, 6500 HB Nijmegen, the Netherlands
| | - Martin Fassnacht
- Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, 97082 Würzburg, Germany
| | - Graeme Eisenhofer
- Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
- Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany
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Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance. Cancers (Basel) 2022; 14:cancers14040917. [PMID: 35205664 PMCID: PMC8869962 DOI: 10.3390/cancers14040917] [Citation(s) in RCA: 22] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2021] [Revised: 02/02/2022] [Accepted: 02/08/2022] [Indexed: 02/07/2023] Open
Abstract
Simple Summary Pheochromocytomas and abdominal paragangliomas (PPGLs) are rare. They can be discovered incidentally by imaging with computed tomography or magnetic resonance imaging and during hormonal surveillance in patients with known genetic variants that are associated with PPGLs. As most PPGLs are functioning, a hormonal work-up evaluating for catecholamine excess is recommended. Classical symptoms, such as tachycardia, hypertension and headache, can be present, but when the PPGL is discovered as an incidentaloma, symptoms may be lacking or be more discrete. PPGLs carry malignant potential, and patients should undergo close surveillance, as recurrence of disease or metastasis may develop. Genetic susceptibility for multifocal disease has gained more attention, and germline variants are commonly detected, thus facilitating detection of hereditary cases and afflicted family members. Any patient with a PPGL should be managed by an expert multidisciplinary team consisting of endocrinologists, radiologists, surgeons, pathologists and clinical geneticists. Abstract Pheochromocytomas and abdominal paragangliomas (PPGLs) are rare tumors arising from the adrenal medulla or the sympathetic nervous system. This review presents a practical guidance for clinicians dealing with PPGLs. The incidence of PPGLs has risen. Most cases are detected via imaging and less present with symptoms of catecholamine excess. Most PPGLs secrete catecholamines, with diffuse symptoms. Diagnosis is made by imaging and tests of catecholamines. Localized disease can be cured by surgery. PPGLs are the most heritable of all human tumors, and germline variants are found in approximately 30–50% of cases. Such variants can give information regarding the risk of developing recurrence or metastases as well as the risk of developing other tumors and may identify relatives at risk for disease. All PPGLs harbor malignant potential, and current histological and immunohistochemical algorithms can aid in the identification of indolent vs. aggressive tumors. While most patients with metastatic PPGL have slowly progressive disease, a proportion of patients present with an aggressive course, highlighting the need for more effective therapies in these cases. We conclude that PPGLs are rare but increasing in incidence and management should be guided by a multidisciplinary team.
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How to Explore an Endocrine Cause of Hypertension. J Clin Med 2022; 11:jcm11020420. [PMID: 35054115 PMCID: PMC8780426 DOI: 10.3390/jcm11020420] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/24/2021] [Revised: 01/04/2022] [Accepted: 01/07/2022] [Indexed: 12/13/2022] Open
Abstract
Hypertension (HTN) is the most frequent modifiable risk factor in the world, affecting almost 30 to 40% of the adult population in the world. Among hypertensive patients, 10 to 15% have so-called “secondary” HTN, which means HTN due to an identified cause. The most frequent secondary causes of HTN are renal arteries abnormalities (renovascular HTN), kidney disease, and endocrine HTN, which are primarily due to adrenal causes. Knowing how to detect and explore endocrine causes of hypertension is particularly interesting because some causes have a cure or a specific treatment available. Moreover, the delayed diagnosis of secondary HTN is a major cause of uncontrolled blood pressure. Therefore, screening and exploration of patients at risk for secondary HTN should be a serious concern for every physician seeing patients with HTN. Regarding endocrine causes of HTN, the most frequent is primary aldosteronism (PA), which also is the most frequent cause of secondary HTN and could represent 10% of all HTN patients. Cushing syndrome and pheochromocytoma and paraganglioma (PPGL) are rarer (less than 0.5% of patients). In this review, among endocrine causes of HTN, we will mainly discuss explorations for PA and PPGL.
