非典型表现的神经纤维瘤病  

Neurofibromatosis of atypical presentation

作　　者：Tato B.P. Sáez A.C. Recuero J.L.D. 吴佳纹 

机构地区：[1]C/Suero de Quiones 4, Madrid 28002, Spain

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第3期16-16,共1页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Neurofibromatosis (NF) is considered to be a heterogeneous neuroectodermal disease clinically denned by the presence of neurofibromas, multiple caf′ e-au-lait spots, intertriginous freckles and Lisch nodules. Mosaicism explains atypical presentations of the disease. Early mutations, before tissue differentiation, give rise to generalized disease. We report an atypical presentation of neurofibromatosis with an unusual distribution of neurofibromas, a peculiar, clinically and pathologically, neurofibroma on the trunk and the association with an ovarian serous cystoadenofibroma.Neurofibromatosis （NF） is considered to be a heterogeneous neuroectodermal disease clinically denned by the presence of neurofibromas, multiple caf＇e-au-lait spots, intertriginous freckles and Lisch nodules. Mosaicism explains atypical presentations of the disease. Early mutations, before tissue differentiation, give rise to generalized disease. We report an atypical presentation of neurofibromatosis with an unusual distribution of neurofibromas, a peculiar, clinically and pathologically, neurofibroma on the trunk and the association with an ovarian serous cystoadenofibroma.

关 键 词：神经纤维瘤病 非典型表现 囊性腺纤维瘤 临床表现 神经外胚层 卵巢浆液性 嵌合现象 组织分化 咖啡斑 多发性 

分 类 号：R730.264[医药卫生—肿瘤] R542.22[医药卫生—临床医学]