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1
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Michalova K, Martinek P, Mezencev R, Gupta S, Williamson S, Wasco M, Mohanty S, Magi-Galluzzi C, Cañete-Portillo S, Aron M, Kandukuri S, Lobo J, Barkan GA, Kilic I, Strakova-Peterikova A, Pivovarcikova K, Michal M, Michal M, Ulbright TM, Acosta AM. Renal Juxtaglomerular Cell Tumors Exhibit Distinct Genomic and Epigenomic Features and Lack Recurrent Gene Fusions: Comprehensive Molecular Analysis of a Multi-institutional Series. Am J Surg Pathol 2025; 49:217-226. [PMID: 39726250 DOI: 10.1097/pas.0000000000002344] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2024]
Abstract
Juxtaglomerular cell tumor (JxGCT) is a rare type of renal neoplasm demonstrating morphologic overlap with some mesenchymal tumors such as glomus tumor (GT) and solitary fibrous tumor (SFT). Its oncogenic drivers remain elusive, and only a few cases have been analyzed with modern molecular techniques. In prior studies, loss of chromosomes 9 and 11 appeared to be recurrent. Recently, whole-genome analysis identified alterations involving genes of MAPK-RAS pathway in a subset, but no major pathogenic alterations have been discovered in prior whole transcriptome analyses. Considering the limited understanding of the molecular features of JxGCTs, we sought to assess a collaborative series with a multiomic approach to further define the molecular characteristics of this entity. Fifteen tumors morphologically compatible with JxGCTs were evaluated using immunohistochemistry for renin, single-nucleotide polymorphism array (SNP), low-pass whole-genome sequencing, and RNA sequencing (fusion assay). In addition, methylation analysis comparing JxGCT, GT, and SFT was performed. All cases tested with renin (n=11) showed positive staining. Multiple chromosomal abnormalities were identified in all cases analyzed (n=8), with gains of chromosomes 1p, 10, 17, and 19 and losses of chromosomes 9, 11, and 21 being recurrent. A pathogenic HRAS mutation was identified in one case as part of the SNP array analysis. Thirteen tumors were analyzed by RNA sequencing, with 2 revealing in-frame gene fusions: TFG::GPR128 (interpreted as stochastic) and NAB2::STAT6 . The latter, originally diagnosed as JxGCT, was reclassified as SFT and excluded from the series. No fusions were detected in the remaining 11 cases; of note, no case harbored NOTCH fusions previously described in GT. Genomic methylation analysis showed that JxGCT, GT, and SFT form separate clusters, confirming that JxGCT represents a distinct entity (ie, different from GT). The results of our study show that JxGCTs are a distinct tumor type with a recurrent pattern of chromosomal imbalances that may play a role in oncogenesis, with MAPK-RAS pathway activation being likely a driver in a relatively small subset.
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Affiliation(s)
- Kvetoslava Michalova
- Department of Pathology, Faculty of Medicine, Charles University
- Bioptical Laboratory, Ltd, Plzen, Czech Republic
| | | | - Roman Mezencev
- School of Biological Sciences, Georgia Institute of Technology
- Parker H. Petit Institute of Bioengineering and Biosciences, Georgia Institute of Technology, Atlanta, GA
| | - Sounak Gupta
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN
| | - Sean Williamson
- Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH
| | | | - Sambit Mohanty
- Department of Pathology, CORE Diagnostics and Advanced Medical Research Institute, Gurgaon, Haryana, India
| | | | | | - Manju Aron
- Department of Pathology, University of Southern California Keck School of Medicine, Los Angeles, CA
| | - Shivani Kandukuri
- Department of Pathology, University of Southern California Keck School of Medicine, Los Angeles, CA
| | - João Lobo
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN
- Department of Pathology, Portuguese Oncology Institute of Porto (IPO Porto)/Cancer Biology and Epigenetics Group, Research Center, Porto Comprehensive Cancer Center Raquel Seruca (P.CCC), R. Dr. António Bernardino de Almeida
- Department of Pathology and Molecular Immunology, ICBAS - School of Medicine and Biomedical Sciences, University of Porto, Porto, Portugal
| | - Güliz A Barkan
- Department of Pathology and Laboratory Medicine, Loyola University School of Medicine, Maywood, IL
| | - Irem Kilic
- Department of Pathology and Laboratory Medicine, Loyola University School of Medicine, Maywood, IL
| | - Andrea Strakova-Peterikova
- Department of Pathology, Faculty of Medicine, Charles University
- Bioptical Laboratory, Ltd, Plzen, Czech Republic
| | - Kristyna Pivovarcikova
- Department of Pathology, Faculty of Medicine, Charles University
- Bioptical Laboratory, Ltd, Plzen, Czech Republic
| | - Michael Michal
- Department of Pathology, Faculty of Medicine, Charles University
- Bioptical Laboratory, Ltd, Plzen, Czech Republic
| | - Michal Michal
- Department of Pathology, Faculty of Medicine, Charles University
- Bioptical Laboratory, Ltd, Plzen, Czech Republic
| | - Thomas M Ulbright
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN
| | - Andres M Acosta
- Department of Pathology, Indiana University School of Medicine, Indianapolis, IN
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2
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Poh EJ, Heng KH, Poh BK, Cheng L, Shi R, Tan PH. Juxtaglomerular cell tumour with unusual clear cells: a diagnostic challenge and differential diagnosis. Pathology 2025:S0031-3025(25)00051-0. [PMID: 39988491 DOI: 10.1016/j.pathol.2024.10.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/25/2024] [Revised: 10/06/2024] [Accepted: 10/25/2024] [Indexed: 02/25/2025]
Affiliation(s)
- Ee Jin Poh
- Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore
| | | | | | - Liang Cheng
- Department of Pathology and Laboratory Medicine, Department of Surgery (Urology), Brown University Warren Alpert Medical School, Lifespan Health, and the Legorreta Cancer Center at Brown University, Providence, RI, USA
| | - Ruoyu Shi
- Department of Pathology and Laboratory Medicine, Kandang Kerbau Women's and Children's Hospital, Singapore
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3
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Hogeboom A, Toldos Ó, García-Muñoz H. A locally infiltrative juxtaglomerular cell tumor with unusual histologic features. Virchows Arch 2024:10.1007/s00428-024-03938-x. [PMID: 39367921 DOI: 10.1007/s00428-024-03938-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2024] [Revised: 09/17/2024] [Accepted: 09/27/2024] [Indexed: 10/07/2024]
Abstract
Juxtaglomerular cell tumor (JGCT) is an exceptionally rare renal tumor with a predominantly benign clinical course and classically bland histology. It commonly presents in young adults and manifests as hypertension related to renin secretion. We report a JGCT initially thought to be a renal cell carcinoma. It was unique because of its size, high-grade histologic features and locally infiltrative nature-extension into the renal pelvis. It is unclear whether features such as tumor necrosis, pleomorphism and increased proliferative activity are predictive of metastatic potential and/or locally aggressive behavior. Clinical follow-up may be warranted in such cases.