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Liu H, Guan X, Xu B, Zeng F, Chen C, Yin HL, Yi X, Peng Y, Chen BT. Computed Tomography-Based Machine Learning Differentiates Adrenal Pheochromocytoma From Lipid-Poor Adenoma. Front Endocrinol (Lausanne) 2022; 13:833413. [PMID: 35388295 PMCID: PMC8977471 DOI: 10.3389/fendo.2022.833413] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2021] [Accepted: 02/24/2022] [Indexed: 12/04/2022] Open
Abstract
OBJECTIVES To assess the accuracy of computed tomography (CT)-based machine learning models for differentiating subclinical pheochromocytoma (sPHEO) from lipid-poor adenoma (LPA) in patients with adrenal incidentalomas. PATIENTS AND METHODS The study included 188 tumors in the 183 patients with LPA and 92 tumors in 86 patients with sPHEO. Pre-enhanced CT imaging features of the tumors were evaluated. Machine learning prediction models and scoring systems for differentiating sPHEO from LPA were built using logistic regression (LR), support vector machine (SVM) and random forest (RF) approaches. RESULTS The LR model performed better than other models. The LR model (M1) including three CT features: CTpre value, shape, and necrosis/cystic changes had an area under the receiver operating characteristic curve (AUC) of 0.917 and an accuracy of 0.864. The LR model (M2) including three CT features: CTpre value, shape and homogeneity had an AUC of 0.888 and an accuracy of 0.832. The S2 scoring system (sensitivity: 0.859, specificity: 0.824) had comparable diagnostic value to S1 (sensitivity: 0.815; specificity: 0.910). CONCLUSIONS Our results indicated the potential of using a non-invasive imaging method such as CT-based machine learning models and scoring systems for predicting histology of adrenal incidentalomas. This approach may assist the diagnosis and personalized care of patients with adrenal tumors.
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Affiliation(s)
- Haipeng Liu
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, China
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Changsha, China
| | - Xiao Guan
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Changsha, China
- Department of Urology, Xiangya Hospital, Central South University, Changsha, China
| | - Beibei Xu
- College of Biology, Hunan University, Changsha, China
| | - Feiyue Zeng
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, China
| | - Changyong Chen
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, China
| | - Hong ling Yin
- Department of Pathology, Xiangya Hospital, Central South University, Changsha, China
| | - Xiaoping Yi
- Department of Radiology, Xiangya Hospital, Central South University, Changsha, China
- National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Changsha, China
- Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Changsha, China
- Hunan Key Laboratory of Skin Cancer and Psoriasis, Xiangya Hospital, Changsha, China
- *Correspondence: Xiaoping Yi, ; Yousong Peng,
| | - Yousong Peng
- College of Biology, Hunan University, Changsha, China
- *Correspondence: Xiaoping Yi, ; Yousong Peng,
| | - Bihong T. Chen
- Department of Diagnostic Radiology, City of Hope National Medical Center, Los Angeles, CA, United States
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Niu Z, Wang J, Yang Y, He J, Wang S, Xie Z, Shao M, Zhu F. Risk prediction model establishment with tri-phasic CT image features for differential diagnosis of adrenal pheochromocytomas and lipid-poor adenomas: Grouping method. Front Endocrinol (Lausanne) 2022; 13:925577. [PMID: 36568104 PMCID: PMC9772429 DOI: 10.3389/fendo.2022.925577] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/21/2022] [Accepted: 11/22/2022] [Indexed: 12/13/2022] Open
Abstract
OBJECTIVES The purpose of this study was to establish a risk prediction model for differential diagnosis of pheochromocytomas (PCCs) from lipid-poor adenomas (LPAs) using a grouping method based on tri-phasic CT image features. METHODS In this retrospective study, we enrolled patients that were assigned to a training set (136 PCCs and 183 LPAs) from two medical centers, along with an external independent validation set (30 PCCs and 54 LPAs) from another center. According to the attenuation values in unenhanced CT (CTu), the lesions were divided into three groups: group 1, 10 HU < CTu ≤ 25 HU; group 2, 25 HU < CTu ≤ 40 HU; and group 3, CTu > 40 HU. Quantitative and qualitative CT imaging features were calculated and evaluated. Univariate, ROC, and binary logistic regression analyses were applied to compare these features. RESULTS Cystic degeneration, CTu, and the peak value of enhancement in the arterial and venous phase (DEpeak) were independent risk factors for differential diagnosis of adrenal PCCs from LPAs. In all subjects (groups 1, 2, and 3), the model formula for the differentiation of PCCs was as follows: Y = -7.709 + 3.617*(cystic degeneration) + 0.175*(CTu ≥ 35.55 HU) + 0.068*(DEpeak ≥ 51.35 HU). ROC curves were drawn with an AUC of 0.95 (95% CI: 0.927-0.973) in the training set and 0.91 (95% CI: 0.860-0.929) in the external validation set. CONCLUSION A reliable and practical prediction model for differential diagnosis of adrenal PCCs and LPAs was established using a grouping method.
