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Epithelioid angiosarcoma arising in schwannoma: Report of three Chinese cases with review of the literature
Author(s) -
Li Chao,
Chen Ying,
Zhang Hong,
Zheng Xiongwei,
Wang Jian
Publication year - 2012
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2012.02827.x
Subject(s) - schwannoma , angiosarcoma , medicine , pathology , immunohistochemistry , gist , metastasis , hemangiosarcoma , cancer , stromal cell
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.