Article Abstract

Primary hepatic neuroendocrine tumors: retrospective analysis of seven cases and literature review

Authors: Qichen Chen, Xiao Chen, Haizhen Lu, Hong Zhao, Jianjun Zhao, Xinyu Bi, Dongbing Zhao, Yihebali Chi, Zhiyu Li, Zhen Huang, Yefan Zhang, Jianguo Zhou, Jianqiang Cai


Background: Primary hepatic neuroendocrine tumors (PHNETs) are extremely rare. Their clinical features, diagnosis and effective treatment strategies are not well understood.
Methods: We aimed to investigate them by combining retrospectively analyzing the clinical data of seven patients with PHNETs who were admitted to our hospital from April 2009 to November 2017 with literature review.
Results: PHNET patients without medical history of hepatitis or liver cirrhosis presented no specific symptom, and hepatic tumor markers such as alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA) were all within the normal range. Imaging of PHNET patients was found to be with a dominant hypervascular hepatic mass accompanied by a lower cystic signal and a higher hemorrhagic signal area on T1-weighted magnetic resonance imaging (MRI), with rapid washout and capsular enhancement on dynamic MR imaging or computed tomography (CT) imaging and restricted diffusion on diffusion-weighted (DW)-MRI. All patients underwent preoperative and postoperative CT examination or MRI examination which identified no extrahepatic tumors. All patients underwent surgery-centered comprehensive individualized treatment. Postoperative pathology confirmed that all patients had NETs. On immunohistochemistry all of the tumors were chromogranin A (CgA) positive, six were synaptophysin (Syn) positive. At the end of the follow-up period ranging from 9 to 87 months, six patients survive without recurrence. The longest postoperative survival time and the longest disease-free survival (DFS) time were all 87 months.
Conclusions: Our findings suggest PHNET patients have unique clinical characteristics. The diagnosis of PHNETs relies on pathology and imaging examination MRI or CT to exclude primary extrahepatic tumors. Surgery-centered individualized comprehensive treatment leads to a good prognosis of patients with PHNETs.