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腹膜外间隙具有非典型特征的孤立性纤维瘤:10 年随访的良性临床过程。病例报告及文献复习。

Solitary fibrous tumor with atypical features of the paravesical space: benign clinical course at the 10-years follow-up. Report of a case and review of the literature.

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 Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria "Policlinico Vittorio Emanuele", Anatomy, School of Medicine, University of Catania, Italy and "G.B. Morgagni" Mediterranean Foundation, Catania.

出版信息

Pathologica. 2020 Dec;112(4):200-209. doi: 10.32074/1591-951X-126.

Abstract

Extra-pleural solitary fibrous tumor (SFT) is a relatively rare soft tissue neoplasm, with only rare cases reported in the pelvic cavity. Most SFTs are histologically benign, with only a few malignant cases reported in the literature so far. We report a rare case of SFT arising in the paravesical space of a 79-year-old man. Histologically the tumor corresponds to an "" according to a risk stratification scheme for metastatic potential, which incorporates patient age, tumor size, mitotic activity and necrosis. Notably tumor showed a benign clinical course without evidence of local recurrence after a 10-years follow-up. Tumor was composed of both spindle and epithelioid cells variably set in a fibro-myxoid stroma, with focal pleomorphic, necrotic and highly mitotic (> 4 mitoses/10HPF) areas. Immunohistochemistry, showing a diffuse CD34 and STAT6 immunoreactivity, supported the diagnosis of SFT. The present case emphasizes that the clinical course of the pelvic SFTs with atypical morphological features is unpredictable on the basis of morphology alone, and thus the term "" should be preferred to "".

摘要

壁外孤立性纤维瘤(SFT)是一种相对罕见的软组织肿瘤,仅在盆腔内有少数罕见病例报道。大多数 SFT 在组织学上为良性,迄今为止文献中仅报道了少数恶性病例。我们报告了一例罕见的发生在 79 岁男性的壁旁间隙的 SFT。根据转移潜能的风险分层方案,肿瘤的组织学符合“”,该方案纳入了患者年龄、肿瘤大小、有丝分裂活性和坏死等因素。值得注意的是,肿瘤表现出良性的临床过程,在 10 年随访后没有局部复发的证据。肿瘤由梭形和上皮样细胞组成,不同程度地存在于纤维黏液样基质中,伴有局灶性多形性、坏死和高度有丝分裂(>4 个有丝分裂/10HPF)区域。免疫组化显示弥漫性 CD34 和 STAT6 免疫反应性,支持 SFT 的诊断。本病例强调,仅基于形态学,具有非典型形态特征的盆腔 SFT 的临床过程是不可预测的,因此应首选“”一词。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/44eb/8183344/5ff6b711b3a5/pathol-2020-04-200-g001.jpg

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