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Kardalas E, Kyriakopoulos G, Antonopoulou V, Kyriakou A, Georgiadi S, Tzanela M, Ntali G. The clinical spectrum of adrenal ganglioneuromas extends from severe hypertension to an asymptomatic incidentaloma; two cases and mini review of literature. Hormones (Athens) 2025:10.1007/s42000-025-00676-w. [PMID: 40434716 DOI: 10.1007/s42000-025-00676-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2025] [Accepted: 05/16/2025] [Indexed: 05/29/2025]
Abstract
PURPOSE Herein, we present two interesting cases of adrenal ganglioneuromas (AGNs), namely, (a) a giant AGN which caused a severe episode of hypertension during a therapeutic dilation and curettage for early pregnancy loss, and (b) a case of a composite clinically silent adrenal ganglioneuroma-pheochromocytoma. Furthermore, we conducted a mini literature review on AGNs. CASE 1: A 31-year-old female patient presented with a history of early pregnancy loss at 9 ½ weeks. She underwent a therapeutic dilation and curettage during which she developed hypertension (~ 210/120 mm Hg). She reported no history of arterial hypertension, flushing, or tachycardia and her medical history was unremarkable. Further work-up, revealed a large heterogeneous tumor with microcalcifications in the left adrenal gland. Endocrine work-up was negative for hormonal excess. Magnetic resonance angiography (MRA) of the abdomen showed that the tumor was 'surrounding and strangling' the left renal vessels and the inferior vena cava. The patient successfully underwent an open left adrenalectomy and nephrectomy. Histology revealed an adrenal mature ganglioneuroma with Schwannian stroma being dominant. Her postoperative course was uneventful and she remains recurrence-free 6 years after surgery. CASE 2: A 37-year-old male patient was admitted to the hospital because of abdominal pain and hematuria. Computer tomography identified a 4-cm right adrenal lesion. Due to elevated urinary metanephrines, he underwent laparoscopic right adrenalectomy after appropriate alpha-blockade preparation preoperatively. Histology was consistent with pheochromocytoma with a component of ganglioneuroma, thus, a composite pheochromocytoma. His post-operative course was excellent and he remains asymptomatic and recurrence-free 60 months post-operatively. CONCLUSION These are two challenging cases of (a) a giant non-secreting AGN presenting with severe hypertension due to renal vessel compression and (b) a clinically silent composite pheochromocytoma-ganglioneuroma presenting with abdominal pain and hematuria. The size in the first case and the concurrence with a pheochromocytoma but a clinically silent phenotype in the second case highlight the fact that AGNs can be diagnostic chameleons.
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Affiliation(s)
- Efstratios Kardalas
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece
| | | | - Vasiliki Antonopoulou
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece
| | - Aggeliki Kyriakou
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece
| | - Sofia Georgiadi
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece
| | - Marinella Tzanela
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece
| | - Georgia Ntali
- Department of Endocrinology "D. Ikkos", Diabetes and Metabolism, European and National Expertise Centre for Rare Endocrine Diseases, European Reference Network, Endo-ERN, Evangelismos Hospital, Athens, Greece.
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Liu J, Swaiti A, Graham S, Martorella A, Yi S, Geisinger K, Khan ZH, Regan K. An Exophytic Pancreatic Mass Diagnosed as Ganglioneuroma. ACG Case Rep J 2025; 12:e01677. [PMID: 40242301 PMCID: PMC12002381 DOI: 10.14309/crj.0000000000001677] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2025] [Accepted: 03/19/2025] [Indexed: 04/18/2025] Open
Abstract
Ganglioneuromas are rare neuroendocrine tumors affecting around one in a million, mostly young adults. These slow-growing, well-differentiated neuroepithelial tumors originate from the neural crest and involve the sympathetic nervous system. We present an unusual case of a 47-year-old African American man with mildly elevated liver enzymes who was incidentally found to have a suspected pancreatic mass. Endoscopic ultrasound and fine-needle biopsies confirmed the diagnosis of a ganglioneuroma, inseparable and exophytic in the body of the pancreas extending into the tail.
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Affiliation(s)
- Jinye Liu
- Department of Internal Medicine, East Carolina University Health Medical Center, Greenville, NC
| | - Abdulazeez Swaiti
- Department of Internal Medicine, East Carolina University Health Medical Center, Greenville, NC
| | - Saeed Graham
- Department of Internal Medicine, East Carolina University Health Medical Center, Greenville, NC
| | - Alessandra Martorella
- Department of Internal Medicine, MedStar Georgetown University Hospital, Washington, DC
| | - Sha Yi
- Department of Pathology, East Carolina University Health Medical Center, Greenville, NC
| | - Kim Geisinger
- Department of Pathology, East Carolina University Health Medical Center, Greenville, NC
| | - Zarak Hassan Khan
- Department of Gastroenterology, East Carolina University Health Medical Center, Greenville, NC
| | - Kara Regan
- Department of Gastroenterology, East Carolina University Health Medical Center, Greenville, NC
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Ebrahimzadeh K, Mirahmadi Eraghi M, Bidari Zerehpoosh F, Tavasol HH, Abbaszdeh M, Dmytriw AA, Jahanshahi F. An intracranial odyssey: pediatric ganglioneuroma arising from the trigeminal ganglion: a case report and review of the literature. J Med Case Rep 2024; 18:600. [PMID: 39695863 DOI: 10.1186/s13256-024-04947-9] [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: 01/12/2024] [Accepted: 09/09/2024] [Indexed: 12/20/2024] Open
Abstract
BACKGROUND Ganglioneuroma represents an uncommon benign tumor arising from the sympathetic nerves, and its development from the fifth nerve is an infrequent entity. Few ganglioneuromas arising from the fifth nerve have been discussed in literature. The authors describe the second pediatric ganglioneuroma arising from the fifth nerve. CASE PRESENTATION A 7 year-old Asian male suffering from left-sided facial paresthesia, accompanied by several episodes of complex seizures over the past 2 years, presented to our clinic. The patient also complained of sudden unconsciousness and concurrent upward gaze lasting less than a minute. A brain computed tomography scan and magnetic resonance imaging revealed an isodense lesion containing focal calcifications measuring 31 × 28 mm and a solid mass extending from the pontine surface to Meckel's cave, probably originating from the fifth nerve or its root entry zone. The lesion was mildly hypointense on T1-weighted sequences and hyperintense on T2-weighted and fluid attenuated inversion recovery sequences. Gadolinium injection revealed only minimal heterogeneous enhancement. Histopathologic and immunohistochemical findings were consistent with ganglioneuroma. A lateral approach via retrosigmoid incision and suboccipital lateral craniectomy toward the cerebellopontine angle was performed under neuromonitoring supervision, and the postoperative period was uneventful. CONCLUSION Although ganglioneuroma arising from the fifth nerve is an extremely rare entity, it should be considered when diagnosing lesions in Meckel's cave or the cerebellopontine angle cistern.
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Affiliation(s)
- Kaveh Ebrahimzadeh
- Skull Base Research Center, Loghman-e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Makhsoos St, South Karegar Ave, Tehran, 1333635445, Iran
| | - Mohammad Mirahmadi Eraghi
- Skull Base Research Center, Loghman-e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Makhsoos St, South Karegar Ave, Tehran, 1333635445, Iran.
- Student Research Committee, School of Medicine, Qeshm International Branch, Islamic Azad University, Qeshm, Iran.
| | - Farahnaz Bidari Zerehpoosh
- Department of Pathology, Loghman-E-Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Hesameddin Hoseini Tavasol
- Skull Base Research Center, Loghman-e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Makhsoos St, South Karegar Ave, Tehran, 1333635445, Iran
| | - Mahkameh Abbaszdeh
- Skull Base Research Center, Loghman-e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Makhsoos St, South Karegar Ave, Tehran, 1333635445, Iran
| | - Adam A Dmytriw
- Skull Base Research Center, Loghman-e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Makhsoos St, South Karegar Ave, Tehran, 1333635445, Iran
| | - Fatemeh Jahanshahi
- Research Committee Member, Faculty of Medicine, Iran University of Medical Sciences, Tehran, Iran
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Rueda-de-Eusebio A, de la Torre Serrano M, Victoria Artalejo A, Mendez R. Adrenal Ganglioneuroma With Radiology-Pathology Correlation. Cureus 2024; 16:e69584. [PMID: 39421099 PMCID: PMC11484185 DOI: 10.7759/cureus.69584] [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] [Accepted: 09/15/2024] [Indexed: 10/19/2024] Open
Abstract
This report presents the case of a 62-year-old male with an incidentally discovered adrenal mass, ultimately diagnosed as a ganglioneuroma after laparoscopic adrenalectomy. Imaging findings, of a 9 cm lobulated mass with heterogeneous enhancement on MRI, were unspecific and malignancy could not be excluded. Histological examination of the specimen revealed a well-demarcated tumor composed of Schwann cells and ganglion cells, confirming the diagnosis. Postoperative follow-up showed no recurrence. This case highlights the critical role of histopathological evaluation for definitive diagnosis, as imaging alone may be insufficient to distinguish adrenal ganglioneuroma from other potential malignancies due to its nonspecific radiological features.
