原发性下消化道上皮样血管肉瘤3例临床病理分析

Primary epithelioid angiosarcoma of the lower digestive tract:a clinicopathologic analysis of three cases

目的探讨原发性下消化道上皮样血管肉瘤(epithelioid angiosarcoma,EAS)的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析3例原发性下消化道EAS的临床病理资料,对根治性切除标本进行常规HE和免疫组化EnVision法染色,并结合相关文献复习,分析其临床病理学特征。结果3例患者年龄68~75岁,男性2例,女性1例;发生于回肠2例,结肠1例;临床均以腹部疼痛就诊。镜下肿瘤均呈侵袭性生长,排列呈蜂窝状、海绵状、脾窦样和裂隙样,裂隙内可见乳头簇形成,瘤细胞呈上皮样,具有显著的异型性,表现为核大空泡状,核仁明显,可见多少不等的核分裂象。免疫表型:瘤细胞CD31、CD34、ERG和Fli-1阳性,Ki-67增殖指数50%~70%。结论原发性下消化道EAS临床罕见,以消化道出血、腹痛和贫血为主要表现,易与消化道癌、胃肠道间质瘤混淆,确诊需结合组织学形态及免疫表型,预后较差。

Purpose To investigate the clinicopathologic features,immunophenotype and differential diagnosis of epithelioid angiosarcoma of the lower gastrointestinal tract.Methods The clinicopathological data of 3 cases of primary epithelioid angiosarcoma of the lower gastrointestinal tract of the stomach were retrospectively analyzed.Routine histopathology and immunohistochemistry EnVision examination were performed on the radical resection specimens,and the clinicopathological features were analyzed based on the review of relevant literature.Results All 3 cases were elderly,aged 68-75 years.Two cases were male,one case was female,and the tumors occurred in ileum in two cases and colon in one case.All patients were treated for abdominal pain.Under the light microscope,the tumor showed invasive growth,and the tumor was arranged in the patterns of honeycomb,spongy,splenic sinus and fissure.Papillary clusters were formed in the cavity,and the tumor cells were epithelioid with significant atypia,presenting as large vacuole of nucleus,prominent nucleoli,and various mitotic figures.Immunohistochemical markers showed that tumor cells expressed CD31,CD34,ERG and Fli-1,and Ki-67 proliferation index ranged from 50%to 70%.Conclusion Primary epithelioid hemangiosarcoma of the lower digestive tract is extremely rare.Its clinical manifestations are mainly gastrointestinal bleeding,abdominal pain and anemia,and it is easy to be confused with other gastrointestinal cancer and gastrointestinal stromal tumor.Diagnosis needs to be combined with histological morphology and immunohistochemistry.The prognosis is poor.