探索血管周围上皮样细胞肿瘤（PEComas）在中枢神经系统：从两个病例报告和全面的文献综述的见解。

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Child's Nervous System Pub Date : 2025-05-27 DOI:10.1007/s00381-025-06845-x

Rik Demaerel, Brecht Decraene, Pegah Masrori, Lukas Marcelis, Raf Sciot, Philippe Demaerel, Frank Van Calenbergh

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引用次数: 0

摘要

血管周围上皮样细胞瘤（PEComas）是中枢神经系统（CNS）中一种罕见而有趣的肿瘤。本报告描述了两个独特的病例，突出了不同的起源和临床行为，为有限的关于中枢神经系统PEComas的知识提供了宝贵的贡献。尽管PEComas很罕见，但在鉴别诊断间充质肿瘤时应考虑其临床、放射学和组织分子特征的非典型组合，特别是当黑色素细胞和/或肌样细胞标志物存在时。mTOR抑制剂治疗可以潜在地稳定大量更具侵袭性的PEComas，甚至那些位于中枢神经系统。

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Exploring perivascular epithelioid cell tumors (PEComas) in the CNS: insights from two case reports and a comprehensive literature review.

Perivascular epithelioid cell tumors (PEComas) represent a rare and intriguing subset of neoplasms within the central nervous system (CNS). This report describes two unique cases that highlight distinct origins and clinical behaviors, offering valuable contributions to the limited body of knowledge regarding CNS PEComas. Despite their rarity, PEComas should be considered in the differential diagnosis of mesenchymal tumors that display an atypical combination of clinical, radiological, and histomolecular features, especially when melanocytic and/or myoid markers are present. Treatment with mTOR inhibitors can potentially stabilize the disease in a significant number of more aggressive PEComas, even those located in the CNS.

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来源期刊

Child's Nervous System 医学-临床神经学

CiteScore

3.00

自引率

7.10%

发文量

322

审稿时长

3 months

期刊介绍： The journal has been expanded to encompass all aspects of pediatric neurosciences concerning the developmental and acquired abnormalities of the nervous system and its coverings, functional disorders, epilepsy, spasticity, basic and clinical neuro-oncology, rehabilitation and trauma. Global pediatric neurosurgery is an additional field of interest that will be considered for publication in the journal.