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Affiliation(s)
| | | | | | - Ramiro Mendez
- Department of Radiology, Hospital Clínico San Carlos, Madrid, ESP
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Hu M, Tang Z, Cai Y, Yuan X. Giant adrenal ganglioneuroma in children: a case report. Discov Oncol 2022; 13:104. [PMID: 36239827 PMCID: PMC9568632 DOI: 10.1007/s12672-022-00573-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Accepted: 10/10/2022] [Indexed: 11/04/2022] Open
Abstract
BACKGROUND Ganglioneuromas (GNs) arise from the Schwann cells, ganglion cells, and neuronal tissues, and are extremely rare, slow-growing, benign tumors. GN has usually grown very large when it is diagnosed since no specific clinical symptoms or laboratory findings indicating GN are available, especially when it occurs in the retroperitoneal space. Total resection of the tumor is the recommended treatment. We present the imaging and pathological findings of a giant adrenal GN in a child. A 7-year-old boy suffered from nausea and postprandial vomiting for 1 week with no precipitating factors. There was no family history of any disease, and the boy did not suffer from any disease in the past. Biochemical examination showed normal results. Physical examination showed an immobilized palpable mass in the left abdominal area. Abdominal computed tomography revealed a 13 cm × 10 cm solid mass in the retroperitoneal space. The mass showed slight and heterogeneous enhancement after injection of a contrasting agent. The mass was surgically resected locally to address the embedded abdominal vessels, and the histopathological and immunohistochemical diagnosis of the mass was GN. After the surgery, the symptoms of nausea and vomiting were relieved, and no complications occurred. CONCLUSION GN should be considered when a child presents with a giant retroperitoneal hypodense mass and the mass presents uneven and delayed enhancement. Histopathology is the golden standard for the diagnosis of GN. Currently, surgical excision is the optimal treatment.
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Affiliation(s)
- Mingqiu Hu
- Department of Urology, MaomingPeoplès Hospital, Maoming, 525000, China.
| | - Zhizhong Tang
- Department of Urology, MaomingPeoplès Hospital, Maoming, 525000, China
| | - Yong Cai
- Department of Computed Tomography, MaomingPeoplès Hospital, Maoming, 525000, China
| | - Xiaolu Yuan
- Department of Pathology, MaomingPeoplès Hospital, Maoming, 525000, China
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Abstract
Adrenal ganglioneuroma (AGN) are sympathetic differentiated tumors that originate from neural crest cells. It is a rare benign tumor in children and young adults. These lesions are usually asymptomatic and tend to be hormonally silent. Their discovery is fortuitous in imaging examinations. Preoperative diagnosis remains difficult, and the gold standard treatment is adrenalectomy. There is a good prognosis after surgery without recurrence. We herein report a case of adrenal ganglioneuroma in a 40-year-old man who benefited from an abdominal CT scan in the face of a complaint of abdominal discomfort and as part of the extension assessment of his colonic adenocarcinoma. Abdominal CT scan with contrast showed a left retroperitoneal mass of triangular shape within the adrenal lodge of tissue density, containing some calcifications not enhanced after injection of contrast product, measuring 90 x 62 mm in diameter (AP x T) with a relative washout calculated at 30%. Biopsy and histological examination of the mass suggested an adrenal ganglioneuroma.
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Kordeni K, Chardalias L, Pantiora E, Massaras D, Papadopoulos K, Primetis E, Kontis E, Fragulidis G. Retroperitoneal ganglioneuroma presenting as lower back pain. J Surg Case Rep 2022; 2022:rjac082. [PMID: 35444793 PMCID: PMC9015710 DOI: 10.1093/jscr/rjac082] [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: 01/19/2022] [Accepted: 02/18/2022] [Indexed: 11/25/2022] Open
Abstract
Retroperitoneal ganglioneuroma (RGN) is a rare benign tumor that arises from the retroperitoneal sympathetic ganglia composed of mature Schwann cells, ganglion cells and nerve fibers. These tumors can occur anywhere along the paravertebral sympathetic plexus and occasionally from adrenal medulla. Although they grow in excessive size, they may cause compression to adjacent organ or structures thus giving rise to symptoms. Resecting RGN’s is a challenging endeavor, as they tend to encase neighboring vessels to their site of origin. The reported case is a 43-year-old male who presented with lumbar pain that increased progressively in intensity over the last 6 months. Preoperative investigations revealed a large tumor with encasement of the origins of the superior mesenteric artery and bilateral renal arteries. The tumor was completely resected and the final pathology confirmed the diagnosis of RGN.
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Affiliation(s)
- Kleoniki Kordeni
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
| | - Leonidas Chardalias
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
| | - Eirini Pantiora
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
| | - Dimitris Massaras
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
| | - Konstantinos Papadopoulos
- Department of Pathology, Aretaieion Hospital, School of Medicine National, Kapodistrian University of Athens, Athens, Greece
| | - Elias Primetis
- 1st Department of Radiology, Aretaieion Hospital, School of Medicine National, Kapodistrian University of Athens, Athens, Greece
| | - Elissaios Kontis
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
| | - Georgios Fragulidis
- 2nd Department of Surgery, Aretaieion Hospital, School of Medicine National and Kapodistrian University of Athens, Athens, Greece
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Abstract
Schwannoma is a common mesenchymal neoplasm; however, adrenal schwannoma is rare, and it is frequently misdiagnosed as adrenal cortical adenoma. We herein report a 91-year-old Japanese man with right adrenal schwannoma that was pathologically diagnosed after adrenalectomy. To our knowledge, this is the first case of adrenal schwannoma in the oldest patient and with the longest follow-up period reported, including radiological images from 10 years earlier.
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Affiliation(s)
- Kenji Yorita
- Department of Diagnostic Pathology, Japanese Red Cross Kochi Hospital, Japan
| | - Takushi Naroda
- Department of Urology, Japanese Red Cross Kochi Hospital, Japan
| | - Masato Tamura
- Department of Urology, Japanese Red Cross Kochi Hospital, Japan
| | - Satoshi Ito
- Department of Radiology, Japanese Red Cross Kochi Hospital, Japan
| | - Kimiko Nakatani
- Department of Radiology, Japanese Red Cross Kochi Hospital, Japan
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Teng Q, Fan B, Wang Y, Wen S, Wang H, Liu T, Wang L. Primary adrenal tuberculosis infection in patients with Behcet's disease presenting as isolated adrenal metastasis by 18F-FDG PET/CT: a rare case report and literature review. Gland Surg 2021; 10:3431-3442. [PMID: 35070903 PMCID: PMC8749099 DOI: 10.21037/gs-21-511] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2021] [Accepted: 11/05/2021] [Indexed: 07/30/2023]
Abstract
Primary adrenal tuberculosis (TB) is a rare type of extrapulmonary tuberculosis (EPTB). A pathological biopsy is usually required to make a definite diagnosis due to nonspecific symptoms. Antituberculous chemotherapy is the main treatment regimen, and cortisol replacement therapy should be added when adrenal insufficiency is involved. Here, we present a 59-year-old man who had recurrence of oral and genital aphthosis for 3 years and was diagnosed with Behcet's disease (BD), which was cured by thalidomide. After 10 days of admission, the patient had sudden abdominal pain in the right upper quadrant with high fever and was diagnosed with acute cholecystitis attack, which was treated by percutaneous transhepatic gallbladder drainage (PTGBD). Further contrast-enhanced CT showed a right adrenal mass with a diameter of 2.0 cm, and PET-CT indicated intense 18F-fluorodeoxyglucose (18F-FDG) uptake in the right adrenal mass with a maximum standardized uptake value (SUVmax) of 15.2. As a metastatic adrenal mass was suspected, the patient underwent retroperitoneal laparoscopic adrenalectomy. Histopathological and immunohistochemical analysis revealed primary adrenal TB. After routine anti-tuberculosis treatment with isoniazid, rifampin, pyrazinamide and ethambutol for six months, the patient was cured and discharged. In summary, primary unilateral adrenal TB without adrenal insufficiency is difficult to diagnose only on the basis of clinical manifestations and examinations. Further studies are needed to develop an easier and more accurate diagnostic examination.
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Affiliation(s)
- Qiliang Teng
- Department of Urology, Second Affiliated Hospital of Dalian Medical University, Dalian, China
| | - Bo Fan
- Department of Urology, Second Affiliated Hospital of Dalian Medical University, Dalian, China
| | - Yutong Wang
- First Clinical College, Dalian Medical University, Dalian, China
| | - Shuang Wen
- Department of Pathology, Dalian Friendship Hospital, Dalian, China
| | - Honglong Wang
- Department of Pathology, Dalian Friendship Hospital, Dalian, China
| | - Tianqing Liu
- Department of Pathology, Dalian Friendship Hospital, Dalian, China
| | - Liang Wang
- Department of Urology, Second Affiliated Hospital of Dalian Medical University, Dalian, China
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Sandru F, Dumitrascu MC, Petca A, Carsote M, Petca RC, Oproiu AM, Ghemigian A. Adrenal ganglioneuroma: Prognostic factors (Review). Exp Ther Med 2021; 22:1338. [PMID: 34630692 DOI: 10.3892/etm.2021.10773] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2021] [Accepted: 08/23/2021] [Indexed: 12/19/2022] Open
Abstract
Ganglioneuroma, a rare neural crest-derived tumor, exhibits a benign profile in contrast to other neuroblastic tumors (neuroblastoma/ganglioneuroblastoma). Ganglioneuromas can be found anywhere autonomic ganglia are located, mostly abdominal/pelvic sites followed by the adrenal glands (one-third of cases), mediastinum/thorax and cervical area. Affecting especially children more than 10 years of age, Ganglioneuroma is either asymptomatic or may cause local compressive effects; rarely inducing nonspecific abdominal complains or arterial hypertension related to oversecretion of epinephrine/norepinephrine/dopamine. Despite a good prognosis, adrenalectomy is necessary in order to rule out a malignancy. Open procedure represents the standard therapeutic option; alternatively, centers with large laparoscopic pediatric experience and good stratification protocols have reported successful procedures. High uptake of I123-MIBG is associated with a more severe outcome in cases with increased mitotic index. In neuroblastic tumors, neuron-specific enolase >33 ng/ml, age at diagnosis <49 months, and blood vessel invasion indicate a poor prognosis. Concurrent extra-adrenal/adrenal ganglioneuroma is associated with a more severe prognosis; post-surgical complications are more frequent in non-adrenal vs. adrenal ganglioneuroma. Exceptionally, immune-mediated paraneoplastic neurologic syndromes have been reported: anti-N-methyl-D-aspartate receptor encephalitis and opsoclonus-myoclonus-ataxia syndrome. ROHHAD syndrome is the underlying cause in 40-56% of cases of neuroendocrine tumors including ganglioneuroma; 70% of tumors are diagnosed within the first 24 months after hypothalamic obesity onset, associated with a severe prognosis due to hypoventilation, sleep apnea, and dysautonomia. Recently, the PKB/AKT/mTOR/S6 pathway was identified as a tumorigenic pathway in pediatric ganglioneuroma, not in neuroblastoma; mTOR inhibitors are a potential option for pre-operatory tumor shrinkage. Pediatric adrenal ganglioneuroma has a good prognosis if adequately treated; its recognition requires adrenalectomy. Further development of specific biomarkers is needed. In the present article, we aimed to introduce a review of the literature involving adrenal ganglioneuroma based on a practical, multidisciplinary perspective of prognostic factors.
