Embryonal adenoma of the kidney associated with polycythemia and von Willebrand disease.

Abstract

We studied the significance, identification and management of acquired von Willebrand disease and polycythemia associated with benign renal tumors in children. Two patients who presented with polycythemia and a renal mass were also found to have acquired von Willebrand disease. One patient was treated with radical nephrectomy and 1 was treated with partial nephrectomy. The patients have been followed for 19 and 10 months, respectively. Excision of the renal mass resulted in prompt resolution of polycythemia and von Willebrand disease in each patient. Perioperatively 1-deamino-(8-D-arginine)-vasopressin was given to control bleeding. Each patient had benign embryonal adenoma of the kidney. Polycythemia and von Willebrand disease may be associated with benign kidney neoplasms. Children and adolescents with a renal mass may benefit from preoperative screening for coagulopathy.