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播散性恶性胸膜孤立性纤维瘤

Disseminated malignant solitary fibrous tumor of the pleura.

作者信息

Zhang Hongquan, Lucas David R, Pass Harvey I, Che Mingxin

机构信息

Department of Pathology, Harper University Hospital, Wayne State University and Karmanos Cancer Institute, Detroit, MI 48201, USA.

出版信息

Pathol Int. 2004 Feb;54(2):111-5. doi: 10.1111/j.1440-1827.2004.01591.x.

Abstract

Solitary fibrous tumor (SFT) of the pleura typically forms a localized pleura-based mass, and most are benign. A rare case of disseminated malignant SFT of the pleura is reported. The patient was a 71-year-old man who presented with complaints of shortness of breath to his primary care physician. A diagnosis of malignant mesothelioma was suspected, based on clinical, radiological and needle biopsy findings. He was referred to our institution for surgery. An extrapleural pneumonectomy, encompassing all pleural masses, was performed. Gross examination of the resected specimen was remarkable for numerous masses, ranging in size from 0.2 to 13.5 cm, covering the majority of the visceral pleura. Histologically, the tumor was composed of short spindle cells admixed with variable proportions of collagenous stroma. There were great intra- and intertumoral heterogeneity in tumor growth pattern, cellularity, pleomorphism and mitoses. Histologically malignant areas were present in all of the masses examined. The neoplastic cells were diffusely and intensely positive for bcl-2. Most tumor cells were also strongly stained for CD34 and CD99. Staining for cytokeratin was negative. The tumor also revealed p53 over-expression. Thus, the histological and immunohistochemical features of the tumor were consistent with a disseminated malignant SFT. This report shows that SFT rarely presents with disseminated pleural involvement, and a panel with CD34, bcl-2 and cytokeratin are valuable for differentiating SFT from malignant mesothelioma and other malignant spindle cell neoplasms of the pleura.

摘要

胸膜孤立性纤维瘤(SFT)通常形成一个基于胸膜的局限性肿块,大多数为良性。本文报道了一例罕见的胸膜播散性恶性SFT病例。患者为一名71岁男性,因呼吸急促向其初级保健医生就诊。根据临床、影像学和针吸活检结果,怀疑为恶性间皮瘤。他被转诊至我院接受手术。实施了包括所有胸膜肿块的胸膜外全肺切除术。对切除标本的大体检查显示有大量肿块,大小从0.2厘米至13.5厘米不等,覆盖了大部分脏层胸膜。组织学上,肿瘤由短梭形细胞组成,并混有不同比例的胶原性间质。肿瘤生长模式、细胞密度、多形性和有丝分裂在肿瘤内和肿瘤间存在很大异质性。在所有检查的肿块中均存在组织学上的恶性区域。肿瘤细胞bcl-2弥漫性且强烈阳性。大多数肿瘤细胞CD34和CD99也呈强阳性染色。细胞角蛋白染色为阴性。肿瘤还显示p53过表达。因此,肿瘤的组织学和免疫组化特征与播散性恶性SFT一致。本报告表明,SFT很少表现为胸膜播散性受累,CD34、bcl-2和细胞角蛋白组合对于鉴别SFT与恶性间皮瘤及其他胸膜恶性梭形细胞瘤很有价值。

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