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Affiliation(s)
- Florica Sandru
- Department of Dermatology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Dermatology, 'Elias' Emergency Hospital, 011461 Bucharest, Romania
| | - Mihai Cristian Dumitrascu
- Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Obstetrics and Gynecology, University Emergency Hospital of Bucharest, 050098 Bucharest, Romania
| | - Aida Petca
- Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Obstetrics and Gynecology, 'Elias' Emergency Hospital, 022461 Bucharest, Romania
| | - Mara Carsote
- Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Endocrinology, 'C. I. Parhon' National Institute of Endocrinology, 011863 Bucharest, Romania
| | - Razvan-Cosmin Petca
- Department of Urology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Urology, 'Prof. Dr. Theodor Burghele' Clinical Hospital, 061344 Bucharest, Romania
| | - Ana Maria Oproiu
- Department of Plastic and Reconstructive Surgery, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Plastic and Reconstructive Surgery, University Emergency Hospital of Bucharest, 050098 Bucharest, Romania
| | - Adina Ghemigian
- Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.,Department of Endocrinology, 'C. I. Parhon' National Institute of Endocrinology, 011863 Bucharest, Romania
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Zilberman DE, Drori T, Shlomai G, Mazeh H, Fishman B, Golan S, Hendel H, Laniado M, Dotan ZA. Adrenal ganglioneuroma resected for suspicious malignancy: multicenter review of 25 cases and review of the literature. Ann Surg Treat Res 2021; 101:79-84. [PMID: 34386456 PMCID: PMC8331556 DOI: 10.4174/astr.2021.101.2.79] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2020] [Revised: 04/14/2021] [Accepted: 06/05/2021] [Indexed: 11/30/2022] Open
Abstract
Purpose We reviewed the experience with adrenal ganglioneuroma (AGN) pathologically confirmed following adrenalectomy in medium- to high-volume medical centers. Methods The medical records of all adrenalectomy cases in 4 medical centers between 2006 and 2020 were retrospectively reviewed for demographics, clinical, radiological and laboratory findings, surgical treatment, pathology results, and outcomes. Results Twenty-five out of 875 adrenalectomy cases (2.9%) were pathologically confirmed as AGN. Those patients' average age was 40.5 years (range, 4-76 years), 13 (52.0%) were males, and 18 lesions (72.0%) were right-sided. One patient had a family history of neurofibromatosis, and another had a succinate dehydrogenase gene mutation. Abdominal/back pain attributed to mass effect was the most common symptom. All 25 patients underwent abdominal computerized tomography scanning in which the average maximal tumor diameter was 6.61 cm. The mean pre- and postcontrast Hounsfield units (HU) values were 35.2 and 59, respectively; and the mean late-phase HU value was 71.1. Twenty-two patients (88.0%) underwent minimally invasive surgery. The average tumor diameter recorded in the final pathology report was 7 cm. Isolated AGN was diagnosed in 21 cases (84.0%), and the additional components reported for the remaining 4 cases included pheochromocytoma (2), ganglioneuroblastoma (1), and neurofibroma (1). The average follow-up length was 16.8 months (range, 1-136 months), during which there was no recurrence or death. Conclusion AGN is a rare, slow-growing, large benign tumor with radiological characteristics similar to those seen in malignant tumor. Final diagnosis is established by pathology after surgical resection, preferably minimally invasive, with an overall excellent prognosis.
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Affiliation(s)
- Dorit Esther Zilberman
- Department of Urology, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
| | - Tomer Drori
- Department of Urology, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
| | - Gadi Shlomai
- Institute of Endocrinology, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.,Department of Internal Medicine D, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, TelAviv University, Tel-Aviv, Israel
| | - Haggi Mazeh
- Department of Surgery, Hadassah Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel
| | - Boris Fishman
- Department of Internal Medicine D, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, TelAviv University, Tel-Aviv, Israel
| | - Shay Golan
- Department of Urology, Rabin Medical Center, Petah Tikva affiliated to the Sackler School of Medicine, Tel-Aviv University, TelAviv, Israel
| | - Hen Hendel
- Department of Urology, Rabin Medical Center, Petah Tikva affiliated to the Sackler School of Medicine, Tel-Aviv University, TelAviv, Israel
| | - Monica Laniado
- Department of General Surgery, Bnai-Zion Medical Center, Haifa, Israel
| | - Zohar A Dotan
- Department of Urology, Chaim Sheba Medical Center, Tel-Hashomer, affiliated to the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
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12
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Zhang QW, Song T, Yang PP, Hao Q. Retroperitoneum ganglioneuroma: imaging features and surgical outcomes of 35 cases at a Chinese Institution. BMC Med Imaging 2021; 21:114. [PMID: 34294064 PMCID: PMC8296746 DOI: 10.1186/s12880-021-00643-y] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Accepted: 07/07/2021] [Indexed: 11/15/2022] Open
Abstract
Background The preoperative evaluation is crucial for diagnosis and surgical plan of retroperitoneum ganglioneuroma (GN). In this study, we reviewed a relatively large series of histopathological proved retroperitoneum GN cases, summarized the imaging features and further depicted risk factors of increased surgical blood loss. Methods A total of 35 (18 male, 17 female) patients were retrospectively enrolled from January 2012 to June 2019 at our institution. Among them, 24 patients had undergone CT scans and 19 patients had undergone MR examination before treatment. The clinical and radiological features were analyzed and the relationships between image features and surgical blood loss were evaluated. Results The media age of the involved 35 patients was 40 years (range, 14–66 years). The histological tumor size was 10.12 ± 4.56 cm for average. Retroperitoneum GN was relatively low density on unenhanced CT images and showed delayed progressive enhancement on enhanced CT and MR images. The whorled sign could be seen in 14 patients. The vessel encasement sign could be found in 17 patients. Univariate analysis revealed maximal tumor size measured on axial image, maximal tumor size measured on coronal image, encasing one or both renal pedicles, encasing the aorta and/or vena cava and whorled sign on MRI showed significant difference between the blood loss ≥ 400 ml and blood loss < 400 ml group. Logistic regression further detected that maximal tumor size measured on axial images (OR: 1.12; 95% CI: 1.02–1.24; P = 0.023) and encasing one or both renal pedicles (OR: 22.39; 95% CI: 1.35–372.99; P = 0.030) were independently correlated with surgical blood loss. Conclusions Preoperative CT and MR imaging analysis was valuable for both diagnosis and surgical risk prediction of retroperitoneum GN.
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Affiliation(s)
- Qian-Wen Zhang
- Department of Radiology, Changhai Hospital, The Navy Medical University (Second Military Medical University), 168 Changhai Road, Shanghai, China
| | - Tao Song
- Department of Radiology, Changhai Hospital, The Navy Medical University (Second Military Medical University), 168 Changhai Road, Shanghai, China
| | - Pan-Pan Yang
- Department of Radiology, Changhai Hospital, The Navy Medical University (Second Military Medical University), 168 Changhai Road, Shanghai, China
| | - Qiang Hao
- Department of Radiology, Changhai Hospital, The Navy Medical University (Second Military Medical University), 168 Changhai Road, Shanghai, China.
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13
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Dages KN, Kohlenberg JD, Young WF, Murad MH, Prokop L, Rivera M, Dy B, Foster T, Lyden M, McKenzie T, Thompson G, Bancos I. Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature. Clin Endocrinol (Oxf) 2021; 95:47-57. [PMID: 33721367 PMCID: PMC8178203 DOI: 10.1111/cen.14460] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 02/20/2021] [Accepted: 03/09/2021] [Indexed: 12/29/2022]
Abstract
OBJECTIVE To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs). DESIGN Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019). PATIENTS Diagnosed with histologically confirmed AGN. MEASUREMENTS Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours). RESULTS The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated. CONCLUSIONS AGNs are rare benign tumours that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.
