源于神经鞘瘤的上皮样血管肉瘤1例并文献复习

发布时间：2018-05-06 18:41

  本文选题：血管肉瘤 + 神经鞘瘤　； 参考：《临床与实验病理学杂志》2014年07期

【摘要】：目的观察源于神经鞘瘤的血管肉瘤临床病理学特征,探讨与神经源性肿瘤相关的血管肉瘤组织学特征及影响预后因素。方法回顾性分析源于神经鞘瘤上皮样血管肉瘤的临床病理特征、免疫表型、治疗及预后,并复习相关文献。结果患者男性,右上臂有一无痛性肿块。肿块呈结节样,切面呈囊实性,肿瘤形态呈多形性,可见梭形细胞区域,部分区域细胞丰富,二者截然分界,细胞丰富区见由不规则血管构成的疏松区,并见上皮样细胞成片分布或呈靶环样排列,核分裂象易见。梭形细胞表达S-100蛋白,丰富区域S-100蛋白表达缺失,但表达GFAP,富于血管的肿瘤细胞表达CD34、CD31、FⅧRAg,上皮样细胞表达广谱CK。患者术后接受放疗,随访34个月,未见复发或转移。结论源于神经鞘瘤的血管肉瘤极为少见,恶性程度高。肿瘤直径(5cm)、细胞核分裂计数、手术切缘阳性以及伴有Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF-1)均为其预后不良因素。
[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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