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鼻窦孤立性纤维瘤:6例临床病理研究并文献综述

Sinonasal Tract Solitary Fibrous Tumor: A Clinicopathologic Study of Six Cases with a Comprehensive Review of the Literature.

作者信息

Thompson Lester D R, Lau Sean K

机构信息

Department of Pathology, Woodland Hills Medical Center, Southern California Permanente Medical Group, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA.

Orange County-Anaheim Medical Center, Anaheim, CA, USA.

出版信息

Head Neck Pathol. 2018 Dec;12(4):471-480. doi: 10.1007/s12105-017-0878-y. Epub 2017 Dec 27.

Abstract

Solitary fibrous tumors (SFTs) are well recognized in the head and neck region, but rarely arise in the sinonasal tract (SNT). Six primary SNT SFTs were identified in the files of Southern California Permanente Medical Group between 2006 and 2017. The patients included five males and one female ranging in age from 33 to 72 years (mean 52 years), most of whom presented clinically with nasal obstruction. Three tumors involved the nasal cavity alone, one involved the paranasal sinuses, and two involved both the nasal cavity and paranasal sinuses. Histologically, the tumors were characterized by a variably cellular proliferation of cytologically bland spindle cells within a collagenous stroma with prominent interspersed branching vessels. Mitotic activity was low (range 0-2 per 10 high power fields) and there was no evidence of pleomorphism or tumor necrosis. Surface ulceration was noted. By immunohistochemistry, the lesional cells were positive for CD34, STAT6 and bcl-2. Clinical follow up information was available for all patients (range 32-102 months; mean 72 months). There were no recurrences or metastases and all were alive with no evidence of disease at last follow-up. SFTs rarely affect the SNT, but should be considered in the differential diagnosis of SNT mesenchymal lesions. Immunohistochemical expression of STAT6 can aid in diagnosis and separation of SFT from other spindle cell lesions occurring at this anatomic site. In combination with cases reported in the literature, primary SNT SFT behave in an indolent manner with conservative treatment.

摘要

孤立性纤维性肿瘤(SFTs)在头颈部区域较为常见,但很少发生于鼻窦道(SNT)。2006年至2017年间,在南加州永久医疗集团的档案中识别出6例原发性鼻窦道SFT。患者包括5名男性和1名女性,年龄在33至72岁之间(平均52岁),大多数患者临床上表现为鼻塞。3例肿瘤仅累及鼻腔,1例累及鼻窦,2例累及鼻腔和鼻窦。组织学上,肿瘤的特征是在胶原基质内有不同程度的细胞增生,由细胞形态温和的梭形细胞组成,并有明显的分支血管穿插其中。有丝分裂活性较低(每10个高倍视野0-2个),没有多形性或肿瘤坏死的证据。可见表面溃疡。免疫组化显示,病变细胞CD34、STAT6和bcl-2呈阳性。所有患者均有临床随访信息(范围32-102个月;平均72个月)。无复发或转移,最后一次随访时所有患者均存活,无疾病证据。SFT很少影响鼻窦道,但在鼻窦道间叶性病变的鉴别诊断中应予以考虑。STAT6的免疫组化表达有助于SFT的诊断及与该解剖部位发生的其他梭形细胞病变相鉴别。结合文献报道的病例,原发性鼻窦道SFT采用保守治疗,病情进展缓慢。

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