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Affiliation(s)
- Adriana Hogeboom
- Department of Anatomic Pathology, San Pedro University Hospital, Logroño, Spain.
| | - Óscar Toldos
- Department of Anatomic Pathology, 12 of October University Hospital, Madrid, Spain
| | - Huberto García-Muñoz
- Department of Anatomic Pathology, 12 of October University Hospital, Madrid, Spain
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4
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Fabian B, Ragnarsson O, Prazic A, Rydén M, Volpe C, Lindgren O. Diagnostic challenges in patients with reninomas and extrarenal renin-producing tumours. Clin Endocrinol (Oxf) 2024; 101:3-9. [PMID: 38696530 DOI: 10.1111/cen.15069] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2024] [Revised: 04/20/2024] [Accepted: 04/22/2024] [Indexed: 05/04/2024]
Abstract
Renin-secreting tumours are rare causes of secondary hypertension and hypokalaemia. They are usually surgically curable, hence proper diagnostic work-up and tumour localisation is essential. In this paper, we present three Swedish patients recently diagnosed with renin secreting tumours, two with reninomas and one with an extrarenal renin-producing tumour, to illustrate diagnostic challenges. We also discuss the biochemical work-up, the pros and cons of different imaging techniques (computer tomography [CT], magnetic resonance imaging and [18F]fluorodeoxyglucose-positron emission tomography-CT), as well as how renal vein sampling (RVC) may contribute to localisation of the tumour.
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Affiliation(s)
- Botond Fabian
- Department of Endocrinology, Skåne University Hospital, Lund, Sweden
| | - Oskar Ragnarsson
- Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
- Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden
- Wallenberg Centre for Molecular and Translational Medicine, University of Gothenburg, Gothenburg, Sweden
| | | | - Mikael Rydén
- Department of Medicine (H7), Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden
| | - Cristina Volpe
- Department of Molecular Medicine and Surgery, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden
| | - Ola Lindgren
- Department of Endocrinology, Skåne University Hospital, Lund, Sweden
- Department of Clinical Sciences in Lund, Lund University, Lund, Sweden
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5
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Dong H, Zuo Y, An X, Li H, Zheng Z, Chen Y, Zou Y, Jiang X. Clinical features, laboratory findings and treatment of juxtaglomerular cell tumors: a systemic review. Hypertens Res 2024; 47:1380-1390. [PMID: 38438720 DOI: 10.1038/s41440-024-01606-w] [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: 09/15/2023] [Revised: 01/06/2024] [Accepted: 01/17/2024] [Indexed: 03/06/2024]
Abstract
Juxtaglomerular cell tumors (JGCTs) or reninoma are rare kidney tumors leading to secondary hypertension, and the non-specific clinical manifestations bring about challenges to the diagnosis. This study is to summarize the clinical features, laboratory findings, and treatment of JGCTs. The PubMed, EMBASE database, and manual search were utilized to find all cases, and 158 reports containing 261 patients were identified. Data on patients' demographics, clinical features, diagnostic methods, and treatment options were collected and analyzed. JGCTs occurred predominantly in female patients (female to male ratio, 2.1:1). The median age of patients was 25 years (IQR:18-34 years). Hypertension (97.24%) was the cardinal manifestation. Hypokalemia was reported in 78.71% (159/202) of subjects, and normal serum potassium accounted for 20.79% (42/202). In cases with assessed plasma renin activity (PRA) levels, the median PRA was 7.89 times the upper limit of normal (IQR:3.58-14.41), and 3.82% (5/131) of cases in the normal range. Tumors were detected in 97.8% (175/179) computed tomography (CT), 94.7% (72/76) magnetic resonance imaging (MRI), and 81.5% (110/135) ultrasound, respectively. For 250/261 patients undergoing surgical procedures, 89.14% (197/221), 94.94% (150/158), and 100% (131/131) of patients were restored to normal blood pressure, PRA, and serum potassium, respectively. JGCTs are commonly associated with hypertension, hypokalemia, and hyperreninemia, whereas patients with normotension, normokalemia, and PRA should be systematically pursued after drug-elution lasting for 2 weeks. CT and MRI are more sensitive imaging diagnostic methods. The blood pressure and biochemical parameters of most patients returned to normal after surgery.
