伴有小梁结构的脑膜瘤:BAP1缺乏的先兆。

IF 3 3区 医学 Q2 CLINICAL NEUROLOGY
Noriko Okuno, Takahiro Tomita, Takuya Akai, Satoshi Kuroda, Hiromasa Takakura, Kenichi Hirabayashi, Masakiyo Sasahara, Akira Kurose, Katsuyoshi Takata
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引用次数: 0

摘要

BRCA(乳腺癌基因)相关蛋白1 (BReast CAncer gene -associated protein 1, BAP1)是一种由BAP1基因编码的肿瘤抑制蛋白。BAP1突变和BAP1免疫组化表达缺失是影响脑膜瘤预后的不良因素。这些突变先前在乳头状和横纹肌脑膜瘤中有报道。然而,伴有小梁结构的bap1缺陷脑膜瘤是罕见的,只有一个先前的报告描述了两个病例。在这里,我们提出的情况下,80岁的男性经历了两次脑膜瘤复发超过14年的时间。最初的肿瘤表现为典型的脑膜上皮性脑膜瘤和伴有小梁结构的脑膜瘤,而第二次复发几乎完全是小梁结构。免疫组化分析显示BAP1缺失。该病例强调了小梁结构作为一种组织学模式的重要性,可能表明BAP1丢失和预后不良。鉴于靶向治疗的潜力,需要进一步的研究来确定这种组织学亚型作为预后不良的独立实体。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Meningioma with trabecular architecture: a harbinger of BAP1 deficiency.

BRCA (BReast CAncer gene)-associated protein 1 (BAP1) is a tumor suppressor protein encoded by the BAP1 gene. BAP1 mutations and the loss of BAP1 expression on immunohistochemistry are poor prognostic factors for meningiomas. These mutations have been previously reported in papillary and rhabdoid meningiomas. However, BAP1-deficient meningiomas with a trabecular architecture are rare, with only one previous report describing two cases. Here, we present the case of an 80-year-old male who experienced two recurrences of meningiomas over a 14-year period. The initial tumor exhibited a mixture of typical meningothelial meningioma and meningioma with trabecular architecture, whereas the second recurrence was almost entirely trabecular. Immunohistochemical analysis revealed a BAP1 deficiency. This case highlights the importance of the trabecular architecture as a histological pattern that may indicate BAP1 loss and poor prognosis. Given the potential for targeted therapies, further research is needed to establish this histological subtype as an independent entity with poor prognosis.

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来源期刊
Brain Tumor Pathology
Brain Tumor Pathology 医学-病理学
CiteScore
5.40
自引率
9.10%
发文量
30
审稿时长
>12 weeks
期刊介绍: Brain Tumor Pathology is the official journal of the Japan Society of Brain Tumor Pathology. This international journal documents the latest research and topical debate in all clinical and experimental fields relating to brain tumors, especially brain tumor pathology. The journal has been published since 1983 and has been recognized worldwide as a unique journal of high quality. The journal welcomes the submission of manuscripts from any country. Membership in the society is not a prerequisite for submission. The journal publishes original articles, case reports, rapid short communications, instructional lectures, review articles, letters to the editor, and topics.Review articles and Topics may be recommended at the annual meeting of the Japan Society of Brain Tumor Pathology. All contributions should be aimed at promoting international scientific collaboration.
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