Epithelioid angiosarcoma arising in schwannoma: report of three Chinese cases with review of the literature.
Angiosarcoma arising in a schwannoma is extremely rare with only eleven cases having been reported in the English literature. We describe here three further cases occurring in adult males with a pre-existing longstanding schwannoma. The tumor arose each from the vagus, ischiadic and adrenergic nerve respectively. None of the patients had von Recklinghausen's disease. Microscopically, the tumor was composed of a mixture of a benign schwannoma and an epithelioid angiosarcoma. The two components changed abruptly within the tumor. The endothelial cell differentiation was confirmed by immunohistochemistry. A review of published reports, including the present cases, suggests a poor prognosis with a high rate of local recurrence, distant metastasis and mortality.
Department of Pathology, Fujian Provincial Cancer Hospital, The Teaching Hospital of Fujian Medical University, Fuzhou, China.
Chao
C
Li
Ying
Y
Chen
Hong
H
Zhang
Xiongwei
X
Zheng
Jian
J
Wang
D000320:Adrenergic Fibers / Q000473:pathology; D000328:Adult; D000368:Aged; D002471:Cell Transformation, Neoplastic; D003131:Combined Modality Therapy; D004730:Endothelium, Vascular / Q000378:metabolism / Q000473:pathology; D015622:Epithelioid Cells / Q000378:metabolism / Q000473:pathology*; D006394:Hemangiosarcoma / Q000378:metabolism / Q000556:secondary* / Q000601:surgery; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary; D009442:Neurilemmoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D012584:Sciatic Nerve / Q000473:pathology; D016896:Treatment Outcome; D014630:Vagus Nerve / Q000473:pathology