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Affiliation(s)
- Kelley N. Dages
- Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
| | - Jacob D. Kohlenberg
- Division of Endocrinology, Diabetes, Metabolism and Nutrition, and Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
| | - William F. Young
- Division of Endocrinology, Diabetes, Metabolism and Nutrition, and Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
| | - Mohammad Hassan Murad
- Evidence-Based Practice Center, Center for the Science of Healthcare Delivery, Mayo Clinic, Rochester, Minnesota
| | - Larry Prokop
- Mayo Clinic Libraries, Mayo Clinic, Rochester, Minnesota, USA
| | - Michael Rivera
- Department of Laboratory Medicine and Pathology, Anatomic Pathology, Mayo Clinic, Rochester, Minnesota
| | - Benzon Dy
- Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Trenton Foster
- Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Melanie Lyden
- Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Travis McKenzie
- Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Geoffrey Thompson
- Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Irina Bancos
- Division of Endocrinology, Diabetes, Metabolism and Nutrition, and Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
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Gokten OE. Laparoscopic adrenalectomy for suspected adrenal mass in lung cancer: ganglioneuroma. TUMORI JOURNAL 2021; 107:NP81-NP83. [PMID: 34058924 DOI: 10.1177/03008916211020080] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND Adrenalectomy is the gold standard for the treatment of primary adrenal ganglioneuroma. Preoperative differential diagnosis of ganglioneuroma is difficult and histopathologic examination is required to confirm the diagnosis. PATIENT A 61-year-old woman, who was followed and treated by the medical oncology department because of lung carcinoma pathology squamous cell T4N0Mx, presented to our clinic due to a 15×8-mm nodule with a calculated relative wash-out rate of 40% in the right adrenal gland lateral edge, which was observed on computed tomography examination. CONCLUSION Adrenal ganglioneuroma is a rarely seen, hormonally inactive, benign tumor. Final diagnosis can be made with histopathologic examination. Adrenalectomy is the standard treatment preferred for ganglioneuroma. Knowing the clinical appearance and good pathologic specialization are important adjunct matters in diagnosis. Laparoscopic adrenalectomy has become the gold standard in most patients with adrenal tumors.
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15
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Fliedner SMJ, Winkelmann PER, Wesley R, Vonthein R, Lehnert H. Ganglioneuromas across age groups: Systematic review of individual patient data. Clin Endocrinol (Oxf) 2021; 94:12-23. [PMID: 32702779 DOI: 10.1111/cen.14297] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2020] [Revised: 06/23/2020] [Accepted: 07/13/2020] [Indexed: 12/18/2022]
Abstract
BACKGROUND Ganglioneuromas are very rare tumours of the sympathetic nervous system. Clinical and pathological knowledge is currently based on largely incomparable registries and case series that focus on paediatric or adrenal cases. To comprehensively characterize the full clinical spectrum across ages and locations, a meta-analysis was performed where amenable and complemented by systematic literature review of individual patient data (IPD). DESIGN Articles containing "ganglioneuroma" in English on humans, published from 1/1/1995-6/27/2018, were identified from PubMed. Aggregate data from 10 eligible patient series on 19 variables were considerably inhomogeneous, restricting meta-analysis to age and gender distribution. To determine basic disease characteristics across ages and locations, IPD were retrieved from case reports and small case series (PROSPERO CRD42018010247). RESULTS Individual patient data representing 364 cases revealed that 65.7% (60.6%-70.4%) were diagnosed in adults, more frequently in females (62%, 56.9%-66.9%). 24.5% (20.3%-39.1%) were discovered incidentally. Most often, ganglioneuromas developed in abdomen/pelvis (66.2, 32.1% adrenal). With age, the proportion of ganglioneuroma localizations with high post-surgical complication rate (35.6% head/neck and 16.3% thorax) decreased. Contrarily, the diagnosis of adrenal ganglioneuromas (<1% post-surgical complications) increased with age. Hormone production, hypertension or coincidence with another non-neuroblastic neural-crest-derived tumour component was more common for adrenal location. Recurrence and metastatic spread have not been reported for ganglioneuromas without secondary tumour component. CONCLUSIONS This work summarizes characteristics of the currently largest number of international GN patients across all ages. The data confirm a benign nature of GN, independent of age. Age-related differences in predominant tumour location, associated post-surgical complications and hormone production suggest case-centred management strategies.
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Affiliation(s)
- Stephanie M J Fliedner
- 1st Department of Medicine, University Medical Center Schleswig-Holstein, Campus Lübeck, University of Lübeck, Lübeck, Germany
| | - Philipp E R Winkelmann
- Department of Hematology and Medical Oncology, University Medical Center Schleswig-Holstein, University of Lübeck, Lübeck, Germany
| | | | - Reinhard Vonthein
- Institut für Medizinische Biometrie, Universität zu Lübeck, Lübeck, Germany
- Institut für Statistik, Ludwig-Maximilians-Universität München, München, Germany
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16
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Papaetis GS, Georgiadis CP, Tsitskari MA, Constantinou PG, Antoniou AP. Retroperitoneal ganglioneuroma causing chronic lower back and leg pain in an 80-year-old man: A case report. Ann Med Surg (Lond) 2020; 61:101-103. [PMID: 33437470 PMCID: PMC7785993 DOI: 10.1016/j.amsu.2020.12.027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2020] [Revised: 12/18/2020] [Accepted: 12/20/2020] [Indexed: 11/28/2022] Open
Abstract
Introduction and importance: Retroperitoneal ganglioneuromas that cause lower back and leg pain are extremely rare and are often misdiagnosed. Surgical resection has excellent prognosis in long-term survival. Case presentation We present an 80-year-old man with two-year worsening left lower back and leg pain. He was treated as presumed lumbar spine spondylosis with several courses of physical therapy together with medical treatment. An abdomen CT scan disclosed a tumour in the left retrorenal space. The tumour was resected and the histopathologic examination suggested a completely excised retroperitoneal ganglioneuroma. During one-year follow-up the patient is free of pain without any local recurrence. Clinical discussion Retroperitoneal ganglioneuromas are rare benign tumors that originate from neural crest-derived cells of the paravertebral sympathetic plexus and sometimes from the adrenal medulla. They are usually asymptomatic and discovered on routine clinical examination or on autopsy. Occasionally they may show symptoms due to local pressure effect or rarely they are hormonally active and present with adrenergic symptoms. Complete resection of the tumor is important in order establish the final diagnosis and alleviate symptoms from pressure effects. Conclusion This case highlights the need for great vigilance among physicians in order to consider any possible retroperitoneal pathology when indicated in the differential diagnosis of lower back and leg pain, before establishing other more common diagnosis, especially in the older population.
Retroperitoneal ganglioneuromas that cause lower back and leg pain are extremely rare. They are composed by Schwann cells on nerve fibres and mature sympathetic ganglion cells. Complete resection of retroperitoneal ganglioneuromas has an excellent prognosis. High suspicion is required to rule out any retroperitoneal pathology in this clinical setting.
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Affiliation(s)
- Georgios S Papaetis
- Internal Medicine and Diabetes Clinic, Eleftherios Venizelos Avenue 62, Paphos, Cyprus.,CDA College, 73 Democratias Avenue, Paphos, Cyprus
| | - Christos P Georgiadis
- Department of Surgery, Evangelismos Hospital, Vasileos Constantinou 87 Street, Paphos, Cyprus
| | - Maria A Tsitskari
- Vascular and Interventional Radiology, Apollonio Hospital, Nicosia, Cyprus
| | - Pavlos G Constantinou
- Histopathology and Cytology Laboratory Services, 41, Andrea Avraamides Street, 2024, Nicosia, Cyprus
| | - Antonis P Antoniou
- Department of Urology, Evangelismos Hospital, Vasileos Constantinou 87 Street, Paphos, Cyprus
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Boutzios G, Papaoiconomou E, Karatza E, Dimitroulis D. Large adrenal ganglioneuroma: A rare entity with atypical radiological characteristics. Hormones (Athens) 2020; 19:609-610. [PMID: 32415649 DOI: 10.1007/s42000-020-00204-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2020] [Accepted: 04/23/2020] [Indexed: 10/24/2022]
Affiliation(s)
- Georgios Boutzios
- Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias Str., 11527, Athens, Greece.
| | - Eleni Papaoiconomou
- Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, 75 Mikras Asias Str., 11527, Athens, Greece
| | - Elli Karatza
- Second Department of Propedaeutic Surgery, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Dimitrios Dimitroulis
- Second Department of Propedaeutic Surgery, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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18
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Burns AJ, Sullivan KM, Koh EK, Tretiakova MS, Zern NK. Dopamine secreting adrenal tumor-ganglioneuroma rather than pheochromocytoma: case report. Gland Surg 2020; 9:2204-2210. [PMID: 33447572 DOI: 10.21037/gs-20-475] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Ganglioneuromas are rare, benign, well-differentiated neural crest tumors arising in the paravertebral sympathetic chain, and are classically non-secretory and clinically asymptomatic. As the diagnosis of ganglioneuroma is based on histopathology, the clinical presentation prior to surgical excision often mirrors that of pheochromocytoma or adrenal cortical adenoma. We describe a case of an incidentally found right sided calcified adrenal mass with evidence of marked dopamine excess, suspicious for pheochromocytoma in a 70-year-old female. The patient endorsed a 6-month history of intermittent right flank pain and a 2-year history of weight loss and fatigue. She reported mild symptoms of hypomania but denied other symptoms of dopamine excess including agitation, anxiety, nausea, and vomiting. Exam revealed isolated mild hypertension. The imaging features of this mass were concerning for malignancy including the presence of macrocalcification and irregular borders. After preoperative alpha blockade, the patient underwent open right adrenalectomy and the final pathology was consistent with ganglioneuroma rather than pheochromocytoma. Following resection, the dopamine level normalized, confirming the resected right adrenal ganglioneuroma as the source of dopamine excess. This case represents a rare presentation of dopamine-secreting adrenal ganglioneuroma. This illustrates that although rare, ganglioneuroma should be included on the differential diagnosis for functional adrenal tumors.
