Epithelioid angiosarcoma arising in schwannoma: Report of three Chinese cases with review of the literature

• Published in: Pathology international Vol. 62; no. 7; pp. 500 - 505
• Main Authors: Li, Chao, Chen, Ying, Zhang, Hong, Zheng, Xiongwei, Wang, Jian
• Format: Journal Article
• Language: English
• Published: Melbourne, Australia Blackwell Publishing Asia 01.07.2012
• Subjects: Adrenergic Fibers - pathology; Adult; Aged; angiosarcoma; Cell Transformation, Neoplastic; Combined Modality Therapy; Endothelium, Vascular - metabolism; Endothelium, Vascular - pathology; Epithelioid Cells - metabolism; Epithelioid Cells - pathology; Hemangiosarcoma - metabolism; Hemangiosarcoma - secondary; Hemangiosarcoma - surgery; Humans; immunohistochemistry; Lymph Nodes - pathology; Lymphatic Metastasis; Male; Middle Aged; Neoplasms, Second Primary; Neurilemmoma - metabolism; Neurilemmoma - pathology; Neurilemmoma - surgery; prognosis; schwannoma; Sciatic Nerve - pathology; Treatment Outcome; Vagus Nerve - pathology
• ISSN: 1320-5463; 1440-1827; 1440-1827
• DOI: 10.1111/j.1440-1827.2012.02827.x

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.