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中枢神经系统上皮样孤立性纤维瘤

Epithelioid solitary fibrous tumor of the central nervous system.

作者信息

Fu Jing, Zhang Rui, Zhang Hongying, Bu Hong, Chen Huijiao, Yin Xiangli, Zhang Zhang, Wei Bing

机构信息

Department of Pathology, West China Hospital, Sichuan University, Guo Xue Xiang 37, 610041 Chengdu, Sichuan, China.

出版信息

Clin Neurol Neurosurg. 2012 Jan;114(1):72-6. doi: 10.1016/j.clineuro.2011.07.021. Epub 2011 Sep 9.

Abstract

Epithelioid solitary fibrous tumor (SFT) has recently been reported and is an extremely rare soft-tissue neoplasm. Herein we present an epithelioid SFT attached to the falx cerebri occurring in a Chinese woman. This patient underwent gross-total tumor resection at the age of 30 years and recurred 68 months following the initial total resection. Histologically, the initial lesion exhibited features of classic spindle cell SFT. In contrast, the recurrent tumor demonstrated exclusively epithelioid morphology with significant atypia. Both the original and recurrent lesions showed positivity for vimentin, CD34, Bcl-2, and CD99, whereas were negative for all the remaining antibodies. The epithelioid feature in SFT seems to be associated with a more aggressive clinical behavior in this case and more cases are awaited to verify this possibility. To the best of authors' knowledge, the present case is the first published example of SFT with epithelioid feature in the central nervous system.

摘要

上皮样孤立性纤维瘤(SFT)最近有报道,是一种极其罕见的软组织肿瘤。在此我们报告一例附着于大脑镰的上皮样SFT,发生于一名中国女性。该患者30岁时接受了肿瘤全切手术,首次全切术后68个月复发。组织学上,最初的病变表现为经典的梭形细胞SFT特征。相比之下,复发肿瘤仅表现为上皮样形态且有明显异型性。原发和复发病变均显示波形蛋白、CD34、Bcl-2和CD99阳性,而其余所有抗体均为阴性。在该病例中,SFT的上皮样特征似乎与更具侵袭性的临床行为相关,还需要更多病例来证实这种可能性。据作者所知,本病例是中枢神经系统中首例有上皮样特征的SFT的报道。

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