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Affiliation(s)
- Hui Dong
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yujie Zuo
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xuanqi An
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hongwu Li
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhihao Zheng
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yang Chen
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yubao Zou
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiongjing Jiang
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
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Wang L, Li M, Jin S, Ouyang Y, Wang F, Lv K, Li J, Jiang Y, Liu H, Zhu Q. How to identify juxtaglomerular cell tumor by ultrasound: a case series and review of the literature. BMC Med Imaging 2024; 24:46. [PMID: 38365645 PMCID: PMC10870572 DOI: 10.1186/s12880-024-01220-9] [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: 02/28/2023] [Accepted: 02/02/2024] [Indexed: 02/18/2024] Open
Abstract
PURPOSE To study the value of ultrasound in the diagnosis of juxtaglomerular cell tumor (JGCT). METHODS From January 2005 to July 2020, fifteen patients diagnosed as JGCT by surgical pathology in Peking Union Medical College Hospital were collected. All patients underwent preoperative ultrasound examination. The clinical, laboratory, ultrasound, computed tomography (CT), surgical, and pathological features of the patients were analyzed retrospectively. RESULTS The 15 patients were 5 males and 10 females with a median age of 29 years (10∼72 years). 14 of them had hypertension and one had normal blood pressure. The tumors were all solitary, with a median diameter of 1.5 cm (0.9-5.9 cm). Among the fifteen patients, eleven were correctly detected by preoperative ultrasound, and four were missed. There was a significant difference in tumor size (2.64 ± 1.48 cm vs. 1.23 ± 0.21 cm) and whether the tumor protruded outward (9/11 vs. 0/4) between the ultrasound-detected group and the ultrasound-missed group (p = 0.010, p = 0.011). Of the 11 tumors detected by ultrasound, four were extremely hypoechoic, two were hypoechoic, three were isoechoic, and two were hyperechoic. Color Doppler showed no blood flow in five tumors with the size range from 0.9 to 2.0 cm, and mild blood flow in six tumors with the size range from 2.8 to 5.9 cm. CONCLUSIONS JGCT is rare, and has characteristic clinical manifestations. Diagnosis should be suspected in case of secondary hypertension, particularly in young women, if no renal vascular cause was found. Ultrasound, combined with clinical manifestations, was helpful for the diagnosis.
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Affiliation(s)
- Li Wang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
- Department of Ultrasound, Tangshan Central Hospital, West of Youyi Road, Lubei District, 063000, Tangshan City, Hebei, China
| | - Meiying Li
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - Siqi Jin
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - Yunshu Ouyang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - Fenglan Wang
- Department of Ultrasound, Tangshan Central Hospital, West of Youyi Road, Lubei District, 063000, Tangshan City, Hebei, China
| | - Ke Lv
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - Jianchu Li
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - Yuxin Jiang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China
| | - He Liu
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China.
| | - Qingli Zhu
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1, Dongcheng District, 100730, Beijing, China.
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7
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Ruan Y, Lian F, Tian Y, Li Q, Huang X. A case of pediatric secondary hypertension caused by juxtaglomerular cell tumor. Quant Imaging Med Surg 2023; 13:6296-6300. [PMID: 37711796 PMCID: PMC10498206 DOI: 10.21037/qims-22-1222] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/05/2022] [Accepted: 06/26/2023] [Indexed: 09/16/2023]
Affiliation(s)
- Yanan Ruan
- Department of Radiology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Fengyu Lian
- Department of Radiology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Yanjin Tian
- Department of Radiology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Qing Li
- Department of Pathology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
| | - Xiaoning Huang
- Department of Radiology, The Second Affiliated Hospital of Nanchang University, Nanchang, China
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8
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Fu X, Deng G, Wang K, Shao C, Xie LP. Pregnancy complicated by juxtaglomerular cell tumor of the kidney: A case report. World J Clin Cases 2023; 11:2541-2548. [PMID: 37123308 PMCID: PMC10130993 DOI: 10.12998/wjcc.v11.i11.2541] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Revised: 01/27/2023] [Accepted: 03/17/2023] [Indexed: 04/06/2023] Open
Abstract
BACKGROUND Juxtaglomerular cell tumor (JGCT) of the kidney, also known as reninoma, is a rare renal tumor that typically clinically manifests as hypertension, hypokalemia, high renin, and high aldosterone. It is a cause of secondary hypertension. Pregnancy with JGCT is rarer and easily misdiagnosed as pregnancy-induced hypertension, thus affecting treatment.
CASE SUMMARY A 28-year-old woman presented in early pregnancy with hypertension (blood pressure of 229/159 mmHg), nausea, and occasional dizziness and headache. The patient was diagnosed with pregnancy-induced hypertension, and no relief was found after symptomatic treatment; hence, the pregnancy was terminated by artificial abortion. Her blood pressure remained high following termination of pregnancy. Blood tests suggested hypokalemia (2.997 mmol/L), blood aldosterone measured 613 ng/L, and computed tomography urography showed a tumor in the right kidney. Therefore, laparoscopic partial nephrectomy was performed. After surgery, the patient’s blood pressure returned to normal, and blood potassium, aldosterone, and renin normalized. Postoperative pathological examination revealed JGCT. After long-term follow-up, the patient became pregnant again 6 mo after surgery. No hypertension occurred during pregnancy, and the patient delivered a healthy female neonate.
CONCLUSION Patients with pregnancy complicated by JGCT are difficult to diagnose. Herein, we advise surgeons on proper handling of such situations.
