泌尿器科・外科
オープンアクセス

概要

Bilateral Giant Renal Angiomyolipoma in Tuberous Sclerosis Complex with Wunderlich Syndrome: A Case Report

Benjamin Chui Jia Tjun, GEL Choo, N.A Nasuha

Tuberous Sclerosis Complex (TSC) is a rare autosomal dominant disease characterized by the classical Vogt’s triad– epilepsy, mental retardation and sebaceous adenomas. TSC can have renal manifestations which include Angio Myo Lipomas (AML) and renal epithelial neoplasms. AML associated with TSC has the highest incidence between the second and third decade, and carries the risk of spontaneous retroperitoneal haemorrhage proportional to its size. Wunderlich syndrome is a rare condition characterized by acute onset of spontaneous, non-traumatic renal hemorrhage into the sub capsular and perirenal spaces, usually from a renal angiomyolipoma. Here we report a very rare case of bilateral giant AML in the setting of TSC with Wunderlich syndrome which necessitated surgery and discuss the management.