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[多发性脑膜瘤与神经纤维瘤病。3例报告]

[Multiple meningiomas and neurofibromatosis. Report of 3 cases].

作者信息

Oliveira M A, Araujo J F, Balbo R J

机构信息

Departamento de Neuro-Psiquiatria, Faculdade de Ciências Médicas da Pontifícia Universidade Católica de Campinas (PUCCAMP), Brasil.

出版信息

Arq Neuropsiquiatr. 1993 Jun;51(2):247-50. doi: 10.1590/s0004-282x1993000200017.

Abstract

Multiple intracranial meningiomas (MIM) may be a specific pathological entity. In general these lesions are associated with neurofibromatosis. The classical clinical picture of neurofibromatosis, as described by von Recklinghausen, may not necessarily be associated with MIM. This possibility is a direct result of the variable penetrability of chromosomic aberrations connected with the chromosome 22. Molecular studies of these tumors confirmed this finding. In our series of 108 patients with intracranial meningiomas only three cases were multiple. In only one of them external stigmata of von Recklinghausen's disease were detected. In the absence of skin manifestations of neurofibromatosis in patients with MIM it is very difficult to diagnosis von Recklinghausen's disease, and the so called "true multiple meningiomas". The authors believe that there are no justificative findings to consider MIM as an independent pathological entity.

摘要

多发性颅内脑膜瘤(MIM)可能是一种特定的病理实体。一般来说,这些病变与神经纤维瘤病相关。正如冯·雷克林豪森所描述的,神经纤维瘤病的典型临床表现不一定与MIM相关。这种可能性是与22号染色体相关的染色体畸变可变外显率的直接结果。对这些肿瘤的分子研究证实了这一发现。在我们的108例颅内脑膜瘤患者系列中,只有3例是多发性的。其中只有1例检测到冯·雷克林豪森病的外部体征。在MIM患者中,若没有神经纤维瘤病的皮肤表现,很难诊断冯·雷克林豪森病以及所谓的“真性多发性脑膜瘤”。作者认为,没有合理的依据将MIM视为一个独立的病理实体。

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[Multiple meningiomas and neurofibromatosis. Report of 3 cases].[多发性脑膜瘤与神经纤维瘤病。3例报告]
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