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Affiliation(s)
- Xian Fu
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Gang Deng
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Kai Wang
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Chang Shao
- Department of Pathology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Li-Ping Xie
- Department of Urology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China
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Sakiyama H, Hamada S, Oshiro T, Hyakuna N, Kuda M, Hishiki T, Aoyama H, Kuroda N, Yorita K, Wada N, Yoshioka T, Koga Y, Nakanishi K. Juxtaglomerular cell tumor with pulmonary metastases: A case report and review of the literature. Pediatr Blood Cancer 2023; 70:e30068. [PMID: 36458675 DOI: 10.1002/pbc.30068] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2022] [Revised: 09/23/2022] [Accepted: 09/26/2022] [Indexed: 12/04/2022]
Affiliation(s)
- Hideki Sakiyama
- Department of Pediatrics, University of the Ryukyus Hospital, Nishihara, Japan
| | - Satoru Hamada
- Department of Pediatrics, University of the Ryukyus Hospital, Nishihara, Japan.,Department of Child Health and Welfare, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
| | - Tokiko Oshiro
- Department of Pediatrics, University of the Ryukyus Hospital, Nishihara, Japan.,Department of Child Health and Welfare, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
| | - Nobuyuki Hyakuna
- Department of Pediatrics, University of the Ryukyus Hospital, Nishihara, Japan.,Department of Child Health and Welfare, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
| | - Masaaki Kuda
- Department of Digestive and General Surgery Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
| | - Tomoro Hishiki
- Department of Pediatric Surgery, Chiba University Graduate School of Medicine, Chiba, Japan
| | - Hajime Aoyama
- Department of Pathology, Heartlife Hospital, Okinawa, Japan
| | - Naoto Kuroda
- Department of Internal Medicine, Kinro Hospital, Kochi, Japan
| | - Kenji Yorita
- Department of Diagnostic Pathology, Japanese Red Cross Kochi Hospital, Kochi, Japan
| | - Naoki Wada
- Department of Pathology and Oncology, Graduate School of Medicine, University of Ryukyus, Nishihara, Japan
| | - Takako Yoshioka
- Department of Pathology, National Center for Child Health and Development, Tokyo, Japan
| | - Yuhki Koga
- Department of Pediatrics, Graduate School of Medical Science, Kyushu University, Fukuoka, Japan
| | - Koichi Nakanishi
- Department of Pediatrics, University of the Ryukyus Hospital, Nishihara, Japan.,Department of Child Health and Welfare, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
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Sawamura M, Sawa N, Oshima Y, Ikuma D, Yamanouchi M, Hayami N, Sekine A, Mizuno H, Hasegawa E, Suwabe T, Hoshino J, Kono K, Kinowaki K, Ohashi K, Ubara Y. A case of malignant nephrosclerosis occurring with serum renin in the normal range. CEN Case Rep 2023; 12:116-121. [PMID: 36038752 PMCID: PMC9892380 DOI: 10.1007/s13730-022-00726-x] [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: 06/01/2022] [Accepted: 07/28/2022] [Indexed: 02/05/2023] Open
Abstract
A 37-year-old Japanese man was admitted to our hospital for evaluation of severe hypertension and visual impairment. His serum creatinine was 4.16 mg/dL. Plasma renin activity was normal (2.7 ng/mL/h), but plasma aldosterone concentration was elevated (27.2 ng/dL). A kidney biopsy showed concentric subendothelial edematous thickening of the arterioles (onion skin pattern) with luminal narrowing or obstruction, and malignant nephrosclerosis was diagnosed. Antihypertensive therapies, including an angiotensin II receptor blocker and spironolactone, were administered and effectively preserved kidney function and normalized blood pressure. This case indicates that hyperaldosteronemia in the presence of normal renin levels might also cause malignant hypertension.
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Affiliation(s)
- Masato Sawamura
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Naoki Sawa
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan
| | - Yoichi Oshima
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Daisuke Ikuma
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Masayuki Yamanouchi
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Noriko Hayami
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Akinari Sekine
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Hiroki Mizuno
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Eiko Hasegawa
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
| | - Tatsuya Suwabe
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan
| | - Junichi Hoshino
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan
| | - Kei Kono
- Department of Pathology, Toranomon Hospital, Tokyo, Japan
| | | | - Kenichi Ohashi
- Department of Pathology, Toranomon Hospital, Tokyo, Japan
- Department of Human Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
| | - Yoshifumi Ubara
- Nephrology Center and Department of Rheumatology, Toranomon Hospital, Kajigaya, 1-3-1, Takatsu, Kawasaki, Tokyo, Kanagawa, 212-0015, Japan.
- Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan.
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11
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Quach P, Hamza A. Juxtaglomerular cell tumor: report of a case with unusual presentation. Autops Case Rep 2022; 12:e2021406. [PMID: 36312876 PMCID: PMC9613376 DOI: 10.4322/acr.2021.406] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2022] [Accepted: 10/12/2022] [Indexed: 11/05/2022]
Abstract
Juxtaglomerular cell tumor is a benign, renin-secreting neoplasm. The tumor arises from the juxtaglomerular apparatus cells of the kidney. Because the tumor is hormonally active, patients usually suffer from hypokalemia, hyperaldosteronism, and hypertension. Herein, we describe a case of a 19-year-old Asian female with a somewhat unusual presentation. A 19-year-old Asian female presented with upper extremity weakness, numbness, and tingling. On physical examination, the only notable finding was hypertension. Extensive workup revealed elevated aldosterone level and plasma renin activity. CT scan of the abdomen revealed a 2.2 cm mass in the lower pole of the left kidney. The mass was resected by partial nephrectomy. On microscopic evaluation, the tumor had glomoid appearance with sheets of uniform, round to polygonal cells with clear to eosinophilic cytoplasm. Immunohistochemical stains showed the tumor cells to be positive for CD117, CD34 and CD10 and negative for ER, PR, CK7, PAX-8, pan-cytokeratin, EMA, S100, Melan-A, HMB45, SMA and CAIX. Diagnosis of Juxtaglomerular cell tumor was rendered. This case highlights the importance of a regular physical exam and a high index of suspicion in patients presenting with unusual complaints.
