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目的探讨原发性肝神经内分泌癌(PHNEC)的临床病理特点、影像学改变及鉴别诊断。方法回顾性分析2例PHNEC的临床病理资料并复习相关文献。结果 2例患者临床表现为上腹部疼痛不适及肝功能不同程度异常。影像学显示肝巨大占位性病变。镜下肿瘤细胞主要呈不规则巢团状或弥漫片状分布,亦可见索状排列,肿瘤内血管丰富。肿瘤细胞体积偏小,短梭形或淋巴细胞样;细胞坏死明显,核分裂象多见。免疫组化:肿瘤细胞Syn、CHG-A和CD56(+)。2例均行选择性肝动脉栓塞(TACE)治疗;1例失访,1例死亡,生存期6个半月。结论原发性肝神经内分泌癌是一种罕见的肿瘤,起病隐匿,早期诊断困难,确诊有赖于常规病理及免疫组化。鉴于该肿瘤恶性程度高,对于不能手术切除的患者,肝动脉栓塞有助于提高患者生存期。
Objective To investigate the clinicopathological features, imaging changes and differential diagnosis of primary hepatic neuroendocrine carcinoma (PHNEC). Methods Retrospective analysis of 2 cases of PHNEC clinical and pathological data and review the relevant literature. Results The clinical manifestations of 2 patients were upper abdominal pain and abnormal liver function. Radiographic imaging showed massive occupying lesions of the liver. Microscopic tumor cells were irregular or irregular nest clusters diffuse distribution, also visible cord-like arrangement, rich in blood vessels within the tumor. Small tumor cells, short fusiform or lymphoid cells; obvious cell necrosis, mitotic common. Immunohistochemistry: tumor cells Syn, CHG-A and CD56 (+). Two patients underwent selective hepatic artery embolization (TACE). One patient lost follow-up and one patient died. The survival time was 6 and a half months. Conclusion Primary hepatic neuroendocrine carcinoma is a rare tumor with occult onset and difficult to diagnose early. The diagnosis depends on routine pathology and immunohistochemistry. Given the high degree of malignancy of the tumor, hepatic artery embolization can help improve patient survival in patients who can not be surgically removed.