Department of Pathology, Fujian Provincial Cancer Hospital, The Teaching Hospital of Fujian Medical University, Fuzhou, China.
Pathol Int. 2012 Jul;62(7):500-5. doi: 10.1111/j.1440-1827.2012.02827.x. Epub 2012 Apr 26.
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.
在神经鞘瘤中发生的血管肉瘤极为罕见，英文文献中仅报道了 11 例。我们在此描述了另外 3 例发生于成年男性、且存在长期存在的神经鞘瘤的病例。肿瘤分别来自迷走神经、坐骨神经和肾上腺素能神经。这些患者均无冯·雷克林豪森氏病。显微镜下，肿瘤由良性神经鞘瘤和上皮样血管肉瘤混合组成。两种成分在肿瘤内突然变化。内皮细胞分化通过免疫组织化学得到证实。对已发表的报告进行回顾，包括本病例在内，提示预后不良，局部复发、远处转移和死亡率均较高。
