Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich’s Syndrome in A Tuberous Sclerosis Patient

摘要

Renal Angiomyolipoma (renal AML) is a benign clonal neoplasm with a incidence of 0.3-3%, occurring as isolated sporadic entity or in association with Tuberous sclerosis (TS) in 80% cases. Multiple, bilateral renal AML are considered diagnostic of Tuberous sclerosis. Wunderlich’s syndrome, a urological emergency is a spontaneous nontraumatic bleeding into subcapsular and or peri-renal space and is a life threatening complication of renal AML occurring in 10% cases which has to be timely diagnosed and treated. Here, we present an unusual case of TS with bilateral, multifocal renal AML in a 25-year-old female who presented with Wunderlich’s syndrome, which is a rare but life threatening complication that has to be suspected, timely diagnosed and treated. We have discussed the importance of early diagnosis, timely treatment, follow up and education of patient and relatives of the possible complications associated.