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胸段脊髓硬膜内髓外孤立性纤维瘤

Intradural Extramedullary Solitary Fibrous Tumor of the Thoracic Spinal Cord.

作者信息

Olmsted Zachary T, Tabor Joanna, Doron Omer, Hosseini Hossein, Schneider Daniel, Green Ross, Wahl Samuel J, Sciubba Daniel M, D'Amico Randy S

机构信息

Neurological Surgery, Northwell Health, New York, USA.

Pathology and Laboratory Medicine, Northwell Health, New York, USA.

出版信息

Cureus. 2021 Oct 8;13(10):e18613. doi: 10.7759/cureus.18613. eCollection 2021 Oct.

Abstract

Solitary fibrous tumors (SFTs) are rare soft tissue neoplasms that can impact the central nervous system (CNS). SFTs comprise <1% of all primary CNS tumors. Here, we describe a rare case of intradural, extramedullary SFT arising within the thoracic spine that was treated with surgical resection. Histological features were evaluated and revealed a highly cellular tumor with positive expression of BCL2, CD34, CD99, and STAT6 proteins that are consistent with a diagnosis of SFT. We discuss the use of surgical intervention for long-term disease control of spinal SFT and evaluate the role of postoperative radiation therapy in management strategies. Lastly, we review the literature reports of intradural, extramedullary SFTs in the thoracic spine. The importance of molecular characterization by histopathology to properly determine diagnosis and prognosis is emphasized.

摘要

孤立性纤维瘤(SFTs)是一种罕见的软组织肿瘤,可累及中枢神经系统(CNS)。SFTs占所有原发性CNS肿瘤的比例不到1%。在此,我们描述了一例罕见的胸椎硬膜内、髓外SFT病例,该病例接受了手术切除治疗。对组织学特征进行了评估,结果显示肿瘤细胞高度密集,BCL2、CD34、CD99和STAT6蛋白呈阳性表达,这与SFT的诊断相符。我们讨论了手术干预在脊柱SFT长期疾病控制中的应用,并评估了术后放疗在治疗策略中的作用。最后,我们回顾了胸椎硬膜内、髓外SFT的文献报道。强调了通过组织病理学进行分子特征分析对准确确定诊断和预后的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b4fb/8572645/15a71131e621/cureus-0013-00000018613-i01.jpg

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