Giant bilateral Angiomyolipoma in tuberous sclerosis

Abstract

Renal angiomyolipoma are uncommon benign tumors that occur in up to 80% of patients with tuberous sclerosis. Uncommonly, they may become extremely large. We report a case of an enormous bilateral renal angiomyolipoma. The patient is a 30-year-old woman with history of seizures since the age of two years and developmental delay, her parents are cousins, one brother died at 24 years. She had a cyclic flank pain. Clinical examination revealed palpable abdominal masses with a contact lumbar, she has a facial angiofibromas and her neurological examination was unremarkable. Urinalysis, serum electrolytes, blood urea nitrogen and creatinine were normal. Computed tomography (CT) showed a voluminous retroperitoneal mass distorted the renal parenchyma and extended into the pelvis. This mass was composed mainly of fat density and measured 30 cm in length (fig). While neprectomy should be avoided when possible in patients with bilateral tumors, it may be necessary, especially to control hemorrhage. We had choose in alternative management an elective arterial embolisation, in fact selective arterial embolisation is an effective way to treat acute hemorrhage but its ability to prevent tumor growth is not established. Our patient was sent to a center of interventional radiology.