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Affiliation(s)
- Priscilla Quach
- University of Kansas Medical Center, Department of Pathology & Laboratory Medicine, Kansas City, Kansas, USA
| | - Ameer Hamza
- University of Kansas Medical Center, Department of Pathology & Laboratory Medicine, Kansas City, Kansas, USA
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Skarakis NS, Papadimitriou I, Papanastasiou L, Pappa S, Dimitriadi A, Glykas I, Ntoumas K, Lampropoulou P, Kounadi T. Juxtaglomerular cell tumour of the kidney: a rare cause of resistant hypertension. Endocrinol Diabetes Metab Case Rep 2022; 2022:EDM210042. [PMID: 35023474 PMCID: PMC8789008 DOI: 10.1530/edm-21-0042] [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: 09/05/2021] [Accepted: 12/21/2021] [Indexed: 11/08/2022] Open
Abstract
SUMMARY Juxtaglomerular cell tumour (JGCT) is an unusually encountered clinical entity. A 33-year-old man with severe long-standing hypertension and hypokalaemia is described. The patient also suffered from polyuria, polydipsia, nocturia and severe headaches. On admission, laboratory investigation revealed hypokalaemia, kaliuresis, high aldosterone and renin levels, and the abdomen CT identified a mass of 4 cm at the right kidney. Kidney function was normal. Following nephrectomy, the histological investigation revealed the presence of a JGCT. Immunostaining was positive for CD34 as well as for smooth muscle actin and vimentin. Following surgery, a marked control of his hypertension with calcium channel blockers and normalization of the serum potassium, renin or aldosterone levels were reached. According to our findings, JGCT could be included in the differential diagnosis of secondary hypertension as it consists of a curable cause. The association of JGCT with hypertension and hypokalaemia focusing on the clinical presentation, diagnostic evaluation and management is herein discussed and a brief review of the existing literature is provided. LEARNING POINTS Juxtaglomerular cell tumours (JGCT), despite their rarity, should be included in the differential diagnosis of secondary hypertension as they consist of a curable cause of hypertension. JGCT could be presented with resistant hypertension along with hypokalaemia, kaliuresis and metabolic alkalosis. Early recognition and management can help to prevent cardiovascular complications. Imaging (enhanced CT scans) may be considered as the primary diagnostic tool for the detection of renal or JGCT. For the confirmation of the diagnosis, a histopathologic examination is needed.
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Affiliation(s)
- Nikitas S Skarakis
- Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece
| | - Irene Papadimitriou
- Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece
| | - Labrini Papanastasiou
- Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece
| | - Sofia Pappa
- Department of Pathology, ‘G. Gennimatas’ General Hospital, Athens, Greece
| | | | - Ioannis Glykas
- Department of Urology, General Hospital of Athens ‘G Gennimatas’, Athens, Greece
| | - Konstantinos Ntoumas
- Department of Urology, General Hospital of Athens ‘G Gennimatas’, Athens, Greece
| | | | - Theodora Kounadi
- Unit of Endocrinology and Diabetes Center, ‘G. Gennimatas’ General Hospital, Athens, Greece
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13
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Zimmermann Y, Tawadros C, Andrejevic-Blant S, Vogel G. [A rare cause of hypertension with hypokalemia: A case of reninoma]. Nephrol Ther 2021; 17:538-542. [PMID: 34334338 DOI: 10.1016/j.nephro.2021.05.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2021] [Revised: 05/17/2021] [Accepted: 05/27/2021] [Indexed: 11/17/2022]
Abstract
Reninoma or juxtaglomerular cell tumor is a rare usually benign renal renin secreting tumor. We report the case of an 18-year old woman, without any medical history, investigated in our hospital's emergency department for a bilateral papilledema. Ambulatory ophthalmological investigations were performed because of a newly occurring blurry vision, associated with diffuse headaches. Cerebral mRI and lumbar puncture recommended by the ophthalmologist and neurologist excluded intra-cranial hypertension. The patient presented with severe hypertension. Laboratory values showed hypokalemia, compensated metabolic alkalosis and microalbuminuria. During the hospital stay, she developed AKIN 1 acute renal injury. Ultrasound revealed a tissular cystic lesion of the superior pole of the right kidney. Abdominal mRI confirmed the lesion and raised suspicion for a renal cell carcinoma without calicial or vascular invasion. Plasma renin value was >500 mUI/L with normal values for plasma aldosterone. Renal biopsy diagnosed a juxtaglomerular cell tumor. After an aggressive initial treatment, hypertension remained well controlled with spironolactone only, finally allowing for withdrawal of all antihypertensive medications. Robot-assisted laparoscopic partial nephrectomy was performed. Studies of the operative specimen confirmed the diagnosis of benign reninoma. Clinical follow-up showed complete resolution of clinical and biological parameters.
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Affiliation(s)
- Yaël Zimmermann
- Service de néphrologie, hôpital Riviera-Chablais site de Rennaz, route du Vieux Sequoia 20, 1847 Rennaz, Suisse
| | - Cécile Tawadros
- Service d'urologie, hôpital Riviera-Chablais site de Rennaz, 1847 Rennaz, Suisse
| | | | - Gérard Vogel
- Service de néphrologie, hôpital Riviera-Chablais site de Rennaz, route du Vieux Sequoia 20, 1847 Rennaz, Suisse.
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14
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Zhang R, Xu M, Xie XY. The Role of Real-Time Contrast-Enhanced Ultrasound in Guiding Radiofrequency Ablation of Reninoma: Case Report and Literature Review. Front Oncol 2021; 11:585257. [PMID: 33686368 PMCID: PMC7935754 DOI: 10.3389/fonc.2021.585257] [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: 07/20/2020] [Accepted: 01/06/2021] [Indexed: 11/23/2022] Open
Abstract
BACKGROUND Reninoma is a rare renal endocrine tumor that can cause secondary hypertension, characterized by hypertension, hypokalemia, high renin and aldosterone with normal aldosterone renin ratio (ARR), and occurs more in young female. Mainstream treatment option is surgery, but is less suitable for small or deep lesions, which makes ablation a promising alternative. CASE PRESENTATION Two young female with typical manifestations of reninoma, including hypertension, hypokalemia, high renin, high aldosterone and normal ARR, were treated successfully with real-time contrast-enhanced ultrasound guided radiofrequency ablation, and contrast-enhanced ultrasound was also performed before and after treatment for diagnosis and postoperative assessment. Afterward, their blood pressure and laboratory tests became normal and remained steady during the follow-up of 32 and 6 months, respectively. CONCLUSION Contrast-enhanced ultrasound guided radiofrequency ablations is a promising alternative for reninoma treatment with comparable safety and efficacy with surgery, and has advantages especially in small or deep lesions.
