原发性肝神经内分泌癌2例临床病理观察

Clinicopathological features of primary hepatic neuroendocrine carcinoma

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 explore the clinicopathological features,image changes,differential diagnosis and prognosis of primary hepatic neuroendocrine carcinoma（ PHNEC）. Methods We summarized the clinicopathological data of two patients having diagnosed as PHNEC and reviewed the related literatures. Results Two patients presented with different degrees of abdominal pain and disorder of liver function. Meanwhile,image showed that liver had a huge tumor. The tumor was composed of a diffuse proliferation of short-spindle-shaped or lymphocyte-shaped tumor cell with abundant blood vessels. There were obvious necrosis and mitosis. The tumor cells expressed Syn,CHG-A and CD56; Two patients had the treatment of TACE（ transcatheter arterial chemoembolization,TACE）,one of them lost for follow-up,the other one died after 6. 5 months. Conclusions PHNEC is a rare neuroendocrine tumor,insidious onset,early diagnosis is difficult.Disease diagnosis depends on morphological observation of pathology and immunohistochemistry. Due to the high degree of malignancy of the tumor,for patients with unresectable tumor,TACE can contribute to improve the survival time of them.