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[脊索样脑膜瘤。临床、神经放射学及解剖病理学特征。附1例新病例并文献复习]

[Chordoid meningiomas. Clinical, neuroradiological and anatomopathological aspects. Apropos of a new case and review of the literature].

作者信息

Civit T, Baylac F, Taillandier L, Auque J, Hepner H

机构信息

Service de Neurochirurgie, Hôpital Saint-Julien, Nancy.

出版信息

Neurochirurgie. 1997;43(5):308-13.

PMID:9686236
Abstract

A 21-year old woman underwent surgery in December 1996 for the removal of a presumed tuberculum sellae meningioma. However, some radio-clinical findings were proved somewhat intriguing:the patient's age, the presence of inflammatory and febrile syndromes together with the diagnosis of aseptic meningitis associated with perilesional edema intensity (an unusual feature in such cases) made us challenge the initial neuroradiological diagnosis evoked in connection with the tumoral location and dural attachment pattern. A right sub-fronto-temporal approach allowed complete tumor resection (confirmed with a postoperative MRI) and clinical recovery of the patient. But while pathological examination suggested a chordoma, the study of immunohistochemical stains revealed a meningioma. The final diagnosis was chordoid meningioma. Our review of the literature has shown that chordoid meningiomas display several areas of physaliferous cells which give the tumor a chordoma-like aspect. However, the results of immunohistochemical studies along with the location of the tumor were not consistent with the diagnosis of chordoma. Eight cases of chordoid meningiomas are reviewed in the literature. They are described as inducing systemic symptoms, particularly anemia. They could also be linked to Castleman's syndrome according to Kepes et al. After careful evaluation, we retained the hypothesis of a cause and effect relationship between the local and generalised inflammatory syndrome and chordoid meningioma.

摘要

一名21岁女性于1996年12月接受手术,以切除疑似鞍结节脑膜瘤。然而,一些放射学和临床检查结果颇具耐人寻味之处:患者的年龄、炎症和发热综合征的存在,以及与病灶周围水肿强度相关的无菌性脑膜炎诊断(这在此类病例中是一个不寻常的特征),使我们对最初基于肿瘤位置和硬脑膜附着模式做出的神经放射学诊断产生了质疑。采用右额颞下入路实现了肿瘤的完整切除(术后MRI证实),患者临床康复。尽管病理检查提示为脊索瘤,但免疫组化染色研究显示为脑膜瘤。最终诊断为脊索样脑膜瘤。我们对文献的回顾表明,脊索样脑膜瘤有几个黏液样细胞区域,使肿瘤呈现出脊索瘤样外观。然而,免疫组化研究结果以及肿瘤的位置与脊索瘤的诊断并不相符。文献中回顾了8例脊索样脑膜瘤病例。它们被描述为可引发全身症状,尤其是贫血。根据凯佩斯等人的研究,它们还可能与卡斯尔曼病有关。经过仔细评估,我们保留了局部和全身性炎症综合征与脊索样脑膜瘤之间存在因果关系的假设。

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