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Affiliation(s)
- Zhongfeng Niu
- Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
| | - Jian Wang
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang, China
| | - Yang Yang
- Department of Radiology, The First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui, China
| | - Jie He
- Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
| | - Subo Wang
- Department of Radiology, Shaoxing Hospital of Traditional Chinese Medicine, Shaoxing, Zhejiang, China
| | - Zongyu Xie
- Department of Radiology, The First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui, China
| | - Meihua Shao
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang, China
| | - Fangmei Zhu
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang, China
- *Correspondence: Fangmei Zhu,
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Shah AN, Saikia UK, Chaudhary BK, Bhuyan AK. Adrenal Incidentaloma Needs thorough Biochemical Evaluation - An Institutional Experience. Indian J Endocrinol Metab 2022; 26:73-78. [PMID: 35662767 PMCID: PMC9162248 DOI: 10.4103/ijem.ijem_335_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Revised: 01/09/2022] [Accepted: 03/22/2022] [Indexed: 11/29/2022] Open
Abstract
CONTEXT Adrenal incidentalomas (AIs) are seen in around 2% of apparently healthy individuals. These require careful evaluation for the hormone excess state and the presence of malignancy prior to intervention. AIMS To study the clinical, biochemical, and imaging characteristics of the patients with AI and correlate the diagnosis with the histopathology findings in patients undergoing surgery. SETTINGS AND DESIGN Retrospective observational study. METHODS AND MATERIAL Patients with adrenal incidentaloma presenting between January 2017 and January 2021 were evaluated as per guidelines provided by the European Society of Endocrinology and the European Network for the Study of Adrenal Tumors. Patients were given final diagnosis on the basis of imaging impression, hormonal activity, and biopsy results (when applicable). RESULTS Forty-eight patients were evaluated, with 25 being male, the mean age being 40.9 years (8-71), and the mean size of the mass being 6.21 (1.4-13.7) cm. Thirty-five (72.9%) of them underwent surgical excision. The most common diagnosis was myelolipoma (16), followed by pheochromocytoma (10) and adenoma (9). Nineteen patients were found to have hormone-secreting masses. Two patients with pheochromocytoma were normotensive. There was discordance between imaging diagnosis and hormonal status in two patients, with final diagnosis of pheochromocytoma. One patient with extramedullary erythropoiesis of the adrenal gland was subsequently diagnosed with sickle cell anemia and adrenal insufficiency. CONCLUSIONS The study highlights the rare possibility of discrepancy between non-contrast CT diagnosis and functional status of AI. There is also a rare possibility of extramedullary erythropoiesis presenting as AI with adrenal insufficiency. Specific evaluation for such rare possibilities should be considered in AI cases as per clinical scenario.
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Affiliation(s)
- Akash N. Shah
- Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, Assam, India
| | - Uma K. Saikia
- Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, Assam, India
| | - Bipul K. Chaudhary
- Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, Assam, India
| | - Ashok K. Bhuyan
- Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, Assam, India
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Schloetelburg W, Ebert I, Petritsch B, Weng AM, Dischinger U, Kircher S, Buck AK, Bley TA, Deutschbein T, Fassnacht M. Adrenal wash-out CT: moderate diagnostic value in distinguishing benign from malignant adrenal masses. Eur J Endocrinol 2021; 186:183-193. [PMID: 34813495 PMCID: PMC8679842 DOI: 10.1530/eje-21-0650] [Citation(s) in RCA: 26] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2021] [Accepted: 11/23/2021] [Indexed: 01/26/2023]
Abstract
OBJECTIVE Reliable results of wash-out CT in the diagnostic workup of adrenal incidentalomas are scarce. Thus, we evaluated the diagnostic accuracy of delayed wash-out CT and determined thresholds to accurately differentiate adrenal masses. DESIGN Retrospective, single-center cohort study including 216 patients with 252 adrenal lesions who underwent delayed wash-out CT. Definitive diagnoses based on histopathology (n = 92) or comprehensive follow-up. METHODS Size, average attenuation values of the adrenal lesions in all CT scan phases, and absolute and relative percentage wash-out (APW/RPW) were determined by an expert radiologist blinded for clinical data. Adrenal lesions with unenhanced attenuation values >10 Hounsfield units (HU) built a subgroup (n = 142). Diagnostic accuracy was calculated. RESULTS The study group consisted of 171 adenomas, 32 other benign tumors, 11 pheochromocytomas, 9 adrenocortical carcinomas, and 29 other malignant tumors. All (potentially) malignant and 46% of benign lesions showed unenhanced attenuation values >10 HU. In this most relevant subgroup, the established thresholds of 60% for APW and 40% for RPW misclassified 35.9 and 35.2% of the masses, respectively. When we applied optimized cutoffs (APW >83%; RPW >58%) and excluded pheochromocytomas, we missed only one malignant tumor by APW and none by RPW. However, only 11 and 15% of the benign tumors were correctly identified. CONCLUSIONS Wash-out CT with the established thresholds for APW and RPW is insufficient to reliably diagnose adrenal masses. Using the proposed cutoff of 58% for RPW, malignant tumors will be correctly identified, but the added value is limited, namely 15% of patients with benign tumors can be prevented from additional imaging or even unnecessary surgery.