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Affiliation(s)
- Alicia J Burns
- School of Medicine, University of Washington, Seattle, WA, USA
| | - Kevin M Sullivan
- Department of Surgery, University of Washington, Seattle, WA, USA
| | - Eun K Koh
- Multicare Endocrinology, Tacoma, WA, USA
| | | | - Nicole K Zern
- Department of Surgery, University of Washington, Seattle, WA, USA
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19
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Shao M, Zhang W, Niu Z, Chen S, Jia Y, An Y, Zhu F, Wang J. Computed tomography characteristics of adrenal ganglioneuroma: a retrospective analysis of 30 pathologically-confirmed cases. J Int Med Res 2020; 48:300060520945510. [PMID: 33176508 PMCID: PMC7673062 DOI: 10.1177/0300060520945510] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023] Open
Abstract
Objective To investigate the computed tomography (CT) characteristics of adrenal ganglioneuromas (AGNs) and to determine the ability of CT scanning to distinguish between large (>3 cm) and small (≤3 cm) AGNs. Methods This retrospective study searched the electronic medical record system of a hospital between January 2008 and July 2019 in order to identify patients with pathologically-confirmed AGNs that underwent three phases of CT scanning. The CT features were compared between large and small AGNs. Results A total of 30 patients with pathologically-confirmed AGNs were included in the study. The majority of patients (76.7%; 23 of 30) were asymptomatic and there were nonspecific symptoms in seven patients. The ‘pointed peach’ sign appeared in more than half of the patients (53.3%; 16 of 30). The CT value of the arterial phase, progressive enhancement, morphology and calcification in the CT images were found to be significantly different between large and small AGNs. Progressive enhancement was more likely to occur in small AGNs. Most large AGNs had irregular shapes, while small AGNs were likely to be round or oval with a smooth border. Calcifications were noted in large AGNs (42.9%; six of 14). Conclusion CT scanning can show many of the key imaging characteristics of AGNs used to distinguish between large and small AGNs.
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Affiliation(s)
- Meihua Shao
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Wenming Zhang
- Department of Radiology, Zhejiang University School of Medicine Sir Run Shaw Hospital, Hangzhou, Zhejiang Province, China
| | - Zhongfeng Niu
- Department of Radiology, Zhejiang University School of Medicine Sir Run Shaw Hospital, Hangzhou, Zhejiang Province, China
| | - Songkuan Chen
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Yuzhu Jia
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Yongyu An
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Fangmei Zhu
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
| | - Jian Wang
- Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China
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20
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Santosh K, Das MK, Ayyanar P, Mandal S, Nayak P, Kumar S. Laparoscopic excision of a large Adrenal Ganglioneuroma masquerading as Pheochromocytoma- A case report & review of literature. Urol Case Rep 2020; 33:101276. [PMID: 32489900 PMCID: PMC7262088 DOI: 10.1016/j.eucr.2020.101276] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2020] [Revised: 05/20/2020] [Accepted: 05/20/2020] [Indexed: 11/28/2022] Open
Abstract
Adrenal Ganglioneuromas are rare. Evaluation for hormone secretion followed by complete excision is the treatment of choice for such masses. We present our experience of a symptomatic large Adrenal Ganglioneuroma, which was removed with laparoscopic procedure. A 44-year gentleman presented with a symptomatic adrenal tumor. Under the impression of Adrenal Pheochromocytoma, tumor was excised laparoscopically without any untoward event. And to our surprise Adrenal Ganglioneuroma was detected in biopsy. Hence, Ganglioneuromas should be considered as a differential for a functional or symptomatic adrenal mass. And by following proper technique even such large functional adrenal masses can be removedlaparoscopically.
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Affiliation(s)
- K. Santosh
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, India
| | - Manoj K. Das
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, India
- Corresponding author. Department of Urology, All India Institute of Medical Sciences, Sijua, Bhubaneswar, 751019, India.
| | - Pavithra Ayyanar
- Department of Pathology, All India Institute of Medical Sciences, Bhubaneswar, India
| | - Swarnendu Mandal
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, India
| | - Prasant Nayak
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, India
| | - Sumit Kumar
- Department of Urology, All India Institute of Medical Sciences, Bhubaneswar, India
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21
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Ahmed AA, Thomas AJ, Ganeshan DM, Blair KJ, Lall C, Lee JT, Morshid AI, Habra MA, Elsayes KM. Adrenal cortical carcinoma: pathology, genomics, prognosis, imaging features, and mimics with impact on management. Abdom Radiol (NY) 2020; 45:945-963. [PMID: 31894378 DOI: 10.1007/s00261-019-02371-y] [Citation(s) in RCA: 21] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Most tumors are either metastatic or locally invasive at the time of diagnosis. Differentiation between ACC and other adrenal masses depends on clinical, biochemical, and imaging factors. This review will discuss the genetics, pathological, and imaging feature of ACC.
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Affiliation(s)
- Ayahallah A Ahmed
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX, 77030, USA
| | - Aaron J Thomas
- Department of Radiology, Baylor College of Medicine, Houston, TX, USA
| | - Dhakshina Moorthy Ganeshan
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX, 77030, USA
| | - Katherine J Blair
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX, 77030, USA
| | - Chandana Lall
- Department of Radiology, University of Florida College of Medicine, Jacksonville, FL, USA
| | - James T Lee
- Department of Radiology, University of Kentucky, Lexington, Kentucky, USA
| | - Ali I Morshid
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX, 77030, USA
| | - Mouhammed A Habra
- Departments of Endocrine Neoplasia and Hormonal Disorders, University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA
| | - Khaled M Elsayes
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, TX, 77030, USA.
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22
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Hanafy AK, Mujtaba B, Roman-Colon AM, Elsayes KM, Harrison D, Ramani NS, Waguespack SG, Morani AC. Imaging features of adrenal gland masses in the pediatric population. Abdom Radiol (NY) 2020; 45:964-981. [PMID: 31538225 DOI: 10.1007/s00261-019-02213-x] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
The spectrum of adrenal masses in the pediatric population markedly differs from that in the adult population. Imaging plays a crucial role in detecting adrenal masses, differentiating malignant from benign lesions, recognizing extra-adrenal lesions in the suprarenal fossa, and directing further management. Ultrasound is the primary imaging modality of choice for the evaluation of adrenal masses in the neonatal period, whereas MRI or CT is used as a problem-solving tool. In older children, computed tomography or magnetic resonance imaging is often required after initial sonographic evaluation for further characterization of a lesion. Herein, we discuss the salient imaging features along with pathophysiology and clinical features of pediatric adrenal masses.
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Affiliation(s)
- Abdelrahman K Hanafy
- The University of Texas MD Anderson Cancer Center, 1515 Holocombe Blvd, Houston, TX, 77030, USA
| | - Bilal Mujtaba
- The University of Texas MD Anderson Cancer Center, 1515 Holocombe Blvd, Houston, TX, 77030, USA
| | - Alicia M Roman-Colon
- Department of Diagnostic Radiology, Baylor College of Medicine, Houston, TX, USA
- Department of Radiology, Texas Children's Hospital, Houston, TX, USA
| | - Khaled M Elsayes
- The University of Texas MD Anderson Cancer Center, 1515 Holocombe Blvd, Houston, TX, 77030, USA
| | - Douglas Harrison
- Department of Pediatrics - Patient Care, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd. Unit 0087, Houston, TX, 77030-4009, USA
| | - Nisha S Ramani
- Department of Anatomic Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX, 77030, USA
| | - Steven G Waguespack
- Department of Endocrine Neoplasia, & Hormonal Disorders, University of Texas MD Anderson Cancer Center, 1515 Holocombe Blvd, Houston, TX, 77030, USA
| | - Ajaykumar C Morani
- The University of Texas MD Anderson Cancer Center, 1515 Holocombe Blvd, Houston, TX, 77030, USA.
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Abstract
Due to the widespread use of imaging, incidental adrenal masses are commonly encountered. A number of pitfalls can result in misdiagnosis of these lesions, including inappropriate choice of imaging technique, presence of pseudolesions, and overlap of imaging features of different adrenal lesions. This article explores the potential pitfalls in imaging of the adrenal glands, on computed tomography and magnetic resonance imaging, that can lead to misinterpretation. Clues to correct diagnoses are provided to evade potential misinterpretation.
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24
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Lorenz K, Langer P, Niederle B, Alesina P, Holzer K, Nies C, Musholt T, Goretzki PE, Rayes N, Quinkler M, Waldmann J, Simon D, Trupka A, Ladurner R, Hallfeldt K, Zielke A, Saeger D, Pöppel T, Kukuk G, Hötker A, Schabram P, Schopf S, Dotzenrath C, Riss P, Steinmüller T, Kopp I, Vorländer C, Walz MK, Bartsch DK. Surgical therapy of adrenal tumors: guidelines from the German Association of Endocrine Surgeons (CAEK). Langenbecks Arch Surg 2019; 404:385-401. [PMID: 30937523 DOI: 10.1007/s00423-019-01768-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2019] [Accepted: 02/19/2019] [Indexed: 12/27/2022]
Abstract
BACKGROUND AND AIMS Previous guidelines addressing surgery of adrenal tumors required actualization in adaption of developments in the area. The present guideline aims to provide practical and qualified recommendations on an evidence-based level reviewing the prevalent literature for the surgical therapy of adrenal tumors referring to patients of all age groups in operative medicine who require adrenal surgery. It primarily addresses general and visceral surgeons but offers information for all medical doctors related to conservative, ambulatory or inpatient care, rehabilitation, and general practice as well as pediatrics. It extends to interested patients to improve the knowledge and participation in the decision-making process regarding indications and methods of management of adrenal tumors. Furthermore, it provides effective medical options for the surgical treatment of adrenal lesions and balances positive and negative effects. Specific clinical questions addressed refer to indication, diagnostic procedures, effective therapeutic alternatives to surgery, type and extent of surgery, and postoperative management and follow-up regime. METHODS A PubMed research using specific key words identified literature to be considered and was evaluated for evidence previous to a formal Delphi decision process that finalized consented recommendations in a multidisciplinary setting. RESULTS Overall, 12 general and 52 specific recommendations regarding surgery for adrenal tumors were generated and complementary comments provided. CONCLUSION Effective and balanced medical options for the surgical treatment of adrenal tumors are provided on evidence-base. Specific clinical questions regarding indication, diagnostic procedures, alternatives to and type as well as extent of surgery for adrenal tumors including postoperative management are addressed.
