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

Author:

Skarakis Nikitas S1ORCID,Papadimitriou Irene1,Papanastasiou Labrini1,Pappa Sofia2,Dimitriadi Anastasia2,Glykas Ioannis3,Ntoumas Konstantinos3,Lampropoulou Penelope4,Kounadi Theodora1

Affiliation:

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

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.

Publisher

Bioscientifica

Subject

Endocrinology, Diabetes and Metabolism,Internal Medicine

Reference10 articles.

1. A hitherto unreported vascular tumor of the kidney: a proposal of ‘juxtaglomerular cell tumor’;Kihara,1968

2. Reninoma: an uncommon cause of renin-mediated hypertension;Trnka,2014

3. Recurrent malignant juxtaglomerular cell tumor: a rare cause of malignant hypertension in a child;Shera,2011

4. Reninoma coexisting with adrenal adenoma during pregnancy: a case report;Xue,2017

5. Juxtaglomerular cell tumor: reviewing a cryptic cause of surgically correctable hypertension;Inam,2019

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