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[脑脂肪性错构瘤——蛛网膜下腔畸形]

[Lipomatous hamartomas of the brain--malformations of the subarachnoid space].

作者信息

Zámecník J, Kyncl M

机构信息

Ustav patologické anatomie 2. LF UK a FN v Motole, Praha.

出版信息

Cesk Patol. 2001 Nov;37(4):163-7.

Abstract

Lipomatous hamartomas are rare disorders affecting the central nervous system. In our report, two observations of this disorder are presented. Both are interhemispheric in location and are associated with a complete agenesis of the corpus callosum, while having different histological structures. In our first patient, the intracranial formation caused refractory seizures, was partially surgically removed, and a biopsy was performed. Light microscopic examination disclosed the presence of a highly vascularized mature adipose tissue with numerous calcifications. The second case was an incidental finding at autopsy. Microscopically, we found adipose tissue together with numerous foci of hemopoiesis and structures of lamelar bone. In both cases, the indistinct demarcation of the collagenous capsule from the surrounding brain tissue and the continuity of the hamartoma with the leptomeninges were striking. In recent findings about the development of meninges and brain commissures, the origin of this disorder is explained as a defective resorption of the embryonic meninx primitiva. This disorder then causes other developmental aberrations of the brain, which are often found in association. The varying microscopic pattern of these disorders can also be satisfactorily explained by their origin in the primitive meninx, which is formed from both mesenchyme and neuroectoderm.

摘要

脂肪性错构瘤是一种影响中枢神经系统的罕见疾病。在我们的报告中,展示了该疾病的两例观察病例。两例均位于大脑半球间,且与胼胝体完全缺如相关,不过组织学结构不同。在我们的首例患者中,颅内肿物引发难治性癫痫,部分肿物经手术切除并进行了活检。光镜检查显示存在高度血管化的成熟脂肪组织,伴有大量钙化。第二例是尸检时偶然发现的。镜下,我们发现脂肪组织伴有众多造血灶和板层骨结构。在这两例中,胶原性包膜与周围脑组织界限不清以及错构瘤与软脑膜连续这两点都很显著。在关于脑膜和脑连合发育的最新研究结果中,该疾病的起源被解释为原始脑膜吸收缺陷。这种疾病继而导致大脑的其他发育异常,这些异常往往同时出现。这些疾病不同的微观模式也可以通过它们起源于由间充质和神经外胚层形成的原始脑膜而得到满意解释。

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