Sato Katsuaki, Murai Hiroshi, Ueda Yoshimichi, Katsuda Shogo
Department of Pathophysiological and Experimental Pathology, Kanazawa Medical University, 1-1Daigaku, Uchinada Kahoku, Ishikawa 920-0293, Japan.
Virchows Arch. 2006 Nov;449(5):585-90. doi: 10.1007/s00428-006-0291-5. Epub 2006 Oct 11.
Sarcomatoid transformation was observed in 4.5% of autopsied cases of intrahepatic cholangiocarcinoma. Here, we report a case of intrahepatic sarcomatoid cholangiocarcinoma with round cell feature, extremely rare variant. An 87-year-old man was incidentally found to have a tumor in the left lobe of the liver by abdominal computed tomography scans. The patient was clinically diagnosed to have intrahepatic cholangiocarcinoma and received palliative care without specific treatment. He died of hepatic insufficiency 3 months after the diagnosis. The autopsied liver specimens showed a reddish gray tumor of 4.0x2.8 cm in size. Histologically, the tumor was centrally hemorrhagic and necrotic and was composed of tubular adenocarcinoma and a round cell component, which has an eccentrically located nucleus and eosinophilic cytoplasm without mucin production. Immunohistochemically, the adenocarcinoma cells expressed cytokeratin 19 and beta-catenin in their cytoplasm, with E-cadherin and CD44s at the plasma membrane. In the round cells, cytokeratin 19 and vimentin was detected in their cytoplasm and CD44s at the plasma membrane. E-cadherin immunoreactivity was weakly present in their cytoplasm and beta-catenin was negative. Loss or reduction of the E-cadherin and beta-catenin expressions and overexpression of CD44s in the round cells are suggested to be contributed to the high propensity for lymphatic permeation and poor prognosis.
在肝内胆管癌尸检病例中，4.5%观察到肉瘤样转化。在此，我们报告一例具有圆形细胞特征的肝内肉瘤样胆管癌病例，这是一种极其罕见的变异型。一名87岁男性通过腹部计算机断层扫描偶然发现肝脏左叶有一个肿瘤。该患者临床诊断为肝内胆管癌，接受了姑息治疗，未进行特异性治疗。诊断后3个月，他死于肝功能不全。尸检肝脏标本显示一个大小为4.0×2.8厘米的红灰色肿瘤。组织学上，肿瘤中央有出血和坏死，由管状腺癌和圆形细胞成分组成，圆形细胞有偏心核和嗜酸性细胞质，不产生黏液。免疫组化显示，腺癌细胞在细胞质中表达细胞角蛋白19和β-连环蛋白，在质膜上表达E-钙黏蛋白和CD44s。在圆形细胞中，细胞质中检测到细胞角蛋白19和波形蛋白，质膜上检测到CD44s。E-钙黏蛋白免疫反应性在其细胞质中弱阳性，β-连环蛋白阴性。圆形细胞中E-钙黏蛋白和β-连环蛋白表达缺失或减少以及CD44s过表达被认为与淋巴浸润倾向高和预后不良有关。
