Abstract: Objective: To investigate the clinical features, diagnosis and effective treatment strategies of primary hepatobiliary neuroendocrine tumor. Methods: The clinical data of 16 patients with primary hepatobiliary neuroendocrine tumor were analyzed retrospectively, and the clinical characteristics and treatment options of primary hepatobiliary neuroendocrine tumor were discussed combined with literature review. Results: There was no obvious increase in AFP and CEA in all patients. On CT, the arterial phase was markedly enhanced, while the portal phase and the delayed phase were weakened. On MRI, the tumors showed low signal on T1-weighted MRI, high signal on T2-weighted MRI, limited diffusion on DWI, and marked annular or heterogeneous enhancement in arterial phase, and decreased enhancement in portal phase and delayed phase. All patients were diagnosed as primary hepatobiliary neuroendocrine tumors by pathological results. The results of immunohistochemistry showed that chromogranin A (CgA) was positive in all tumors and synaptophysin (Syn) was positive in 11 cases. Follow up for 3 to 96 months showed no recurrence in 14 cases. The longest disease-free survival period was 96 months. No other neuroendocrine tumors were found in all patients. Conclusions: At present, for patients with neuroendocrine tumor of hepatobiliary system, the treatment plan should be formulated according to the specific situation of patients, and the relevant examination should be fully improved to reduce the occurrence of misdiagnosis. For resectable lesions, surgical resection is the first choice.

Key words: Primary hepatobiliary neuroendocrine tumors, Medical imageology, Treatment, Prognosis