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Affiliation(s)
- Wiebke Schloetelburg
- Department of Radiology, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
- Department of Nuclear Medicine, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Ines Ebert
- Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Bernhard Petritsch
- Department of Radiology, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Andreas Max Weng
- Department of Radiology, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Ulrich Dischinger
- Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Stefan Kircher
- Institute of Pathology, University of Würzburg, Würzburg, Germany
| | - Andreas Konrad Buck
- Department of Nuclear Medicine, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Thorsten Alexander Bley
- Department of Radiology, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
| | - Timo Deutschbein
- Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
- Medicover Oldenburg MVZ, Oldenburg, Germany
| | - Martin Fassnacht
- Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
- Correspondence should be addressed to M Fassnacht;
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Araujo-Castro M, Pascual-Corrales E, Nattero Chavez L, Martínez Lorca A, Alonso-Gordoa T, Molina-Cerrillo J, Lorca Álvaro J, Mínguez Ojeda C, Redondo López S, Barberá Durbán R, Polo López R, Moreno Mata N, Caballero Silva U, Pian H, Ruz-Caracuel I, Sanjuanbenito Dehesa A, Gómez Dos Santos V, Serrano Romero AB. Protocol for presurgical and anesthetic management of pheochromocytomas and sympathetic paragangliomas: a multidisciplinary approach. J Endocrinol Invest 2021; 44:2545-2555. [PMID: 34304388 DOI: 10.1007/s40618-021-01649-7] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Accepted: 07/21/2021] [Indexed: 02/07/2023]
Abstract
OBJECTIVE To offer a practical guide for the presurgical and anesthetic management of pheochromocytomas and sympathetic paragangliomas (PGLs). METHODS This protocol was based on a comprehensive review of the literature and on our own multidisciplinary team's experience from managing pheochromocytoma and sympathetic PGLs at a referral center. RESULTS Patients with pheochromocytomas and sympathetic paragangliomas (PGLs) may develop potentially life-threatening complications, especially during surgical procedures. A complete biochemical, radiological, genetic, and cardiological assessment is recommended in the preoperative stage as it provides an evaluation of the risk of surgical complications and malignancy, allowing individualization of the presurgical treatment. Treatment with α-blockade and proper volume expansion in the preoperative stage significantly reduces the perioperative morbidity. During surgery, the anesthesiologist should look for a deep anesthetic level that inhibits the cardiovascular effects of catecholamines to minimize the risk of intraoperative complications. CONCLUSIONS An optimal presurgical evaluation of pheochromocytomas/ sympathetic PGL requires a multidisciplinary approach, including a complete hormonal, radiological, cardiac, genetic, and functioning evaluation in most cases. A proper preoperative evaluation in combination with strict blood pressure and heart rate control, and blood volume status optimization, will significantly reduce the risk of intraoperative and perioperative complications. In those patients who unfortunately develop intraoperative complications, the role of the anesthesiologist is essential since the selection of the appropriate management has a direct impact on morbimortality reduction.
