69例1型神经纤维瘤病患者的恶性周围神经鞘瘤相关症状的回顾性研究
Symptoms associated with malignancy of peripheral nerve sheath tumours:A retrospective study of 69 patients with neurofibromatosis 1作者机构：Department of Dermatology Paris XII University Henri-Mondor Hospital F-94010 Créteil Cedex France
出 版 物：《世界核心医学期刊文摘（皮肤病学分册）》 (Digest of the World Core Medical JOurnals:Dermatology)
年 卷 期：2005年第1卷第10期
页 面：26-27页
学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学]
主 题：神经纤维瘤病 丛状神经纤维瘤 神经鞘瘤 组织学检查 手术活检 预测值 死亡原因 临床因素 神经功能障碍 多变量分析
摘 要：Background:Neurofibromatosis 1 (NF1) is a common genetic disorder with variable clinical manifestations and an unpredictable course. Plexiform neurofibromas are common complications of NF1. Their malignant transformation is the main cause of mortality in adult patients with NF1. Objectives:To identify clinical factors associated with malignant transformation of plexiform neurofibromas. Methods:Using the database of our neurofibromatosis clinic we included in a retrospective study all patients with NF1 having at least one peripheral nerve sheath tumour for which they underwent surgery or surgical biopsy. Predictive values for malignant transformation of three clinical symptoms, i.e. pain, enlargement of mass and neurological symptoms, were evaluated in association with histological parameters. Results:Of 69 patients studied, 48 had at least one plexiform neurofibroma and 21 had a malignant peripheral nerve sheath tumour. Only enlargement of the tumour had high negative and positive predictive values for malignant transformation:0.92 and 0.95, respectively. In multivariate analysis, tumour enlargement was independently associated with malignant transformation (OR 167.8,95%CI 14.0～2012.1). Conclusions:From a practical point of view, pain, neurological deficit and enlargement of a preexisting peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation.
维普期刊数据库
同方期刊数据库