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      Juxtaglomerular cell tumour of the kidney: a rare cause of resistant hypertension

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          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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          Most cited references10

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          Reninoma: case report and literature review.

          Reninoma is a tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin. We describe a case of reninoma and provide a review of the literature, with a discussion emphasizing the diagnostic evaluation for such patients. The subject had persistent elevation of both plasma renin activity (PRA) and aldosterone. Imaging studies revealed the presence of a lesion in the renal cortex, which was further identified as a renin-producing lesion via selective venous catheterization following administration of an angiotensin-converting enzyme inhibitor (ACE-I). Following partial nephrectomy, the PRA and plasma aldosterone levels declined rapidly and the blood pressure and potassium supplementation requirements normalized. This case demonstrates the utility of both appropriate imaging studies and selective venous catheterization following provocative administration of an ACE-I for diagnosis.
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            Juxtaglomerular cell tumor of the kidney--a new classification scheme.

            To introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma. Five cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized. The 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels. The classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice. Crown Copyright 2010. Published by Elsevier Inc. All rights reserved.
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              Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

              Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. Reninomas are usually diagnosed in adolescents and young adults with occasional reports in younger children. Patients typically present with a long history of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system. The clue to clinical diagnosis is the presence of hypokalemia and metabolic alkalosis on the first blood sample drawn before any treatment is instituted. Elevated blood levels of renin and aldosterone confirm the clinical suspicion of renin-mediated hypertension. Diagnostic imaging is employed to identify the source of excessive renin production. While renal ultrasound can miss reninoma, contrast CT or magnetic resonance imaging of the kidneys are diagnostic modalities of choice leading to the correct diagnosis. Renal vein renin sampling with lateralization might help to identify the site of excessive renin production. Nephron-sparing surgery is curative with maintenance of normal blood pressure after discontinuation of antihypertensive medications in the majority of patients. In this paper, we present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor. We also discuss important points in the management of children presenting with renin-mediated hypertension.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                21 December 2021
                2022
                : 2022
                : 21-0042
                Affiliations
                [1 ]Unit of Endocrinology and Diabetes Center , ‘G. Gennimatas’ General Hospital, Athens, Greece
                [2 ]Department of Pathology , ‘G. Gennimatas’ General Hospital, Athens, Greece
                [3 ]Department of Urology , General Hospital of Athens ‘G Gennimatas’, Athens, Greece
                [4 ]Department of Radiology , General Hospital of Athens ‘G Gennimatas’, Athens, Greece
                Author notes
                Correspondence should be addressed to N S Skarakis; Email: skar.nik.ns@ 123456gmail.com
                Author information
                http://orcid.org/0000-0001-5350-2846
                Article
                EDM210042
                10.1530/EDM-21-0042
                8789008
                35023474
                eb0b5478-2ead-476f-b8f5-9eca9832e19d
                © The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

                History
                : 05 September 2021
                : 21 December 2021
                Categories
                Adult
                Male
                White
                Greece
                Kidney
                Endocrine-Related Cancer
                Nephrology
                Unique/Unexpected Symptoms or Presentations of a Disease
                Unique/Unexpected Symptoms or Presentations of a Disease

                adult,male,white,greece,kidney,endocrine-related cancer,nephrology,unique/unexpected symptoms or presentations of a disease,january,2022

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