Primary hepatic neuroendocrine tumor: an unusual cystic presentation

Primary neuroendocrine tumors of the liver are exceedingly rare and unlike metastatic neuroendocrine tumor rarely cause carcinoid syndrome. There are only about 60 such cases reported in the current literature. We present a case of a 57-year-old male with a primary hepatic neuroendocrine tumor which presented as cystic mass and successfully resected. The case presented required meticulous radiological, histopathological, and immunohistochemical work-up to rule out an occult extrahepatic malignancy with hepatic metastasis to confirm the primary nature of hepatic tumors. Here we intend to put forward a review of the current literature regarding the diagnosis, pathology, and management of this disease.