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Affiliation(s)
- K Lorenz
- Universitätsklinikum Halle, Halle/Saale, Germany.
| | | | - B Niederle
- Ordination Siebenbrunnenstrasse, Wien, Austria
| | - P Alesina
- Kliniken Essen-Mitte, Essen, Germany
| | - K Holzer
- Universitätsklinikum Marburg, Marburg, Germany
| | - Ch Nies
- Marienhospital Osnabrück, Osnabrück, Germany
| | - Th Musholt
- Universitatsklinikum Mainz, Mainz, Germany
| | - P E Goretzki
- Charité Universitätsmedizin Berlin, Berlin, Germany
| | - N Rayes
- Universitätsklinikum Leipzig, Leipzig, Germany
| | - M Quinkler
- Endokrinologiepraxis Berlin, Berlin, Germany
| | - J Waldmann
- MIVENDO Klinik Hamburg, Hamburg, Germany
| | - D Simon
- Evangelisches Krankenhaus BETHESDA Duisburg, Duisburg, Germany
| | - A Trupka
- Klinikum Starnberg, Klinikum Starnberg, Germany
| | - R Ladurner
- Ludwig-Maximilians-Universität München, München, Germany
| | - K Hallfeldt
- Ludwig-Maximilians-Universität München, München, Germany
| | - A Zielke
- Diakonie-Klinikum Stuttgart, Stuttgart, Germany
| | - D Saeger
- Universitätsklinikum Hamburg, Hamburg, Germany
| | - Th Pöppel
- Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany
| | - G Kukuk
- Universitätsklinikum Bonn, Bonn, Germany
| | - A Hötker
- Universitätsklinikum Zürich, Zürich, Switzerland
| | - P Schabram
- RAE Ratacjzak und Partner, Sindelfingen, Germany
| | - S Schopf
- Krankenhaus Agatharied, Hausham, Germany
| | - C Dotzenrath
- HELIOS Universitätsklinikum Wuppertal, Wuppertal, Germany
| | - P Riss
- Medizinische Universität Wien, Wien, Austria
| | - Th Steinmüller
- Deutsches Rotes Kreuz Krankenhaus Berlin, Berlin, Germany
| | - I Kopp
- AWMF, Frankfurt am Main, Germany
| | - C Vorländer
- Bürgerhospital Frankfurt, Frankfurt am Main, Germany
| | - M K Walz
- Kliniken Essen-Mitte, Essen, Germany
| | - D K Bartsch
- Universitätsklinikum Marburg, Marburg, Germany
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Xie J, Dai J, Zhou WL, Sun FK. Adrenal Ganglioneuroma: Features and Outcomes of 42 Cases in a Chinese Population. World J Surg 2018; 42:2469-2475. [PMID: 29383425 DOI: 10.1007/s00268-018-4499-8] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
BACKGROUND Adrenal ganglioneuromas (AGNs) are extremely rare benign neoplasms. This study is to share our experience regarding diagnostic and operative management of these tumors. METHODS Clinical details as well as follow-up data were retrospectively analyzed in 42 primary AGN patients who received operative resection at a tertiary referral hospital in China between May 2005 and July 2016. RESULTS The mean age of the patients at diagnosis was 35.3 years (range 13-59 years). Among the patients, 28 were asymptomatic. The mean preoperative size was 5.3 ± 2.2 cm (range 2.1-14.0 cm), and 17 cases were larger than 5 cm. All patients underwent operative resection as open adrenalectomy in 20 patients and laparoscopy in 22 patients. The mean tumor size on pathologic examination was 6.0 ± 2.6 cm (range 2.2-17.0 cm). Laparoscopic operation showed its advantages over open surgery on the postoperative duration (5.0 vs. 7.3 days, p < 0.001) and estimated blood loss (69.5 vs. 157.5 ml, p = 0.047). No patient had recurrence or metastasis during a median follow-up of 70.5 months (range 3.7-164.3 months). CONCLUSIONS We describe the largest AGN series reported to date. The preoperative diagnosis of AGN remains difficult despite of the progression of imaging examination. After complete resection, the prognosis of AGN patients is excellent. Laparoscopic approach has its advantages in the resection of AGNs.
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Affiliation(s)
- Jing Xie
- Department of Urology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Ruijin Second Road No. 197, Shanghai, 200025, China
| | - Jun Dai
- Department of Urology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Ruijin Second Road No. 197, Shanghai, 200025, China
| | - Wen-Long Zhou
- Department of Urology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Ruijin Second Road No. 197, Shanghai, 200025, China
| | - Fu-Kang Sun
- Department of Urology, Shanghai Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Ruijin Second Road No. 197, Shanghai, 200025, China.
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Romero-Pérez D, Jegou MH, Lecointre C, Penchet I, Cribier B. Primary cutaneous ganglioneuroma: anatomico-clinical study of 4 cases with focus on Merkel cells. J Cutan Pathol 2018; 45:403-411. [DOI: 10.1111/cup.13144] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2018] [Revised: 03/08/2018] [Accepted: 03/13/2018] [Indexed: 11/25/2022]
Affiliation(s)
- David Romero-Pérez
- Department of Dermatology; University General Hospital of Alicante; Alicante Spain
| | | | - Claire Lecointre
- Department of Pathology; Regional Hospital Center of Orléans; Orléans France
| | | | - Bernard Cribier
- Department of Dermatopathology and Dermatology, Faculty of Medicine; University Hospital of Strasbourg, University of Strasbourg; Strasbourg France
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Nardi WS, Quildrian SD. Left adrenal ganglioneuroma: Report of a new case. Int J Surg Case Rep 2018; 45:143-145. [PMID: 29631229 PMCID: PMC6000988 DOI: 10.1016/j.ijscr.2018.02.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2018] [Accepted: 02/10/2018] [Indexed: 11/17/2022] Open
Abstract
INTRODUCTION Adrenal ganglioneuroma (AGN) represents about 20% of the reported cases. PRESENTATION OF CASE We present a 37-year-old man referred to our institution for a history of abdominal discomfort and a left adrenal solid mass incidentally discovered in CT abdominal scan (24 × 20 mm). Patient underwent laparoscopic surgical excision of the tumor. CONCLUSION The histopathology examination showed areas of spindle cells and scattered mature ganglionic cells compatible with AGN.
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Affiliation(s)
- Walter Sebastián Nardi
- Department of General Surgery, British Hospital of Buenos Aires, Buenos Aires, Perdriel 74, CABA, 1280AEB, Argentina.
| | - Sergio Damián Quildrian
- Soft Tissue Tumors Unit, Department of General Surgery, British Hospital of Buenos Aires, Buenos Aires, Perdriel 74, CABA, 1280AEB, Argentina.
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Varda BK, Cho P, Wagner AA, Lee RS. Collaborating with our adult colleagues: A case series of robotic surgery for suspicious and cancerous lesions in children and young adults performed in a free-standing children's hospital. J Pediatr Urol 2018; 14:182.e1-182.e8. [PMID: 29503221 PMCID: PMC5970046 DOI: 10.1016/j.jpurol.2018.01.003] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2017] [Accepted: 01/03/2018] [Indexed: 02/07/2023]
Abstract
BACKGROUND In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital. DESIGN We retrospectively reviewed all robotic cases performed at our institution from 2014 to 2016 for patients with a GU malignancy or a suspicious mass. The surgeries were performed by a pediatric urologist with robotic experience and a fellowship-trained MIS adult urologist specializing in oncology. Perioperative and oncologic outcomes were recorded. RESULTS A total of eight robotic cases were performed: four partial nephrectomies (PN) with retroperitoneal lymph node dissection (LND) (OT 269-338 min, EBL 5-300 mL, LOS 3-6 days), one adrenalectomy with LND (6.4 cm mass; OT 172 min, EBL 5 mL, LOS 3 days), one nephrectomy with pericaval LND (9.8 cm mass; 234 min, EBL 25 mL, LOS 3 days), and two retroperitoneal LNDs (OT 572 and 508 min, EBL 250 and 100, LOS 3 and 4 days). Patient weights ranged from 14 to 79 kg (mean 53.4 kg). There were no major complications (Clavien 3-5). Pathology results for PN included papillary RCC (AJCC pT1aNx) and two cases of segmental cystic renal dysplasia with nephrogenic rests. Bilateral template RPLNDs yielded paratesticular rhabdomyosarcoma (43 nodes; COG low risk group II stage I) and mixed non-seminomatous germ cell tumor (74 nodes; COG stage III). The nephrectomy yielded an undifferentiated sarcoma, low grade; the adrenalectomy favorable-type ganglioneuroma. DISCUSSION In pediatrics, urologic oncology cases are often managed with open surgery. Our series demonstrates the feasibility of using the robotic approach in carefully selected cases. In doing so, the patient benefits from a minimally invasive surgery, while the surgeon benefits from robotic surgical dexterity. We seamlessly advanced these new techniques through a step-wise collaboration between an adult urologist who routinely performs robotic oncology procedures and a pediatric urologist experienced in robotics for benign conditions. CONCLUSION In this small series, we safely and effectively adapted adult robotic techniques for genitourinary oncology cases in children and young adults.
