Juxtaglomerular cell tumor
Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells that typically secretes renin. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain.
Juxtaglomerular cell tumor was first described in 1967 in a paper by Robertson et al., and first named by Kihara et al. in 1968. Since then, approximately 100 case reports have been published. Karyotyping of a small number of these tumors revealed a common loss of chromosomes 9 and 11.
By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. Both of these conditions may be corrected by surgical removal of the tumor. Asymptomatic cases have been reported.
Pre-operatively, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT. However, other kidney tumors can cause hypertension by secreting renin. JCTs have a variable appearance and have often being misdiagnosed as renal cell carcinomas; dynamic computed tomography is helpful in the differential diagnosis. Post-operatively, presence of renin granules in pathology specimens differentiates this tumor from renal hemangiopericytoma and other renal tumors.
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