Angiomyolipome der Nieren bei Morbus Bourneville-Pringle

Summary

Three cases with renal angiomyolipoma in Bourneville's disease are reported. In two patients advanced renal insufficiency developed, one being treated by chronic intermittent hemodialysis. In the third patient rupture of angiomyolipoma with severe retroperitoneal bleeding occurred. In one patient, tumorous enlargement initially was diagnosed only in the right kidney and preceded angiomyolipoma of the left kidney for several years. In this patient nephrectomy was performed because malignancy was assumed before diagnosis of Bourneville's disease was established. Occasionally, histology may suggest sarcomatous changes. However, the absence of distant metastasis and the overall long survival of patients with renal angiomyolipoma in Bourneville's disease underline the benign character of the tumor. Treatment should be conservative because surgical intervention with loss of kidney parenchyma may enhance progression to end-stage renal failure.