一例罕见的具有软骨肉瘤分化的去分化颅内单发纤维瘤。

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Neuropathology Pub Date : 2024-12-17 DOI:10.1111/neup.13023
Shikhar Chohan, Sana Ahuja, Sufian Zaheer
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引用次数: 0

摘要

本报告详细介绍了一例罕见病例:一名 30 岁女性出现神经系统症状,包括头痛、癫痫发作和左侧肢体无力。影像学检查发现,她的右侧大脑镰旁区域有一个肿块。手术切除发现肿瘤有两个不同的组成部分。第一个部分表现出典型的单发纤维瘤(SFT)特征,具有典型的成纤维细胞和分支血管。第二部分显示为高级别肉瘤,并伴有软骨肉瘤分化,这是单发纤维瘤的罕见特征。免疫组化证实,与传统的 SFT 相比,肉瘤区的 STAT6 表达减少,出现了去分化。该病例凸显了处理去分化 SFT 的挑战,尤其是在脑部,因为手术的限制增加了风险。尽管这种病例很少见,但它强调了识别这种变异以进行适当诊断和管理的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare case of dedifferentiated intracranial solitary fibrous tumor with chondrosarcomatous differentiation.

This report details a rare case of a 30-year-old female presenting with neurological symptoms, including headaches, seizures, and left-sided weakness. Imaging revealed a mass in the right parafalcine region of her brain. Surgical resection identified a tumor with two distinct components. The first component exhibited characteristics of a classic solitary fibrous tumor (SFT) with typical fibroblastic cells and branching blood vessels. The second component showed high-grade sarcoma with chondrosarcomatous differentiation, a rare feature in SFT. Immunohistochemistry confirmed dedifferentiation with decreased STAT6 expression in the sarcomatous areas compared to the conventional SFT. This case highlights the challenges of managing dedifferentiated SFTs, especially in the brain, where surgical limitations increase risks. Despite the rarity of this presentation, it emphasizes the importance of recognizing this variant for appropriate diagnosis and management.

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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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