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[胸膜外孤立性纤维瘤伴罕见组织学表现:7例临床病理分析]

[Extrapleural solitary fibrous tumor with uncommon histology: a clinicopathologic analysis of 7 cases].

作者信息

Zhao M, Yang Z R, Wang Y B, Chen Y, Qi G W, Yan Y J, Xu W J, Ru G Q, He X L

机构信息

Department of Pathology, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou 310014, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2018 Jan 8;47(1):51-56. doi: 10.3760/cma.j.issn.0529-5807.2018.01.010.

Abstract

To investigate the clinicopathologic characteristics, immunophenotypes, and differential diagnostic features of extra-pleural solitary fibrous tumor (SFT) with uncommon histology. Seven cases of extra-pleural SFT with uncommon histology were collected during January 2015 and December 2016 in Zhejiang Provincal People's Hospital; the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed. EnVision method was used for immunohistochemical staining of STAT6, CD34 and other differential diagnosis associated markers. There were five male and two female patients, age from 23 to 54 years (mean=39 years). Three tumors were located in the soft tissue of head and neck, two in trunk subcutaneous soft tissue, one in sella region, and one in the kidney. Grossly the tumors ranged from 0.4 to 8.0 cm (mean=3.1 cm). Microscopically, all three head and neck cases resembled giant cell angiofibroma/giant cell subtype SFT, and one case showed sheet-like pattern of the multinucleated syncytial cells, creating a biphasic arrangement similar to myofibroma. Both truncal tumor resembled lipomatous type SFT, with one similar to dermatofibrosarcoma protuberans and the other to atypical spindle cell lipomatous tumor. The sella tumor showed morphology of a conventional SFT with high grade sarcomatous transformation. The renal tumor demonstrated a malignant SFT with entrapped benign renal tubules, mimicking a biphase synovial sarcoma or a malignant mixed epithelial and stromal tumor. By immunohistochemistry, all seven SFTs showed diffuse and strong nuclear reactivity to antibody against STAT6. Extra-pleural SFTs show a significant heterogeneity of morphology and biological behavior which could cause differential confusion.Careful attention to its characteristic histomorphology with the use of STAT6 immunohistochemistry can help distinguish this tumor from its many mimickers.

摘要

探讨具有罕见组织学表现的胸膜外孤立性纤维性肿瘤(SFT)的临床病理特征、免疫表型及鉴别诊断特点。收集2015年1月至2016年12月期间浙江省人民医院收治的7例具有罕见组织学表现的胸膜外SFT病例;分析其临床及影像学特征、组织形态学、免疫表型及预后。采用EnVision法对STAT6、CD34及其他鉴别诊断相关标志物进行免疫组化染色。患者中男性5例,女性2例,年龄23~54岁(平均39岁)。3例肿瘤位于头颈部软组织,2例位于躯干皮下软组织,1例位于鞍区,1例位于肾脏。大体肿瘤大小为0.4~8.0 cm(平均3.1 cm)。镜下,3例头颈部病例类似巨细胞血管纤维瘤/巨细胞亚型SFT,1例表现为多核合体细胞的片状排列,形成类似肌纤维瘤的双相结构。2例躯干肿瘤类似脂肪瘤样型SFT,1例类似隆突性皮肤纤维肉瘤,另1例类似非典型梭形细胞脂肪瘤。鞍区肿瘤表现为具有高级别肉瘤样转化的传统SFT形态。肾脏肿瘤为恶性SFT,伴有包绕的良性肾小管,类似双相滑膜肉瘤或恶性混合性上皮和间质肿瘤。免疫组化显示,所有7例SFT对STAT6抗体均呈弥漫性强核反应。胸膜外SFT在形态和生物学行为上具有显著异质性,可导致鉴别困难。仔细观察其特征性组织形态学表现并结合STAT6免疫组化有助于将该肿瘤与其众多相似病变相鉴别。

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