Isolated Renal Multifocal Epithelioid Angiomyolipoma Leading to Non-Functioning Kidney in a Case of Tuberous Sclerosis Complex with Piebaldism

Abstract

A 19-year-old gentleman presented to the outpatient department with chief complaint of a right loin mass which had been increasing in size since the past 1 year and had been insidious in onset, causing right loin pain, discomfort, early satiety, along with loss of weight and loss of appetite. Patient was a known case of tuberous sclerosis complex with piebaldism since childhood and had related clinical features like Ash-leaf macules on the trunk, retinal hamartomas along with a recurrent intra-oral disfiguring hamartoma (histopathologically proven acanthosis with hyperkeratosis).