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Affiliation(s)
- Briony K Varda
- Boston Children's Hospital, Department of Urology, Harvard Medical School, Boston, MA, USA.
| | - Patricia Cho
- Boston Children's Hospital, Department of Urology, Harvard Medical School, Boston, MA, USA
| | - Andrew A Wagner
- Beth Israel Deaconess Medical Center, Department of Urology, Harvard Medical School, Boston, MA, USA
| | - Richard S Lee
- Boston Children's Hospital, Department of Urology, Harvard Medical School, Boston, MA, USA
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Mylonas KS, Schizas D, Economopoulos KP. Adrenal ganglioneuroma: What you need to know. World J Clin Cases 2017; 5:373-377. [PMID: 29085827 PMCID: PMC5648998 DOI: 10.12998/wjcc.v5.i10.373] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2017] [Revised: 06/14/2017] [Accepted: 07/10/2017] [Indexed: 02/05/2023] Open
Abstract
Adrenal ganglioneuromas (GNs) constitute rare, differentiated tumors which originate from neural crest cells. GNs are usually hormonally silent and tend to be discovered incidentally on imaging tests. Adrenalectomy is the gold standard for the treatment of primary adrenal GNs. Nevertheless, preoperative differential diagnosis of GNs remains extremely challenging, and thus histopathological examination is required in order to confirm the diagnosis of GN. Overall, prognosis after surgical resection seems to be excellent, without any recurrences or need for adjuvant therapy.
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Affiliation(s)
- Konstantinos S Mylonas
- Division of Pediatric Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, United States
- Surgery Working Group, Society of Junior Doctors, 11852 Athens, Greece
| | - Dimitrios Schizas
- Surgery Working Group, Society of Junior Doctors, 11852 Athens, Greece
- First Department of Surgery, Laiko General Hospital, National and Kapodistrian University of Athens, 11527 Athens, Greece
| | - Konstantinos P Economopoulos
- Surgery Working Group, Society of Junior Doctors, 11852 Athens, Greece
- Department of Surgery, Duke University Medical Center, Durham, NC 27710, United States
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Abstract
The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours. Amongst them, schwannoma and haematological tumours are newly documented. The major updates in adrenal cortical lesions are noted in the genetics of the cortical carcinoma and cortical adenoma based on the data from The Cancer Genome Atlas (TCGA). Also, a system for differentiation of oncocytoma from oncocytic cortical carcinoma is adopted. Tumours of the adrenal medulla and extra-adrenal paraganglia comprise pheochromocytoma, paraganglioma (head and neck paraganglioma and sympathetic paraganglioma), neuroblastic tumours (neuroblastoma, nodular ganglioneuroblastoma, intermixed ganglioneuroblastoma, and ganglioneuroma), composite pheochromocytoma, and composite paraganglioma. In this group, neuroblastic tumours are newly included in the classification. The clinical features, histology, associated pathologies, genetics, and predictive factors of pheochromocytoma and paraganglioma are the main changes introduced in this chapter of WHO classification of endocrine tumours. The term "metastatic pheochromocytoma/paraganglioma" is used to replace "malignant pheochromocytoma/paraganglioma." Also, composite pheochromocytoma and composite paraganglioma are now documented in separate sections instead of one. Overall, the new classification incorporated new data on pathology, clinical behaviour, and genetics of the adrenal tumours that are important for current management of patients with these tumours.
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Affiliation(s)
- Alfred King-Yin Lam
- Cancer Molecular Pathology, School of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Q4222, Australia.
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Adrenal Collision Tumor: Coexistence of Pigmented Adrenal Cortical Oncocytoma and Ganglioneuroma. Case Rep Surg 2017; 2016:5790645. [PMID: 28053800 PMCID: PMC5178330 DOI: 10.1155/2016/5790645] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2016] [Revised: 10/13/2016] [Accepted: 11/08/2016] [Indexed: 11/17/2022] Open
Abstract
Background. Adrenal collision tumors (ACTs), in which distinct tumors coexist without intermingling in the same adrenal gland, are rare and their actual prevalence is unknown. ACTs commonly consist of adrenal cortical adenoma, pheochromocytoma, or metastatic malignant tumor. Case Report. A 32-year-old woman who had been experiencing gastric discomfort for one month was referred to our hospital with abnormal imaging findings. The physical examination and the laboratory data including endocrine studies were unremarkable. Abdomen computed tomography (CT) and magnetic resonance imaging (MRI) showed two adjacent masses in the left suprarenal fossa, and a laparoscopic left adrenalectomy was done. Histological and immunohistochemical (IHC) examinations revealed two distinct tumors: a pigmented adrenal cortical oncocytoma (ACO) and a ganglioneuroma, respectively. Conclusion. Both tumors are rare in the adrenal gland and exist as ACTs only exceptionally rarely. This is the first reported case of coexisting oncocytoma and ganglioneuroma in the same adrenal gland to our knowledge.
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Khan N, Michael A, Choucair A, Bit-Ivan E. Trigeminal Ganglioneuroma: A Rare Case of Trigeminal Neuralgia Caused by Cerebellopontine Angle Tumor. World Neurosurg 2017; 99:811.e7-811.e10. [PMID: 28043883 DOI: 10.1016/j.wneu.2016.12.085] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2016] [Revised: 12/19/2016] [Accepted: 12/20/2016] [Indexed: 11/28/2022]
Abstract
BACKGROUND Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from the peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in the literature, all in East Asia. CASE DESCRIPTION A 52-year-old male presented to his primary care physician for evaluation of right facial and periorbital pain for over 18 months. He also reported a two-week history of diplopia with right lateral gaze, which resolved spontaneously. An MRI brain with and without contrast revealed a 3.0 × 2.6 × 2.4 cm enhancing extra-axial mass involving right Meckel's cave with a posterior 1.0 × 1.4 cm lobular component protruding into the prepontine cistern. The patient underwent a right middle fossa craniotomy for a subtemporal surgical resection of the tumor. Histologic section of the tumor demonstrated mature ganglion cells with surrounding satellite cells in a schwannian rich stroma, consistent with mature type ganglioneuroma. CONCLUSIONS To the best of our knowledge, this is the sixth case of trigeminal ganglioneuroma; however, it is the first case reported in the United States.
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Affiliation(s)
- Nadeem Khan
- Department of Neurology, Southern Illinois University School of Medicine, Springfield, Illinois, USA.
| | - Alex Michael
- Department of Neurosurgery, Southern Illinois University School of Medicine, Springfield, Illinois, USA
| | - Ali Choucair
- Department of Neurology, Southern Illinois University School of Medicine, Springfield, Illinois, USA
| | - Esther Bit-Ivan
- Department of Pathology, Southern Illinois University School of Medicine/Memorial Medical Center, Springfield, Illinois, USA
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Ganglioneuroma suprarrenal en una mujer gestante. Cir Esp 2017; 95:49-51. [DOI: 10.1016/j.ciresp.2016.05.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2016] [Revised: 05/16/2016] [Accepted: 05/17/2016] [Indexed: 01/11/2023]
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Deng X, Fang J, Luo Q, Tong H, Zhang W. Advanced MRI manifestations of trigeminal ganglioneuroma: a case report and literature review. BMC Cancer 2016; 16:694. [PMID: 27576442 PMCID: PMC5006494 DOI: 10.1186/s12885-016-2729-8] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2015] [Accepted: 08/19/2016] [Indexed: 12/26/2022] Open
Abstract
BACKGROUND Ganglioneuroma is a rare benign tumor originating from the sympathetic nerves, and its origination from the trigeminal nerves is even rarer. Only 4 cases of ganglioneuroma originating from the trigeminal nerve have previously been reported, and these studies only reported conventional MRI manifestations. To our knowledge, the advanced MRI features of trigeminal ganglioneuroma have not been reported thus far. CASE PRESENTATION This study reports a case of trigeminal ganglioneuroma in the left cerebellopontine angle. Advanced MRI showed the following tumor characteristics: significantly increased perfusion on perfusion imaging; isointense on diffusion-weighted imaging, whorled appearance within the tumor and no significant signs of damage to the white matter fiber tracts in the fractional anisotropy color map, and compare to the adjacent brain tissue, Choline didn't show markedly elevation, and N-acetylaspartate peak showed slightly reduction on magnetic resonance spectroscopy. The tumor was completely resected, and the diagnosis of ganglioneuroma was confirmed by postoperative pathological examination. CONCLUSION This case demonstrates the conventional as well as advanced MRI manifestations of this rare extra-axial tumor, which have never been previously reported. In addition, we reviewed the literature to demonstrate the advanced MRI features of trigeminal ganglioneuroma, in order to aid preoperative diagnosis and differentiation.
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Affiliation(s)
- Xiaojuan Deng
- Department of Radiology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, 400042, Chongqing, China
| | - Jingqin Fang
- Department of Radiology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, 400042, Chongqing, China
| | - Qingya Luo
- Department of Pathology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, China
| | - Haipeng Tong
- Department of Radiology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, 400042, Chongqing, China
| | - Weiguo Zhang
- Department of Radiology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, 400042, Chongqing, China. .,State Key Laboratory of Trauma, Burns and Combined Injury, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, China.