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Affiliation(s)
| | - Ming Xu
- Department of Medical Ultrasonics, The First Affiliated Hospital, Institute of Diagnostic and Interventional Ultrasound, Sun Yat-sen University, Guangzhou, China
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Spindle Cell Hemangioma and Atypically Localized Juxtaglomerular Cell Tumor in a Patient with Hereditary BRIP1 Mutation: A Case Report. Genes (Basel) 2021; 12:genes12020220. [PMID: 33546375 PMCID: PMC7913579 DOI: 10.3390/genes12020220] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2020] [Revised: 01/28/2021] [Accepted: 01/29/2021] [Indexed: 11/17/2022] Open
Abstract
Spindle cell hemangioma is a benign vascular tumor typically occurring in the dermis or subcutis of distal extremities as red-brown lesions that can grow in both size and number over time. They can be very painful and potentially disabling. A family history of cancer or previous history may be relevant and must be taken into consideration. Juxtaglomerular cell tumor (reninoma) is an extremely rare cause of secondary hypertension diagnosed mostly among adolescents and young adults. Excessive renin secretion results in secondary hyperaldosteronism. Subsequent hypokalemia and metabolic alkalosis, together with high blood pressure, are clues for clinical diagnosis. Histological examination of the excised tumor leads to a definitive diagnosis. Reninoma is found in subcapsular localization, in most cases as a solitary mass, in imaging studies of kidneys. Exceptionally, it can be located in another part of a kidney. Both spindle cell hemangioma and reninoma are extremely rare tumors in children and adolescents. Herein, the authors present a case report of a patient with hereditary BRCA1 interacting protein C-terminal helicase 1 (BRIP1) mutation, spindle cell hemangioma, and secondary hypertension caused by atypically localized reninoma.
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A case of juxtaglomerular cell tumor with an unusual clinical presentation. HUMAN PATHOLOGY: CASE REPORTS 2020. [DOI: 10.1016/j.ehpc.2020.200446] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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Jiang S, Yang Y, Wu R, Yang Q, Zhang C, Tang Y, Mo C. Characterization and Management of Juxtaglomerular Cell Tumor: Analysis of 9 Cases and Literature Review. Balkan Med J 2020; 37:287-290. [PMID: 32441904 PMCID: PMC7424182 DOI: 10.4274/balkanmedj.galenos.2020.2019.12.79] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
Abstract
Aims: Juxtaglomerular cell tumor is a rare kidney tumor. This study aimed to report the clinic features of juxtaglomerular cell tumor and our treatment experience. Methods: The medical records of 9 juxtaglomerular cell tumor patients treated in our hospital from 1997 to 2017 were retrospectively reviewed. Clinical characteristics, immunohistochemical findings, treatments and outcomes were collected. Results: The mean age of 9 patients was 24±8.1 years (range: 18-37). All cases had symptoms of hypertension, hyperaldosteronism, high plasma renin, high plasma angiotensin II. Four cases had hypokalemia. The renal masses were found by enhanced contrast tomography in all patients. One case received ultrasound-guided ablation and was clinically diagnosed with juxtaglomerular cell tumor. Among the remaining 8 cases, 2 cases received nephrectomy while 6 underwent partial nephrectomy. The 8 cases were pathologically diagnosed with juxtaglomerular cell tumor. Immunohistochemical findings showed that juxtaglomerular cell tumor was positive for vimentin, CD34, and actin but negative for chromogranin A. After treatment, all the patients had normal levels of blood pressure, serum renin activity, potassium, and aldosterone. No patients had tumor progress or metastasis within a median follow-up period of 94 (range: 33-241) months. Conclusion: Hypertension combined with hyperaldosteronism and hypokalemia secondary to high plasma renin activity are the typical symptoms of juxtaglomerular cell tumor. Partial nephrectomy is an optimal treatment for juxtaglomerular cell tumor.
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Affiliation(s)
- Shuangjian Jiang
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Yun Yang
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Rongpei Wu
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Qiyun Yang
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Chi Zhang
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Yiming Tang
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
| | - Chengqiang Mo
- Department of Urology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
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Hagiya A, Zhou M, Hung A, Aron M. Juxtaglomerular Cell Tumor With Atypical Pathological Features: Report of a Case and Review of Literature. Int J Surg Pathol 2019; 28:87-91. [DOI: 10.1177/1066896919868773] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Juxtaglomerular cell tumor (JGCT) is a rare renal tumor with a predominantly benign clinical course. It affects young adults, who often present with hypertension, hypokalemia, and hyperaldosteronism. The tumor cells are round to spindle-shaped with occasional mild to moderate atypia, but mitotic figures are usually absent. Surgical resection is the treatment of choice. Typically, the blood pressure and renin levels normalize after removal of the tumor. Rare cases of metastatic and recurrent JGCT have been reported including cases with vascular invasion. These cases typically occur in older adults and present with larger tumor size (9-15 cm). We report a case of JGCT, 5.5 cm in greatest dimension, with atypical pathological features including invasion of the renal vein, lymphovascular invasion, and significant pleomorphism with rhabdoid morphology, along with a brief review of the literature.
