Abstract: Objective The aim of this study was to summarize the clinical features of patients with primary hepatic neuroendocrine neoplasm (PHNEN). Methods The clinical manifestations, laboratory tests, imaging, pathological examination and clinical outcomes in four patients with PHNEN encountered in our hospital were summarized by retrospective analysis. Results The age of the four female patients was 56 (40-73) years; there were no symptoms in 2 cases and abdominal pain in 2 cases; serum ALT levels were 28.3(18.0-49.8)U/L, bilirubin levels were 11.5(9.5-16.3)μmol/L, neuron-specific enolase levels were 44.1(20.2-77.1) ng/ml; the abdominal CT scan showed multiple lesions in all cases, and in contrast-enhanced scan, different degrees of ring or uneven enhancement in the arterial phases, and decreased enhancement in the venous phase; all diagnoses were confirmed by pathological examination, and surgical operation, interventional therapy and chemotherapy were given in 3 cases, and one case did not receive any treatment; during the follow-up period of 11 to 81 months, 2 cases died and 2 cases still survived. Conclusion The PHNEN is a rare tumor in clinic, and lacks of specific laboratory and pathological markers. It should be considered after excluding other liver tumors. Early detection and several therapy combination are recommended to improve the prognosis.

Key words: Primary hepatic neuroendocrine neoplasm, Diagnosis, Treatment, Literature review