Demonstration of both primary and secondary reninism in renal tumors in children

Abstract

Two infants with renal tumors and associated hypertension are presented. By using an antibody to purified human renal renin, the sites of renin production were localized immunohistochemically in each tumor. The first case was a 9-month-old girl with Beckwith-Wiedemann syndrome. She presented with bilateral renal masses and hypertension (140/90 mm Hg). Following a left nephrectomy and chemotherapy and radiotherapy, her BP returned to normal. Her tumor was a Wilms' tumor of favorable histology, composed predominantly of glomeruloid structures. Renin was localized within a part of these neoplastic glomeruloid bodies. We therefore designated this as a Wilms' tumor with glomeruloid differentiation having primary reninism. The second case was a 24-day-old girl with hypertension (140/70 mm Hg). A renal tumor was found and successfully removed. Her BP returned to normal. The tumor was histologically confirmed as a congenital mesoblastic nephroma. By indirect immunoperoxidase staining, renin was localized only in the hypertrophied juxtaglomerular cells adjacent to the residual glomeruli entrapped by the tumor. None was seen in the tumor cells. We concluded that this was a case of secondary reninism--a case of hypertension secondary to the local ischemia at the entrapped glomeruli.