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Affiliation(s)
- Ashley Hagiya
- University of Southern California, Los Angeles, CA, USA
| | - Ming Zhou
- UT Southwestern Medical Center, Dallas, TX, USA
| | - Andrew Hung
- University of Southern California, Los Angeles, CA, USA
| | - Manju Aron
- University of Southern California, Los Angeles, CA, USA
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A 10-year history of secondary hypertension: a challenging case of renin-secreting juxtaglomerular cell tumor. J Hypertens 2019; 36:1772-1774. [PMID: 29952855 DOI: 10.1097/hjh.0000000000001774] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Case Report of Atypical Juxtaglomerular Cell Tumor. Case Rep Pathol 2018; 2018:6407360. [PMID: 30473900 PMCID: PMC6220404 DOI: 10.1155/2018/6407360] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2018] [Revised: 10/14/2018] [Accepted: 10/17/2018] [Indexed: 11/17/2022] Open
Abstract
Juxtaglomerular cell tumor (JGCT) is a rare renal tumor, producing renin and behaving almost in a benign fashion. So far, only three cases have been reported as malignant. We report a rare case with atypical JGCT. A 74-year-old male was referred to our hospital due to hypertension, proteinuria, and hematuria. Abdominal CT revealed a mass measured in 9.7×7.0 cm in the lower portion of the right kidney. Right kidney was removed laparoscopically. Grossly, white to tan tumor with massive hemorrhage and necrosis occupied the lower portion of the right kidney. Microscopically, tumor grew in a solid fashion. Tumor cells were polygonal to ovoid cells with round nuclei and clear to eosinophilic cytoplasm. Mitosis was found in 5 per 10 HPF. Immunohistochemically, tumor cells were stained by vimentin and CD34. Some tumor cells were also positive for renin. Electron micrograph showed near rhomboid crystalline structure in the tumor cells. Because of massive necrosis and mitotic figures, diagnosis of atypical (potentially malignant) JGCT was rendered. Gene mutations for IDH1, PIK3CA, K-ras, N-ras, Braf, and EGFR were not found by MBP-QP system.
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21
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Hommos MS, Schwartz GL. Clinical value of plasma renin activity and aldosterone concentration in the evaluation of secondary hypertension, a case of reninoma. ACTA ACUST UNITED AC 2018; 12:641-643. [DOI: 10.1016/j.jash.2018.06.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2018] [Accepted: 06/01/2018] [Indexed: 11/26/2022]
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22
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Wang F, Shi C, Cui Y, Li C, Tong A. Juxtaglomerular cell tumor: Clinical and immunohistochemical features. J Clin Hypertens (Greenwich) 2017; 19:807-812. [PMID: 28317244 DOI: 10.1111/jch.12997] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2017] [Revised: 02/28/2017] [Accepted: 01/24/2017] [Indexed: 11/29/2022]
Abstract
Juxtaglomerular cell tumor (JGCT) is a rare tumor, with approximately 100 cases reported in the literature. The authors respectively studied the clinical data of 11 patients diagnosed with JGCT in Peking Union Medical College Hospital from 2004 to 2014, and investigated the immunohistochemical profiles in 10 tumors. Nine of the 11 patients were diagnosed before the age of 40 years. Hypertension was present in all patients, while hypokalemia occurred in seven of 11 patients. Computed tomography detected JGCTs with a sensitivity of 100%. Immunoreactivities for CD34 and vascular endothelial growth factor were observed in most tumor specimens, suggesting that JGCTs express a variety of vessel-related immunohistochemical markers, although JGCTs are considered a tumor without abundant blood supply. Nuclear accumulation of cyclin D1 was common in JGCTs. Results from immunohistochemistry were negative for BRAF, HER2, and TFE3, suggesting that BRAF, HER2, and TFE3 genes might not play a part in tumorigenesis in JGCTs.
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Affiliation(s)
- Fen Wang
- Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Chuan Shi
- Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Yunying Cui
- Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Chunyan Li
- Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
| | - Anli Tong
- Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China
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Xue M, Chen Y, Zhang J, Guan Y, Yang L, Wu B. Reninoma coexisting with adrenal adenoma during pregnancy: A case report. Oncol Lett 2017; 13:3186-3190. [PMID: 28521424 DOI: 10.3892/ol.2017.5802] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2016] [Accepted: 12/20/2016] [Indexed: 11/05/2022] Open
Abstract
Reninoma is a rare form of secondary hypertension. The present study reported a case of reninoma that coexisted with an adrenal adenoma in a young female with secondary hypertension during pregnancy. The patient, a 31-year-old female exhibiting hypertension for >1 year, developed a mass in the right adrenal gland, which was detected by an ultrasound scan in the 33rd gestational week. In addition, a well-defined solid mass on the left kidney was detected by a magnetic resonance imaging scan1 month subsequent to the termination of the pregnancy. The blood pressure (BP) of the patient did not change subsequent to the laparoscopic right adrenalectomy. The patient subsequently underwent laparoscopic left partial nephrectomy 2 months later. The BP levels demonstrated a significant reduction from 177/115 to 125/80 mmHg in the same day postoperatively, and stabilized to be within the normal range. The BP level of the patient remained within the normal range subsequent to a 6-month follow-up. Pathological analysis revealed reninoma on the left kidney. The present study aimed to provide information for radiologists who may encounter this type of benign tumor in the future, which exhibits elevated BP levels.
