OBJECTIVETo investigate the clinical features, diagnostic and therapeutic methods of primary hepatic neuroendocrine carcinoma.

METHODSThe clinicopathological data of fourteen patients with primary hepatic neuroendocrine carcinoma confirmed by pathology were analyzed retrospectively and related literatures were reviewed.

RESULTSThe fourteen patients, including eight males and six females, had an age range of 23-58 years (mean 45.9 years). Four tumors were located in the right liver lobe, four in the left liver lobe and six in both. The clinical manifestations were nonspecific and variable. The most common clinical manifestation was abdominal distention or right upper quadrant pain. Radiological findings were not specific and could not distinguish primary hepatic neuroendocrine tumor from hepatocellular carcinoma. Diagnosis of primary hepatic neuroendocrine tumor was confirmed by pathology using immunohistochemical staining and by the absence of extrahepatic primary lesions. Extrahepatic primary neuroendocrine carcinoma was ruled out by ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT), preoperative gastrointestinal endoscopy and long-term postoperative follow up. Three patients received surgical treatment, two cases received surgical resection and radiofrequency ablation (RFA), six patients received transarterial chemoembolization, one case received orthotopic liver transplantation, one case only received exploratory laparotomy, and one case received chemotherapy. All 14 patients were followed up and seven of them are still alive, the others died of liver failure or recurrence.

CONCLUSIONSPrimary hepatic neuroendocrine carcinomas are extremely rare. Its diagnosis should be confirmed by pathology. Preoperative fine needle biopsy is strongly recommended. Prognosis is relatively favorable. Surgical resection is treatment of first choice, and TACE, RFA, and chemotherapy can be used for unresectable patients.