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.
Département de Pathologie, Centre René Huguenin, Saint-Cloud, France.
M
M
Trassard
V
V
Le Doussal
B N
BN
Bui
J M
JM
Coindre
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