M. Trassard et al., ANGIOSARCOMA ARISING IN A SOLITARY SCHWANNOMA (NEURILEMOMA) OF THE SCIATIC-NERVE, The American journal of surgical pathology, 20(11), 1996, pp. 1412-1417
Angiosarcomas rarely develop within a peripheral nerve or a peripheral
nerve sheath tumor. We describe an epithelioid angiosarcoma that aros
e 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 wa
s predominantly arranged in solid sheets or nests and composed of epit
helioid cells. The endothelial origin of the tumor was suggested by Fa
ctor VIII R-ag, Ulex europaeus-I, CD34, CD31, BNH9, and vimentin immun
oreactivity, along with the ultrastructural evidence of occasional Wei
bel-Palade bodies. In this location, epithelioid angiosarcoma should b
e distinguished from malignant transformation of a schwannoma with epi
thelioid changes. This observation stresses the importance of immunohi
stochemical and ultrastructural analysis in the differential diagnosis
of vascular tumors with features of epithelioid sarcoma.