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[中枢神经系统海绵状血管瘤的组织病理学]

[Histopathology of central nervous system cavernomas].

作者信息

Mosnier J-F, Brunon J, Nuti C

机构信息

Laboratoire d'anatomie pathologique B, hôpital Laennec, CHU de Nantes, boulevard Jacques-Monod, 44093 Nantes cedex 01, France.

出版信息

Neurochirurgie. 2007 Jun;53(2-3 Pt 2):131-5. doi: 10.1016/j.neuchi.2007.02.004.

Abstract

Central nervous system cavernomas are vascular malformations, which occur in two circumstances: sporadic forms and familial autosomal dominant forms. The lesion consists of enlarged, closely packed vessels without interposition of brain parenchyma, surrounded by hemosiderin and gliosis, calcified in few cases. In 80% of sporadic forms the lesion is unique, multiple lesions are rare (median: 4). In familial forms the lesions are always multiple. Cavernomas are often associated with other vascular malformations, especially with venous developmental anomalies. The size of cavernomas is variable from 1 mm to several centimeters. About 70% of cases are supratentorial and 30% in the posterior fossa, particularly in the brain stem. Macroscopic and histopathological findings are typical and the diagnostic is generally easy.

摘要

中枢神经系统海绵状血管瘤是一种血管畸形,有两种情况:散发性和常染色体显性遗传家族性。病变由扩张、紧密排列的血管组成,其间无脑实质,周围有含铁血黄素和胶质增生,少数情况下有钙化。在80%的散发性病例中,病变是单发的,多发病变罕见(中位数:4个)。在家族性病例中,病变总是多发的。海绵状血管瘤常与其他血管畸形相关,尤其是静脉发育异常。海绵状血管瘤的大小从1毫米到几厘米不等。约70%的病例位于幕上,30%位于后颅窝,尤其是脑干。宏观和组织病理学表现具有典型性,诊断一般容易。

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