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Maciel CA, Tang YZ, Coniglio G, Sahdev A. Imaging of rare medullary adrenal tumours in adults. Clin Radiol 2016; 71:484-94. [PMID: 26944698 DOI: 10.1016/j.crad.2016.01.025] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2015] [Revised: 12/14/2015] [Accepted: 01/26/2016] [Indexed: 12/29/2022]
Abstract
Although adrenal medullary tumours are rare, they have important clinical implications. They form a heterogeneous group of tumours, ranging from benign, non-secretory, incidental masses to hormonally active tumours presenting acutely, or malignant tumours with disseminated disease and a poor prognosis. Increasingly, benign masses are incidentally detected due to the widespread use of imaging and routine medical check-ups. This review aims to illustrate the multimodality imaging appearances of rare adrenal medullary tumours, excluding the more common phaeochromocytomas, with clues to the diagnosis and to summarise relevant epidemiological and clinical data. Careful correlation of clinical presentation, hormone profile, and various imaging techniques narrow the differential diagnosis. Image-guided percutaneous adrenal biopsy can provide a definitive diagnosis, allowing for conservative management in selected cases. A close collaboration between the radiologist, endocrinologist, and surgeon is of the utmost importance in the management of these tumours.
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Affiliation(s)
- C A Maciel
- Department of Radiology, St Bartholomew's Hospital, Barts Health, West Smithfield, London EC1A 7BE, UK.
| | - Y Z Tang
- Department of Radiology, St Bartholomew's Hospital, Barts Health, West Smithfield, London EC1A 7BE, UK
| | - G Coniglio
- Department of Radiology, St Bartholomew's Hospital, Barts Health, West Smithfield, London EC1A 7BE, UK
| | - A Sahdev
- Department of Radiology, St Bartholomew's Hospital, Barts Health, West Smithfield, London EC1A 7BE, UK
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Imaging Features of Various Adrenal Neoplastic Lesions on Radiologic and Nuclear Medicine Imaging. AJR Am J Roentgenol 2015; 205:554-63. [PMID: 26295641 DOI: 10.2214/ajr.15.14467] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
OBJECTIVE The purpose of this review is to describe the features of diverse adrenal neoplastic lesions on radiologic and nuclear medicine imaging. CONCLUSION Various neoplastic lesions with or without malignant potential can occur in the adrenal gland. Knowledge of imaging features of adrenal lesions on radiologic and nuclear medicine imaging will facilitate differential diagnosis and assessment of malignant potential.
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Kerkhofs TM, Roumen RM, Demeyere TB, van der Linden AN, Haak HR. Adrenal tumors with unexpected outcome: a review of the literature. Int J Endocrinol 2015; 2015:710514. [PMID: 25883649 PMCID: PMC4389822 DOI: 10.1155/2015/710514] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2014] [Revised: 10/01/2014] [Accepted: 10/01/2014] [Indexed: 12/21/2022] Open
Abstract
The finding of an adrenal mass should induce a diagnostic work-up aimed at assessing autonomous hormone production and differentiating between benign and (potentially) malignant lesions. The common differential diagnosis in adrenal incidentaloma consists of (non-)functioning adenoma, pheochromocytoma, myelolipoma, metastasis, and primary carcinoma. There remains a category of lesions that are hormonally inactive and display nonspecific imaging characteristics. We provide a succinct literature review regarding pathologies from this category. Imaging and histological characteristics are discussed, as well as clinical management. In conclusion, an adrenal mass may present a diagnostic challenge. After exclusion of most common diagnoses, it can be difficult to differentiate between possible pathologies based on preoperative diagnostic tests. Surgical resection of possibly harmful tumors is indicated, for example, lesions with malignant potential or risk of spontaneous hemorrhage. Resection of an obviously benign lesion is not necessary, unless problems due to tumor size are expected.
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Affiliation(s)
- Thomas M. Kerkhofs
- Department of Internal Medicine, Máxima Medical Center, Ds. Th. Fliednerstraat 1, 5631 BM Eindhoven, The Netherlands
- *Thomas M. Kerkhofs:
| | - Rudi M. Roumen
- Department of Surgery, Máxima Medical Center, De Run 4600, 5504 DB Veldhoven, The Netherlands
- Research School GROW, School for Oncology and Developmental Biology, Maastricht University Medical Centre, P.O. Box 616, 6200 MD Maastricht, The Netherlands
| | - Thomas B. Demeyere
- Department of Pathology, Stichting PAMM, Michelangelolaan 2, 5623 EJ Eindhoven, The Netherlands
| | | | - Harm R. Haak
- Department of Internal Medicine, Máxima Medical Center, Ds. Th. Fliednerstraat 1, 5631 BM Eindhoven, The Netherlands
- Department of Internal Medicine, Division of General Internal Medicine, Maastricht University Medical Centre, P.O. Box 5800, 6202 AZ Maastricht, The Netherlands
- Department of Health Services Research and CAPHRI School for Public Health and Primary Care, Maastricht University Medical Centre, P.O. Box 616, 6200 MD Maastricht, The Netherlands
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Shawa H, Elsayes KM, Javadi S, Sircar K, Jimenez C, Habra MA. Clinical and radiologic features of pheochromocytoma/ganglioneuroma composite tumors: a case series with comparative analysis. Endocr Pract 2014; 20:864-9. [PMID: 24641930 DOI: 10.4158/ep14010.or] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVE To describe and compare the clinical, biochemical, radiologic, and pathologic features of adrenal pheochromocytoma-ganglioneuroma (PC-GN) composites with the features of isolated pheochromocytomas (PCs) and adrenal ganglioneuromas (AGNs). METHODS We reviewed data for PC-GN composite cases seen at a single tertiary center between 1993 and 2012 and compared them with cases of isolated AGN and relatively similar median-size PCs. RESULTS Nine PC-GN composites were included. The median age at diagnosis was 52 years (range, 28 to 83 years) for PC-GN compared with 55 years (range, 24 to 78 years) for PC patients and 40 years (range, 18 to 64 years) for AGN patients. Similar to PCs, all PC-GN composites were associated with catecholamine overproduction, whereas AGNs were nonfunctioning. On pathology, the median tumor sizes were 7 cm (range, 2.5 to 13 cm) for PC-GN tumors, 6.5 cm (range, 3.5 to 7 cm) for PCs, and 8 cm (range, 3.2 to 20 cm) for AGNs. On computed tomography (CT) imaging, PC-GN composites and PCs were heterogeneous, with both having significantly higher postcontrast density values than AGNs, which typically looked homogeneous and had a progressive enhancement pattern without contrast washout in most cases. CONCLUSION The presence of a PC component significantly increases tumor heterogeneity and postcontrast density values. CT imaging could be very helpful in distinguishing AGNs from both PC-GN and PC tumors, but only pathologic examination can yield the diagnosis. Clinically and radiologically, PC-GN composites are indistinguishable from PCs and need to be managed similarly.
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Affiliation(s)
- Hassan Shawa
- Department of Endocrinology, Albany Medical Center, Albany, New York
| | - Khaled M Elsayes
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Sanaz Javadi
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Kanishka Sircar
- Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Camilo Jimenez
- Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas
| | - Mouhammed Amir Habra
- Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas
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Kacagan C, Basaran E, Erdem H, Tekin A, Kayikci A, Cam K. Case report: Large adrenal ganglioneuroma. Int J Surg Case Rep 2014; 5:253-5. [PMID: 24709621 DOI: 10.1016/j.ijscr.2014.03.004] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2013] [Revised: 02/17/2014] [Accepted: 03/04/2014] [Indexed: 11/26/2022] Open
Abstract
INTRODUCTION Ganglioneuromas are localized tumors derived from neural crest tissues. Characteristically, they originate in the posterior mediastinum. Pure adrenal gangliomas are extremely rare. PRESENTATION OF CASE A left adrenal mass with the size of 68mm×50mm×86mm on magnetic resonance imaging was documented in a 53-year-old female patient. Endocrine tests revealed a non-functioning adrenal mass. The actual size of the mass was macroscopically measured to be 16cm×8.5cm×6cm after the surgery. Histopathological examination indicated ganglioneuroma. DISCUSSION Most adrenal ganglioneuromas can incorrectly be diagnosed as other adrenal tumors, since they are rare neurogenic benign tumors with no specific imaging properties. They have a slow growth pattern and usually asymptomatic. Our case represents a huge adrenal ganglioneuroma in a female patient with nondiagnostic flank pain. Radiological imaging showed a large adrenal mass with no differentiation from other adrenal tumors. Endocrine evaluation should be performed for such adrenal masses. Since our case had a relatively large size, open surgery was preferred. Pathology revealed the definitive diagnosis. CONCLUSION This case suggests that ganglioneuromas can wrongly be diagnosed as other adrenal tumors. It is significant that a proper differential diagnosis should be performed by using hormonal and imaging techniques. Nevertheless, pathological examination is usually required for definitive diagnosis.
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Affiliation(s)
- Coşkun Kacagan
- Department of Urology, Duzce University School of Medicine, Duzce, Turkey.
| | - Ekrem Basaran
- Department of Urology, Duzce University School of Medicine, Duzce, Turkey
| | - Havva Erdem
- Department of Pathology, Duzce University School of Medicine, Duzce, Turkey
| | - Ali Tekin
- Department of Urology, Duzce University School of Medicine, Duzce, Turkey
| | - Ali Kayikci
- Department of Urology, Duzce University School of Medicine, Duzce, Turkey
| | - Kamil Cam
- Department of Urology, Duzce University School of Medicine, Duzce, Turkey
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