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Affiliation(s)
- Mei Xue
- Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
| | - Yan Chen
- Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
| | - Jin Zhang
- Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
| | - Youyan Guan
- Department of Urology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
| | - Lin Yang
- Department of Pathology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
| | - Bing Wu
- Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Beijing 100021, P.R. China
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Kang S, Guo A, Wang H, Ma L, Xie Z, Li J, Tonge X, Ye H. Magnetic Resonance Imaging Features of a Juxtaglomerular Cell Tumor. J Clin Imaging Sci 2015; 5:68. [PMID: 26900492 PMCID: PMC4736060 DOI: 10.4103/2156-7514.172976] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2015] [Accepted: 12/24/2015] [Indexed: 01/25/2023] Open
Abstract
OBJECTIVE To retrospectively determine whether magnetic resonance imaging (MRI) findings can help differentiate a juxtaglomerular cell tumor (JCT) from clear cell renal cell carcinoma (ccRCC). MATERIALS AND METHODS Eight patients with JCTs and 24 patients with pathologically proven ccRCC were included for image analysis. All patients underwent unenhanced MRI and dynamic contrast-enhanced MRI. Fat-suppressed T2-weighted imaging (T2WI), diffusion-weighted imaging (DWI), in- and opposed-phase imaging, and fat-suppressed preliver acquisitions with volume acceleration sequences were performed before enhancement. After the administration of contrast, dynamic imaging was performed in the corticomedullary, nephrographic, and excretory phases. Student's t-test, t'-test, Chi-square test, and nonparametric Kruskal-Wallis H-test were used to determine the significance of the difference between the two groups. The sensitivity and specificity of the MRI findings were calculated. RESULTS In patients with a JCT, a cystic part of the lesion of <10%, isointensity or mild hyperintensity on T2WI, heterogeneous hyperintensity on DWI, less signal drop (<10%) in in- and opposed-phase imaging, and a degree of enhancement <200% in the corticomedullary phase showed statistically significant differences compared with those of ccRCC (P < 0.05). After combining a lower apparent diffusion coefficient (ADC) value (heterogeneous hyperintensity) on DWI and a degree of enhancement <200% in the corticomedullary phase using a parallel test, the sensitivity and specificity were 90.9% and 91.7%, respectively. CONCLUSIONS Isointensity or mild hyperintensity on T2WI, a lower ADC value (heterogeneous hyperintensity) on DWI, and a degree of enhancement <200% in the corticomedullary phase are the major MRI findings for JCTs, combined with relative clinical manifestations and excluding other renal masses. A main solid tumor, less signal drop (<10%) in in- and opposed-phase imaging, and a less-washout pattern of <10% in the delayed phase are secondary MRI findings for JCTs.
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Affiliation(s)
- Suhai Kang
- Department of Radiology, Chinese PLA General Hospital, Beijing, PR China
| | - Aitao Guo
- Department of Pathology, Chinese PLA General Hospital, Beijing, PR China
| | - Haiyi Wang
- Department of Radiology, Chinese PLA General Hospital, Beijing, PR China
| | - Lu Ma
- Department of Radiology, Chinese PLA General Hospital, Beijing, PR China
| | - Zongyu Xie
- Department of Radiology, First Affiliated Hospital of Bengbu Medical College, Anhui Province, PR China
| | - Jinglong Li
- Department of MRI, First Hospital of Qinhuangdao, Hebei Province, PR China
| | - Xinyuan Tonge
- Statistical Teaching and Research Section, Chinese PLA General Hospital, Beijing, PR China
| | - Huiyi Ye
- Department of Radiology, Chinese PLA General Hospital, Beijing, PR China
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Rosei CA, Giacomelli L, Salvetti M, Paini A, Corbellini C, Tiberio G, Muiesan ML. Advantages of renin inhibition in a patient with reninoma. Int J Cardiol 2015; 187:240-242. [PMID: 25838223 DOI: 10.1016/j.ijcard.2015.03.280] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2015] [Accepted: 03/19/2015] [Indexed: 10/23/2022]
Affiliation(s)
| | - Laura Giacomelli
- Department of Clinical & Experimental Sciences, University of Brescia, Italy
| | - Massimo Salvetti
- Department of Clinical & Experimental Sciences, University of Brescia, Italy
| | - Anna Paini
- Department of Clinical & Experimental Sciences, University of Brescia, Italy
| | - Claudia Corbellini
- Department of Clinical & Experimental Sciences, University of Brescia, Italy
| | - Guido Tiberio
- Department of Clinical & Experimental Sciences, University of Brescia, Italy
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[Case of juxtaglomerular cell tumor (reninoma) treated with laparoscopic partial nephrectomy]. Nihon Hinyokika Gakkai Zasshi 2015; 105:202-6. [PMID: 25757351 DOI: 10.5980/jpnjurol.105.202] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
We report a case of a 22-year-old male with juxtaglomerular cell tumor treated with laparoscopic partial nephrectomy. He was referred to our hospital with hypertension, high concentration of plasma renin activity (PRA) and renal mass. Dynamic enhanced computed tomography showed 17-mm weak contrast-enhancing tumor at the upper pole of the left kidney. Renin suppression and stimulation test revealed autonomous renin secretion although renal venous sampling failed to show significant difference in the PRA between the right and left renal vein. We performed laparoscopic left partial nephrectomy. The histological diagnosis was juxtaglomerular cell tumor. After the operation, his blood pressure and PRA were immediately normalized. Juxtaglomerular cell tumor is an important renal tumor as a curable cause of secondary hypertension.
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Mao J, Wang Z, Wu X, Dai W, Tong A. Recurrent hypertensive cerebral hemorrhages in a boy caused by a reninoma: rare manifestations and distinctive electron microscopy findings. J Clin Hypertens (Greenwich) 2012; 14:802-5. [PMID: 23126354 DOI: 10.1111/jch.12015] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
Recurrent cerebral hemorrhages caused by hypertension secondary to reninoma are extremely rare in children. Because of its detrimental effects on children's health, the importance of early diagnosis of and treatment for reninoma should be emphasized. Here, the authors present a 10-year-old boy with intermittent headaches and neurologic deficiency symptoms caused by hypertension. A reninoma in the right kidney was detected and successfully treated with laparoscopic partial nephrectomy. Two cell types were revealed in the tumor tissue under electron microscopy: renin secreting tumor cells and mast cells. This rare case expands our knowledge of hypertension in children and provides direct evidence that mast cells may infiltrate reninoma.
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Affiliation(s)
- Jiangfeng Mao
- Department of Endocrinology, Peking Union Medical College Hospital, Beijing, China
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