EMBRYONAL ADENOMA OF THE KIDNEY ASSOCIATED WITH POLYCYTHEMIA AND VON-WILLIEBRAND-DISEASE

Purpose: We studied the significance, identification and management of acquired von Willebrand disease and polycythemia associated with beni gn renal tumors in children. Materials and Methods: Two patients who p resented with polycythemia and a renal mass were also found to have ac quired von Willebrand disease. One patient was treated with radical ne phrectomy and 1 was treated with partial nephrectomy. The patients hav e been followed for 19 and 10 months, respectively. Results: Excision of the renal mass resulted in prompt resolution of polycythemia and vo n Willebrand disease in each patient. Perioperatively 1-deamino-(8-D-a rginine)-vasopressin was given to control bleeding. Each patient had b enign embryonal adenoma of the kidney. Conclusions: Polycythemia and v on Willebrand disease may be associated with benign kidney neoplasms. Children and adolescents with a renal mass may benefit from preoperati ve screening for coagulopathy.