EPITHELIOID ANGIOSARCOMA OF DEEP SOFT-TISSUE - A DISTINCTIVE TUMOR READILY MISTAKEN FOR AN EPITHELIAL NEOPLASM
EPITHELIOID ANGIOSARCOMA OF DEEP SOFT-TISSUE - A DISTINCTIVE TUMOR READILY MISTAKEN FOR AN EPITHELIAL NEOPLASM
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DOI：
10.1097/00000478-199110000-00001
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发表时间：
1991-10-01
影响因子：
5.6
通讯作者：
MARLEY, NJE
中科院分区：
文献类型：
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作者：
FLETCHER, CDM;BEHAM, A;MARLEY, NJE
我们报告八例上皮样血管肉瘤发生在深部，通常是肌肉软组织。 所有患者均为男性（平均年龄58岁）。 所有病变均发生于肢体或肢带。 主要形态学特征为弥漫性、片状生长模式，仅有局灶性明显的血管分化，上皮样肿瘤细胞伴一定程度的胞浆内空泡化/管腔形成。 免疫组化，所有8例共表达角蛋白以及内皮标志物。 在3例病例中，内皮分化被证实超微结构。 在临床上，深部上皮样血管瘤是高度恶性肿瘤，迅速发展转移。 这些发现扩大了上皮样内皮肿瘤的范围，并提供了角蛋白表达的进一步证据。 胞浆内腔/空泡（有时含有红细胞）的存在与特征性网硬蛋白模式和因子VIII-RAg的显著阳性相结合，提供了与上皮转移的最清晰的区分手段。
We report eight cases of epithelioid angiosarcoma arising in deep, usually intramuscular soft tissue. All the patients were men (mean age, 58). All the lesions arose in a limb or limb girdle. Cardinal morphologic features were the diffuse, sheetlike growth pattern, with only focally apparent vascular differentiation, and epithelioid tumor cells with a degree of intracytoplasmic vacuolation/lumen formation. Immunohistochemically, all eight cases coexpressed keratin as well as endothelial markers. In three cases, endothelial differentiation was confirmed ultrastructurally. Clinically, deep-seated epithelioid angiosarcomas are high-grade neoplasms that rapidly develop metastases. These findings expand the range of recognized epithelioid endothelial tumors and provide further evidence of keratin expression by such lesions. The presence of intracytoplasmic lumina/vacuoles (sometimes containing red blood cells) combined with the characteristic reticulin pattern and striking positivity for Factor VIII-RAg provide the clearest means of distinction from an epithelial metastasis.