本文選題：血管肉瘤 + 神經(jīng)鞘瘤　； 參考：《臨床與實驗病理學雜志》2014年07期

【摘要】：目的觀察源于神經(jīng)鞘瘤的血管肉瘤臨床病理學特征,探討與神經(jīng)源性腫瘤相關的血管肉瘤組織學特征及影響預后因素。方法回顧性分析源于神經(jīng)鞘瘤上皮樣血管肉瘤的臨床病理特征、免疫表型、治療及預后,并復習相關文獻。結果患者男性,右上臂有一無痛性腫塊。腫塊呈結節(jié)樣,切面呈囊實性,腫瘤形態(tài)呈多形性,可見梭形細胞區(qū)域,部分區(qū)域細胞豐富,二者截然分界,細胞豐富區(qū)見由不規(guī)則血管構成的疏松區(qū),并見上皮樣細胞成片分布或呈靶環(huán)樣排列,核分裂象易見。梭形細胞表達S-100蛋白,豐富區(qū)域S-100蛋白表達缺失,但表達GFAP,富于血管的腫瘤細胞表達CD34、CD31、FⅧRAg,上皮樣細胞表達廣譜CK�；颊咝g后接受放療,隨訪34個月,未見復發(fā)或轉移。結論源于神經(jīng)鞘瘤的血管肉瘤極為少見,惡性程度高。腫瘤直徑(5cm)、細胞核分裂計數(shù)、手術切緣陽性以及伴有Ⅰ型神經(jīng)纖維瘤病(neurofibromatosis type 1,NF-1)均為其預后不良因素。
[Abstract]:Objective to observe the clinicopathological features of angiosarcoma derived from neurilemmoma and to investigate the histological features and prognostic factors of angiosarcoma associated with neurogenic tumors. Methods the clinicopathological features, immunophenotype, treatment and prognosis of epithelioid angiosarcoma derived from schwannoma were retrospectively analyzed. Results the patient had a painless mass in his right upper arm. The tumor was nodular, cystic and solid, and the shape of the tumor was pleomorphic. The fusiform cell area was seen, and some of the regions were rich in cells. The two areas were separated completely, and the loose areas composed of irregular blood vessels could be seen in the rich areas. The epithelioid cells were distributed or arranged like target rings, and mitotic images were easy to be seen. S-100 protein was expressed in fusiform cells, S-100 protein was absent in rich region, GFAPs were expressed, CD34- CD31- F 鈪，

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