NEUROFIBROMATOSIS OF CHOROID
NEUROFIBROMATOSIS OF CHOROID
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DOI：
10.1016/0002-9394(62)93279-8
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发表时间：
1962-01-01
影响因子：
4.2
通讯作者：
GONZALES.R
中科院分区：
文献类型：
--
作者：
WOLTER, JR;MANKIN, WJ;GONZALES.R
神经纤维瘤病（von Recklinghausen病）可累及眼眶，其临床表现多种多样。其肿瘤可为弥漫性或局限性，多发或单发。它们可能代表丛状神经瘤，神经节神经瘤，弥漫性神经纤维瘤，视神经或视网膜胶质瘤，面部半肥厚，直至纤维瘤。然而，所有这些都被认为是在先天性异常的基础上发展起来的，与周围神经或脑神经有关，并且来自雪旺细胞。大脑、视神经和视网膜的肿瘤是个例外，Duke-Elder1写道：“现在人们普遍认为，神经纤维瘤代表神经外胚层组织的发育缺陷，通常是家族性的。”通常皮肤上的色素斑（cafC-aulait斑）是疾病的一部分。神经纤维瘤病可能终生保持静止，也可能表现出进行性生长。纤维肉瘤的发展从这类肿瘤的中胚层成分已被报道，但是例外。真实的本性
Neurofibromatosis(von Recklinghausen’s disease) may involve the orbital region in a great diversity of clinical appearances. Its tumors may be diffuse or localized, multiple or solitary. They may represent changes ranging from plexiforme neuroma, ganglion neuroma, diffuse neurofibroma, optic nerve or retinal glioma, facial hemihypertrophy, up to molluscum fibrosum. However, all of these are believed to develop on the basis of a congenital anomaly, are associated with peripheral or cranial nerves and are derived from the cells of Schwann. The tumors of the brain, optic nerve and retina are an exception, Duke-Elder1 writes:“It is now generally accepted that neurofibroma represents a developmental defect of the neuroectodermal tissues which often is familial.” Usually pigment patches in the skin (cafC-aulait spots) are part of the disease. Neurofibromatosis may remain stationary through life or may show progressive growth. Development of fibrosarcoma from the mesodermal component of such tumors has been reported, but is exceptional. The true nature