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Wunderlich's syndrome with hepatic angiomyolipoma in tuberous sclerosis

Introduction

Wunderlich's syndrome is one of the most feared complications of renal angiomyolipoma and should be managed aggressively. Nephron sparing approach is preferable, especially in TS with bilateral and multicentric angiomyolipomas. Hepatic angiomyolipomas, though rare, may be found in tuberous sclerosis, especially in patients with bilateral diffuse renal angiomyolipomas Wunderlich's syndrome refers to spontaneous non-traumatic renal bleeding into the subcapsular and/or perirenal space. The commonest cause of spontaneous renal hemorrhage in most series is an angiomyolipoma (AML). [1] AML is a benign clonal neoplasm consisting of varying amounts of mature adipose tissue, smooth ...

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