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具有小梁结构的脑膜瘤:BAP1缺乏的预兆。

Meningioma with trabecular architecture: a harbinger of BAP1 deficiency.

作者信息

Okuno Noriko, Akai Takuya, Kuroda Satoshi, Takakura Hiromasa, Hirabayashi Kenichi, Sasahara Masakiyo, Kurose Akira, Takata Katsuyoshi

机构信息

Department of Pathology, Academic Assembly Faculty of Medicine, University of Toyama, Toyama, 930-0194, Japan.

Department of Diagnostic Pathology, Toyama University Hospital, Toyama, Toyama, Japan.

出版信息

Brain Tumor Pathol. 2025 Sep 13. doi: 10.1007/s10014-025-00516-0.

Abstract

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.

摘要

乳腺癌(BRCA)相关蛋白1(BAP1)是一种由BAP1基因编码的肿瘤抑制蛋白。BAP1突变以及免疫组化检测中BAP1表达缺失是脑膜瘤预后不良的因素。这些突变先前已在乳头状和横纹肌样脑膜瘤中报道过。然而,具有小梁状结构的BAP1缺陷型脑膜瘤很罕见,之前仅有一篇报道描述了两例。在此,我们报告一例80岁男性患者,其在14年期间经历了两次脑膜瘤复发。最初的肿瘤表现为典型的脑膜内皮型脑膜瘤和具有小梁状结构的脑膜瘤的混合,而第二次复发几乎完全是小梁状的。免疫组化分析显示BAP1缺陷。该病例突出了小梁状结构作为一种组织学模式的重要性,它可能提示BAP1缺失和预后不良。鉴于靶向治疗的潜力,需要进一步研究以将这种组织学亚型确立为预后不良的独立实体。

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