肝脏钙化性巢状间质-上皮肿瘤1例报道及文献复习

Calcifying nested stromal-epithelial tumor of the liver:a case of report and review of literature

目的探讨肝脏钙化性巢状间质-上皮肿瘤的临床病理学特征、免疫表型、诊断和鉴别诊断。方法对1例肝脏的钙化性巢状间质-上皮肿瘤进行大体、组织形态学和免疫组化检查,结合文献分析其临床生物学行为。结果组织学上,肿瘤主要由梭形到上皮样细胞呈巢状排列伴周围明显的促纤维间质,梭形细胞胞质淡染或嗜酸性,核杆状;胞界不清,上皮样细胞胞质透亮,细胞界限清楚,核圆形位于中央,核分裂象<1个/10HPF;可见骨化和微囊形成。免疫组化染色显示肿瘤细胞表达CKpan、vimentin和WT1胞质阳性,S-100蛋白仅在骨化区域表达,肿瘤间质细胞表达SMA,而AFP、HepPar1、CK19、CD56、CD34、CD117、desmin、myogenin、ER、PR、CgA、Syn均表达阴性,Ki-67增值指数约5%。结论肝脏钙化性巢状间质-上皮肿瘤是一种罕见的肝脏原发性低度恶性肿瘤,应与肝脏其他侵袭性恶性肿瘤鉴别。

Purpose To study the clinicopathologic features,immunophenotype,diagnosis and differential diagnosis of calcifying nested stromal-epithelial tumor of the liver（CNEST）.Methods One case of CNEST of the liver was observed by gross,histomorphologic and immunohistochemical technique and review of literatures.Results Histology,tumor shared an organoid arrangement of cellular nests that were comprised of an admixture of both spindled and epithelioid cells with an accompanying desmoplastic stroma.The spindle cells had pale or acidophilia cytoplasma and rod nucleus with stippled chromatin.The epithelial cells had pale cytoplasma and distinct cellular borders with a bland,round to oval nuclei.Scattered mitotic figures were identified（1/10 HPF）.Osteoid formation and microcystic within nests were observed.Immunohistochemically,thmor cells were positive for AE1/AE3、vimentin and WT1 cytoplasmic staining and negative for AFP、Hep Par1、CK19、CD56、CD34、CD117、desmin、myogenin、ER、PR、CgA and Syn.S-100 protein only seen in osteoid area.The tumor stromal cells were positive for SMA.The proliferating index was about 5%.Conclusions CNEST of the liver is a rare low-grade primary liver malignant tumor and should not be confused with other aggressive hepatic malignancies.