肝原发性神经内分泌癌1例尸检报道并文献复习

Primary neuroendocrine carcinoma of liver: an autopsy case report and review of literature

目的 探讨肝原发性神经内分泌癌的病理组织学、免疫组织化学以及超微结构特征。方法 对1例尸检诊断的肝原发性神经内分泌癌病理组织学、免疫组织化学以及超微结构进行研究，并结合文献复习。结果 男性，84岁。肝重2410g，可见大小不等的结节弥漫分布，最大者5cm×4cm×4cm。瘤细胞小至中等大小，呈弥漫分布的巢状结构，富于血窦。瘤细胞免疫表型AE1、Syn、CD99、NSE和VIP（＋），1EA、AFP、Hepar-1（-）。电镜下可见成簇的大小为150～350nm不等的电子密度致密的核心颗粒。结论 肝原发性神经内分泌癌非常少见，其诊断需结合病理组织形态、免疫表型及超微结构特征，并除外其他脏器原发病变的肝转移。

Objective To investigate the pathological features, immunohistochemistry, and ultrastructure of hepatic neuroendocrine carcinoma. Methods The pathologic, and ultrastructural features were studied in an autopsy ease of hepatic neuroendocrine carcinoma, and the literature was reviewed. Results A 84-year-old man died of liver tumor. The liver weighted 2 410 gram and had a variable solid nodules, the greatest one measuring 5 cm × 4 cm × 4 cm, arranged diffusely. The tumor cells were small to medium in size, and exhibited nests arrangement with irregular blood sinusoids in mesenchymal tissue. The immunophenotype of the tumor cells was AE1 （ ＋ ）,LCA （ - ）, AFP （ - ）, Hepar-1 （ - ）, Syn （ ＋ ）, CD99 （ ＋ ）, NSE （ ＋ ）, VIP （ ＋ ）. Electron microscopy showed a duster of dense core granules with 150 nm～350 nm in diameter in the cytoplasm of the tumor cells. Conclusions Primary neuroendocrine carcinoma of the liver is very rare, and the diagnosis needs to combine pathomorphology and immunophenotype with ultrastruetural features, eliminating the possibility of metastases from other organs to the liver as veil.