Aggressive hepatic angiomyolipoma in a child

Angiomyolipoma is a tumor of the kidney and, more rarely, the liver, which histologically consists of smooth muscle cells, adipose cells, and abnormal blood vessels in varying proportions. This tumor is generally benign and resection is curative, but here the authors present the case of a 14-year-old girl with an unusual primary hepatic angiomyolipoma that recurred following resection and behaved aggressively. Despite stabilization with a number of novel therapies, the child ultimately died of progressive disease.