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Affiliation(s)
- M Araujo-Castro
- Neuroendocrinology Division, Department of Endocrinology and Nutrition, IRYCIS, Hospital Universitario Ramón y Cajal, Colmenar Viejo street, S/N, 28034, Madrid, Spain.
| | - E Pascual-Corrales
- Neuroendocrinology Division, Department of Endocrinology and Nutrition, IRYCIS, Hospital Universitario Ramón y Cajal, Colmenar Viejo street, S/N, 28034, Madrid, Spain
| | - L Nattero Chavez
- Neuroendocrinology Division, Department of Endocrinology and Nutrition, IRYCIS, Hospital Universitario Ramón y Cajal, Colmenar Viejo street, S/N, 28034, Madrid, Spain
| | - A Martínez Lorca
- Department of Nuclear Medicine, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - T Alonso-Gordoa
- Department of Medical Oncology, IRYCIS, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - J Molina-Cerrillo
- Department of Medical Oncology, IRYCIS, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - J Lorca Álvaro
- Department of Urology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - C Mínguez Ojeda
- Department of Urology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - S Redondo López
- Department of Vascular Surgery, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - R Barberá Durbán
- Department of Otorhinolaryngology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - R Polo López
- Department of Otorhinolaryngology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - N Moreno Mata
- Department of Thoracic Surgery, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - U Caballero Silva
- Department of Thoracic Surgery, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - H Pian
- Department of Pathology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - I Ruz-Caracuel
- Department of Pathology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - A Sanjuanbenito Dehesa
- Department of General and Digestive Surgery, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - V Gómez Dos Santos
- Department of Urology, Hospital Universitario Ramón y Cajal, Madrid, Spain
| | - A B Serrano Romero
- Department of Anesthesia, Hospital Universitario Ramón y Cajal, Madrid, Spain
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Ceccato F, Barbot M, Scaroni C, Boscaro M. Frequently asked questions and answers (if any) in patients with adrenal incidentaloma. J Endocrinol Invest 2021; 44:2749-2763. [PMID: 34160793 PMCID: PMC8572215 DOI: 10.1007/s40618-021-01615-3] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2020] [Accepted: 06/14/2021] [Indexed: 12/20/2022]
Abstract
PURPOSE Adrenal incidentalomas (AIs) are incidentally discovered adrenal masses, during an imaging study undertaken for other reasons than the suspicion of adrenal disease. Their management is not a minor concern for patients and health-care related costs, since their increasing prevalence in the aging population. The exclusion of malignancy is the first question to attempt, then a careful evaluation of adrenal hormones is suggested. Surgery should be considered in case of overt secretion (primary aldosteronism, adrenal Cushing's Syndrome or pheochromocytoma), however the management of subclinical secretion is still a matter of debate. METHODS The aim of the present narrative review is to offer a practical guidance regarding the management of AI, by providing evidence-based answers to frequently asked questions. CONCLUSION The clinical experience is of utmost importance: a personalized diagnostic-therapeutic approach, based upon multidisciplinary discussion, is suggested.
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Affiliation(s)
- F Ceccato
- Endocrinology Unit, Department of Medicine DIMED, University of Padova, Via Ospedale Civile, 105-35128, Padova, Italy.
- Endocrine Disease Unit, University-Hospital of Padova, Padova, Italy.
- Department of Neuroscience DNS, University of Padova, Padova, Italy.
| | - M Barbot
- Endocrinology Unit, Department of Medicine DIMED, University of Padova, Via Ospedale Civile, 105-35128, Padova, Italy
- Endocrine Disease Unit, University-Hospital of Padova, Padova, Italy
| | - C Scaroni
- Endocrinology Unit, Department of Medicine DIMED, University of Padova, Via Ospedale Civile, 105-35128, Padova, Italy
- Endocrine Disease Unit, University-Hospital of Padova, Padova, Italy
| | - M Boscaro
- Endocrinology Unit, Department of Medicine DIMED, University of Padova, Via Ospedale Civile, 105-35128, Padova, Italy
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Abstract
Incidental findings are common in the evaluation of surgical patients. Understanding the appropriate assessment and management of these frequent occurrences is important for the provision of comprehensive quality care. This review details the epidemiology, considerations, and recommendations for management of common incidental manifestations in surgical patients, including Meckel diverticulum, adrenal incidentaloma, thyroid nodule, solitary pulmonary nodule, small bowel intussusception, gallstones, and incidental appendectomy.
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Affiliation(s)
- Keely Reidelberger
- University of Nebraska Medical Center College of Medicine, 986880 Nebraska Medical Center, Omaha, NE 68198-6880, USA
| | - Abbey Fingeret
- Department of Surgery, Division of Surgical Oncology, University of Nebraska Medical Center, 986880 Nebraska Medical Center, Omaha, NE 68198-6880, USA.
