Neuroendocrine tumor in the liver of a patient with isolated polycystic liver disease: A case report and review of the literature

Neuroendocrine tumors (NETs) frequently metastasize to the liver, but it is rare to find them there as primary tumors. Isolated polycystic liver disease (PCLD) is a rare autosomal dominant disease. There is no known association between polycystic liver disease and neuroendocrine or other tumors. We report a case of a 64-year-old female with a past medical history of isolated PCLD who presented with increasing abdominal pain over a two-week period. Our patient underwent open surgical biopsy one month after presentation. The histological examination and immunohistochemical findings suggested an intermediate grade neuroendocrine tumor. A 24-h delayed whole-body scintigraphy technique was utilized for the identification and localization of neuroendocrine tumors via the administration of In-111-labeled OctreoScan; however, no extrahepatic accumulation was observed. No previous studies in the literature describe a patient with PCLD and a primary or metastatic neuroendocrine tumor of the liver.