Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve.
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.
Trassard
M
M
Département de Pathologie, Centre René Huguenin, Saint-Cloud, France.
Le Doussal
V
V
Bui
B N
BN
Coindre
J M
JM
D000368:Aged; D015415:Biomarkers; D006394:Hemangiosarcoma / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008854:Microscopy, Electron; D009378:Neoplasms, Multiple Primary / Q000737:chemistry / Q000473:pathology*; D009380:Neoplasms, Nerve Tissue / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D009383:Neoplasms, Vascular Tissue / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D009442:Neurilemmoma / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D012584:Sciatic Nerve / Q000737:chemistry / Q000473:pathology*; D013848:Thigh / Q000473:pathology / Q000601:surgery
D015415:Biomarkers