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48
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Johns AM, Vuong AH, Hassan OA, Randle RW, Gorris MA. Multiple endocrine neoplasia 2A presenting in a family with a history of Hirschprung's disease. Oxf Med Case Reports 2021; 2021:omab122. [PMID: 34987852 PMCID: PMC8713586 DOI: 10.1093/omcr/omab122] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2021] [Revised: 10/12/2021] [Accepted: 11/03/2021] [Indexed: 11/18/2022] Open
Abstract
Hirschprung's disease co-occurs with multiple endocrine neoplasia type 2A infrequently but at a higher rate with certain RET mutations. We present a case of a patient evaluated for an adrenal incidentaloma with a history of familial Hirschprung's. Our patient was found to have synchronous pheochromocytoma and medullary thyroid carcinoma illustrating the importance of genetic testing in these patients to determine appropriate screening for endocrine tumors.
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Affiliation(s)
- Allison M Johns
- Division of Endocrinology and Metabolism, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA
| | - Angela H Vuong
- Department of Internal Medicine, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA
| | - Omer A Hassan
- Department of Pathology, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA
| | - Reese W Randle
- Department of General Surgery, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA
| | - Matthew A Gorris
- Division of Endocrinology and Metabolism, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA
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Abstract
Adrenal masses are frequently incidentally identified from cross-sectional imaging studies, which are performed for other reasons. The intensity of the approach to the patient with such a mass is tailored to the clinical situation, ranging from a quick evaluation to a detailed work-up. In all cases, the three components of the evaluation are clinical assessment, review of the images, and biochemical testing with the goal of ruling out malignancy and identifying hormonally active lesions. This article incorporates recent information to produce a logical, systematic assessment of these patients with risk stratification and proportionate follow-up.
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Affiliation(s)
- Xin He
- Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA
| | - Patricia R Peter
- Section of Endocrinology, Department of Internal Medicine, Yale School of Medicine, 333 Cedar Street, FMP 110, PO Box 208020, New Haven, CT 06520, USA
| | - Richard J Auchus
- Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA; Department of Pharmacology, University of Michigan, Ann Arbor, MI, USA; Ann Arbor Veterans Affairs Medical Center, Ann Arbor, MI, USA.
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50
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Badawy M, Gaballah AH, Ganeshan D, Abdelalziz A, Remer EM, Alsabbagh M, Westphalen A, Siddiqui MA, Taffel MT, Itani M, Shaaban AM, Elsayes KM. Adrenal hemorrhage and hemorrhagic masses; diagnostic workup and imaging findings. Br J Radiol 2021; 94:20210753. [PMID: 34464549 DOI: 10.1259/bjr.20210753] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Adrenal hemorrhage (AH) is a rare condition. It can be traumatic or non-traumatic. Most common causes are septicemia, coagulopathy or bleeding diathesis, and underlying neoplasms. Other reported less common causes of AH are COVID-19 and neonatal stress. Clinical diagnosis of AH is challenging due to its non-specific presentation and occurrence in the setting of acute medical illness. Therefore, most cases are diagnosed incidentally on imaging. Having high clinical suspicion in the proper clinical setting for AH is crucial to avoid life-threatening adrenal insufficiency that occurs in 16-50% of patients with bilateral AH. We discuss the clinical situations that predispose to AH, review the imaging features on different imaging modalities, highlight a variety of clinical cases, imaging features that should be concerning for an underlying neoplasm, and outline the potential role of interventional radiology in management of AH.
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Affiliation(s)
- Mohamed Badawy
- Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, TX, United States
| | - Ayman H Gaballah
- Department of Radiology, University of Missouri Health care, Columbia, MO, United States
| | | | - Amr Abdelalziz
- Department of Radiology, University of Missouri Health care, Columbia, MO, United States
| | - Erick M Remer
- Department of Radiology, Cleveland Clinic Foundation, Cleveland, OH, United States
| | - Mustafa Alsabbagh
- Department of Radiology, University of Missouri Health care, Columbia, MO, United States
| | - Antonio Westphalen
- Department of Radiology, University of Washington, Seattle, WA, United States
| | - Mohammed A Siddiqui
- Department of Radiology, University of Missouri Health care, Columbia, MO, United States
| | - Myles T Taffel
- Department of Radiology, NYU Langone Medical Center, New York, NY, United States
| | - Malak Itani
- Department of Radiology, University of Washington, Seattle, WA, United States
| | - Akram M Shaaban
- Department of Diagnostic Imaging, University of Utah, Salt Lake City, UT, United States
| | - Khaled M Elsayes
- Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, TX, United States
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