Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve

Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve
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DOI:
10.1097/00000478-199611000-00014
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发表时间:
1996-11-01
影响因子:
5.6
通讯作者:
Coindre, JM
Coindre, JM
中科院分区:
医学1区
文献类型:
--
作者:
Trassard, M;LeDoussal, V;Coindre, JM

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血管瘤很少发生在周围神经或周围神经鞘肿瘤内。我们报告一位65岁的男性,其右大腿的良性神经鞘瘤(神经鞘瘤)中发生上皮样血管肉瘤。组织学上,切除的肿瘤是一个高级别的未分化肉瘤,主要是安排在固体片或巢,上皮样细胞组成。通过因子VIII R-ag、荆豆-I、CD 34、CD 31、BNH 9和波形蛋白免疫反应性,沿着偶尔出现的韦伯-帕拉德小体的超微结构证据,提示肿瘤的内皮起源。在这个部位,上皮样血管肉瘤应该与伴有上皮样改变的神经鞘瘤恶变相鉴别。这一观察强调了免疫组化和超微结构分析在鉴别诊断具有上皮样肉瘤特征的血管性肿瘤中的重要性。
Angiosarcomas rarely develop within a peripheral nerve or a peripheral nerve sheath tumor. We describe an epithelioid angiosarcoma that arose in a benign schwannoma (neurilemoma) of the right thigh in a 65-year-old man who did not have von Recklinghausen's disease. Histologically, the resected tumor was a high-grade undifferentiated sarcoma that was predominantly arranged in solid sheets or nests and composed of epithelioid cells. The endothelial origin of the tumor was suggested by Factor VIII R-ag, Ulex europaeus-I, CD34, CD31, BNH9, and vimentin immunoreactivity, along with the ultrastructural evidence of occasional Weibel-Palade bodies. In this location, epithelioid angiosarcoma should be distinguished from malignant transformation of a schwannoma with epithelioid changes. This observation stresses the importance of immunohistochemical and ultrastructural analysis in the differential diagnosis of vascular tumors with features of epithelioid sarcoma.