Vascular changes in cutaneous neurofibromas.

Neurofibromatosis Pub Date : 1988-01-01
F Teixeira, A Martinez-Palomo, V M Riccardi, J Fernandez-Diez
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Abstract

A variety of vascular changes has been associated with neurofibromatosis, and morphological alterations of arteries have been described previously. However, little attention has been paid to structural modifications of the microvasculature in neurofibromas themselves. Small vessels of 10 cutaneous neurofibromas, excised from patients with neurofibromatosis, were studied by using transmission electron microscopy and compared with normal skin vessels. The major alterations were: (1) increased thickness of the cytoplasm of endothelial cells which send numerous long processes to the lumen and, in some instances, obliterate it; (2) larger numbers of endothelial cell processes on the abluminal surface which are apposed to pericyte processes; (3) increased amounts of filaments and free ribosomes in the cytoplasm of endothelial cells, and (4) abundant pericytes with bundles of cytoplasmic filaments and many small subplasmalemmal densities. Mitotic figures were not seen in any of the vascular elements. These changes were interpreted to be the result of a generalized malformative process of blood vessels in neurofibromas rather than a consequence of the action of a tumor angiogenesis factor.

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皮肤神经纤维瘤的血管改变。
多种血管改变与神经纤维瘤病有关,以前也曾报道过动脉的形态改变。然而,很少有人注意到神经纤维瘤本身的微血管结构改变。本文用透射电镜对10例神经纤维瘤患者皮肤小血管进行了观察,并与正常皮肤血管进行了比较。主要的改变是:(1)内皮细胞的细胞质厚度增加,将许多长过程输送到管腔,在某些情况下,使管腔消失;(2)腹腔表面内皮细胞突起较多,与周细胞突起相对;(3)内皮细胞细胞质中纤维和游离核糖体数量增加;(4)周细胞丰富,胞质纤维成束,质下密度小。在任何维管元件中均未见有丝分裂象。这些变化被解释为神经纤维瘤中血管普遍畸形过程的结果,而不是肿瘤血管生成因子作用的结果。
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