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横纹肌样脑膜瘤:一项临床病理病例系列研究。

Rhabdoid papillary meningioma: a clinicopathologic case series study.

机构信息

Department of Pathology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.

出版信息

Neuropathology. 2011 Dec;31(6):599-605. doi: 10.1111/j.1440-1789.2011.01201.x. Epub 2011 Mar 7.

DOI:10.1111/j.1440-1789.2011.01201.x
PMID:21382093
Abstract

World Health Organization (WHO) grade III meningiomas are subclassified on the basis of their architectural pattern into papillary and rhabdoid subtypes. Some meningiomas even combine papillary architecture with rhabdoid cytology. Additionally, they always show malignant histological features, follow an aggressive clinical course and tend to spread through the CSF after frequent local recurrence. We render the first series of rhabdoid papillary meningioma with review of the literature to further elucidate its biological behavior. From six patients (three male, three female), nine specimens of rhabdoid papillary meningioma were obtained between 1994 and 2010. Correlations of histologic parameters, immunohistochemical study, and clinical features were assessed. The mean age of patients was 44.7 years at their first operation. The mean postoperative follow-up period was 63.2 months. Five patients experienced tumor recurrence, and one of them died from the disease after diffuse leptomeningeal dissemination. The mean time to first recurrence was 28 months. Only one patient was free of tumoral recurrence after an 8-year follow-up. Immunohistochemically, all tumors were positive for vimentin and epithelial membrane antigen. MIB-1 labeling indices were higher following tumor recurrence. The present study expands the clinicopathologic horizon of rhabdoid papillary meningioma and suggests that it will behave aggressively based on its histology and concomitant features of atypia or malignancy or high MIB-1 labeling indices. Close follow-up and aggressive treatments of these tumors are warranted.

摘要

世界卫生组织(WHO)将 III 级脑膜瘤基于其结构模式进一步细分为乳头型和横纹肌样型亚型。一些脑膜瘤甚至结合了乳头状结构和横纹肌样细胞学特征。此外,它们通常表现出恶性组织学特征,具有侵袭性的临床病程,并在频繁局部复发后倾向于通过 CSF 扩散。我们报告了首例横纹肌样乳头状脑膜瘤,并复习了文献,以进一步阐明其生物学行为。从 1994 年至 2010 年,我们从 6 名患者(3 名男性,3 名女性)中获得了 9 个横纹肌样乳头状脑膜瘤标本。评估了组织学参数、免疫组织化学研究和临床特征之间的相关性。患者的平均年龄为首次手术时的 44.7 岁。平均术后随访时间为 63.2 个月。5 名患者出现肿瘤复发,其中 1 名患者因弥漫性软脑膜播散而死于该疾病。首次复发的平均时间为 28 个月。在 8 年的随访后,只有 1 名患者无肿瘤复发。免疫组织化学染色显示,所有肿瘤均表达波形蛋白和上皮膜抗原。肿瘤复发后 MIB-1 标记指数较高。本研究扩展了横纹肌样乳头状脑膜瘤的临床病理范围,并表明根据其组织学和伴随的异型性或恶性或高 MIB-1 标记指数,它将具有侵袭性。需要对这些肿瘤进行密切随访和积极治疗。

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