Epithelioid angiosarcoma of bone
Epithelioid angiosarcoma of bone
复制标题
DOI：
10.1016/s0046-8177(97)90016-2
复制
发表时间：
1997-08-01
期刊：
影响因子：
3.3
通讯作者：
Sano, T
中科院分区：
文献类型：
--
作者：
Hasegawa, T;Fujii, Y;Sano, T
骨血管肉瘤是一种罕见的血管源性高级别肉瘤。本文报告一例48岁男性肱骨上皮样血管肉瘤，影像学检查显示右肱骨近端干骺端有多房性溶骨性病变，边缘不清，皮质骨和髓骨破坏，伴有大的软组织肿块。肿瘤，切除截肢，主要是由增生的恶性细胞与上皮样形态。它有一个主要的片状生长模式，偶尔假腺或肺泡排列，模仿腺癌。扩张的吻合血管间隙内衬上皮样内皮细胞和胞浆内腔/空泡，有时含有红细胞表明局灶性内皮分化。在免疫组化研究中，许多肿瘤细胞表达细胞角蛋白和内皮标记物：因子VIII相关抗原、CD 31和UEA-I。肿瘤的超微结构与血管肉瘤一致。我们的病人在诊断后不久死于疾病，这意味着一个积极的临床过程。意识到骨骼上皮样血管肉瘤的存在，结合胞浆内腔或至少小血管形成病灶的鉴定以及内皮标记物的免疫组化阳性，为区分这种肿瘤与转移性癌提供了最好的指导。版权所有（C）1997 W.B.桑德斯公司
Angiosarcoma of bone is a rare, high-grade sarcoma of vascular origin. This article describes an epithelioid angiosarcoma in the humerus of a 48-year-old man. A multilocular osteolytic lesion with undefined margins and destroyed cortical and medullary bone, associated with a large soft tissue mass was demonstrated radiologically in the proximal metaphysis of the right humerus. The tumor, resected by amputation, was composed mostly of proliferating malignant cells with an epithelioid morphology. It had a predominantly sheet-like growth pattern, and an occasional pseudoglandular or alveolar arrangement, mimicking an adenocarcinoma. The dilated anastomotic vascular spaces lined by epithelioid endothelial cells and the intracytoplasmic lumina/vacuoles that sometimes contained erythrocytes suggested focal endothelial differentiation. On immunohistochemical investigation, many neoplastic cells expressed cytokeratin and endothelial markers: factor-VIII related antigen, CD31, and UEA-I. The ultrastructure of the tumor was consistent with that of an angiosarcoma. Our patient died of disease shortly after the diagnosis, implying an aggressive clinical course. Awareness of the existence of skeletal epithelioid angiosarcoma, combined with the identification of intracytoplasmic lumina, or at least small vasoformative foci, and immunohistochemical positivity for endothelial markers provide the best guide for distinguishing this tumor from metastatic carcinomas. Copyright (C) 1997 by W.B. Saunders Company.