Primary Hepatic Neuroendocrine Tumor in a Patient With Tuberous Sclerosis

Tuberous sclerosis is a rare multisystemic disease with common benign manifestations. However, recent research suggests a rare association with malignant tumors. We describe the case of a primary hepatic neuroendocrine tumor in a patient with tuberous sclerosis that is, to our knowledge, unique in the literature. A 23-year-old woman with tuberous sclerosis presented with an enlarging liver mass. Biopsy specimen pathology was consistent with a neuroendocrine tumor. She received chemotherapy and had an extensive resection which revealed a 12-cm, well-differentiated, primary hepatic neuroendocrine tumor.