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一例巨大胸膜孤立性纤维瘤:罕见病例的诊断意义及文献复习

A giant solitary fibrous tumor of the pleura: diagnostic implications in an unusual case with literature review.

作者信息

Mune Swati, Rekhi Bharat, More Nutan, Jambhekar Nirmala A

机构信息

Department of Pathology, Tata Memorial Hospital, Mumbai, India.

出版信息

Indian J Pathol Microbiol. 2010 Jul-Sep;53(3):544-7. doi: 10.4103/0377-4929.68289.

Abstract

A solitary fibrous tumor of the pleura (SFTP) is an uncommon tumor, in contrast to a relatively common mesothelioma in pleura. Its average size varies from 6 to 8 cm. We report herein a case of a giant SFTP in a 53-year-old man who presented with cough since five years along with chest pain on the left side and progressive dyspnea since two months. Radiological findings revealed a large pleural mass measuring 25 cm in its largest dimension, filling the pleural cavity with effusion. Biopsy showed a spindle cell tumor with areas of dense sclerosis. Subsequent excision unraveled a large multinodular, grey-white tumor, histologically, composed of spindle cells in a 'patternless' arrangement in dense collagenous stroma with areas of hyalinization. Focal areas showed hypercellularity with atypia and mitoses, but less than 4/10 High-Power Field, unassociated with necrosis. On immunohistochemistry, tumor cells showed diffuse positivity with vimentin, CD34 and BCL2 along with cytoplasmic positivity for MIC2 (CD99), whereas cytokeratin, EMA, calretinin and HBME-1 were negative. Diagnosis of an SFTP was substantiated over a close differential of a desmoplastic mesothelioma. In view of atypical features, a close follow-up of the case was recommended.

摘要

胸膜孤立性纤维瘤(SFTP)是一种罕见的肿瘤,与相对常见的胸膜间皮瘤形成对比。其平均大小在6至8厘米之间。我们在此报告一例53岁男性的巨大SFTP病例,该患者自五年前起出现咳嗽,伴有左侧胸痛,近两个月来进行性呼吸困难。影像学检查发现一个最大径为25厘米的巨大胸膜肿块,伴有胸腔积液。活检显示为梭形细胞瘤,有致密硬化区域。随后的切除标本显示为一个巨大的多结节灰白色肿瘤,组织学上,由梭形细胞在致密胶原性基质中呈“无模式”排列组成,伴有玻璃样变区域。局灶区域显示细胞增多、异型性和核分裂象,但每10个高倍视野少于4个,且无坏死。免疫组化检查显示,肿瘤细胞波形蛋白、CD34和BCL2弥漫阳性,MIC2(CD99)呈胞质阳性,而细胞角蛋白、EMA、钙视网膜蛋白和HBME - 1均为阴性。与促纤维组织增生性间皮瘤进行仔细鉴别后,确诊为SFTP。鉴于存在非典型特征,建议对该病例进行密切随访。

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