Tuberous Sclerosis Associated with Biphasic Adult Wilms' Tumor: A Case Report and Review of Literature

Abstract Tuberous sclerosis is an autosomal dominant genetic disorder characterized by lesions in the skin and benign tumors in various organs—retina, lungs, heart, brain, skin, and kidneys. Wilms' tumor is rare in adults. We present a unique case of Wilms' tumor in an adult with tuberous sclerosis. A 17-year-old girl presented with chief complaints of a large abdominal lump in the left loin region, associated with multiple acne-like dark skin patches all over the body (suggesting tuberous sclerosis), of 2 months' duration. Contrast-enhanced computed tomography suggested a large left renal tumor along with multiple variably sized, heterogeneously enhancing lymph nodes at preaortic, left para-aortic, left hilar, and mesenteric regions. She underwent laparoscopic radical nephrectomy and regional lymphadenectomy. Histopathology suggested a biphasic Stage 1 Wilms' tumor and was started on 16-week chemotherapy regimen of vincristine and dactinomycin. A patient with tuberous sclerosis complex presented with an abdominal mass and was diagnosed with biphasic Wilms' tumor as per the histopathology report. This presentation is unique and needs management accordingly.