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眼眶区孤立性纤维瘤:病例报告并强调 STAT-6 的诊断效用

Solitary fibrous tumor of the orbital region: report of a case with emphasis on the diagnostic utility of STAT-6.

机构信息

Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria "Policlinico Vittorio Emanuele", Anatomic Pathology, School of Medicine, University of Catania, Italy.

Department of Ophthalmology, University of Catania, Italy.

出版信息

Pathologica. 2020 Dec;112(4):195-199. doi: 10.32074/1591-951X-9-20.

DOI:10.32074/1591-951X-9-20
PMID:33393522
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8183349/
Abstract

Solitary fibrous tumor (SFT) is a relatively rare soft tissue neoplasm originally described in the pleura. Since its first description, several cases arising in extra-pleural superficial and deep soft tissues have been reported in the literature. SFT arising in the head and neck region is quite rare, representing about the 6% of all SFTs, and the sinonasal tract is the most common involved region, followed by the orbit, the oral cavity and the salivary glands. Herein, we report the clinico-pathologic features of a rare case of SFT of the orbital region, emphasizing the diagnostic role of the immunomarker STAT-6. A 52-year-old female presented to our hospital with a nodular mass in the left orbital region. Histological examination revealed a uniformly hypercellular tumor composed of pale to slightly eosinophilic bland-looking spindle cells arranged in intersecting short fascicles with interspersed stellate-shaped, keloid-type collagen fibers. Notable hypocellular areas, perivascular hyalinization and hemangiopericytoma-like branching vascular pattern were absent. Immunohistochemically, neoplastic cells were diffusely positive for vimentin, CD34 and STAT-6. The introduction of STAT-6 in daily diagnostic practice is helpful to confidentially render a diagnosis of SFT even in the presence of unusual morphology and site.

摘要

孤立性纤维瘤(Solitary fibrous tumor,SFT)是一种相对罕见的软组织肿瘤,最初在胸膜中描述。自首次描述以来,文献中已有几例发生在胸膜外的浅表和深部软组织中的病例报道。发生在头颈部的 SFT 非常罕见,约占所有 SFT 的 6%,鼻窦是最常见的受累部位,其次是眼眶、口腔和唾液腺。在此,我们报告了一例罕见的眼眶区 SFT 的临床病理特征,强调了免疫标志物 STAT-6 的诊断作用。一名 52 岁女性因左眼眶区结节状肿块就诊于我院。组织学检查显示,肿瘤由均匀增生的细胞组成,由淡染至轻度嗜酸性的、外观温和的梭形细胞排列成短束状,穿插有星状、瘢痕样胶原纤维。无明显细胞稀少区、血管周围玻璃样变和血管外皮细胞瘤样分支血管模式。免疫组化染色显示,肿瘤细胞弥漫性表达波形蛋白、CD34 和 STAT-6。STAT-6 的引入有助于在存在不典型形态和部位的情况下,自信地做出 SFT 的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a251/8183349/686cdbc44a49/pathol-2020-04-195-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a251/8183349/669bf8a7e067/pathol-2020-04-195-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a251/8183349/686cdbc44a49/pathol-2020-04-195-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a251/8183349/669bf8a7e067/pathol-2020-04-195-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a251/8183349/686cdbc44a49/pathol-2020-04-195-g002.jpg

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