巨大或多发性先天性黑色素细胞痣:1008例患者中神经皮肤黑色素细胞增多症的发生率  

Large or multiple congenital melanocytic nevi: Occurrence of neurocutaneous mela-nocytosis in 1008 persons

作　　者：Bett B.J. 朱国兴 

机构地区：[1]Nevus Network,West Salem, Ohio, USA Dr.

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第8期43-44,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic nevi (MCMN). Objective: The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods: Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results: Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations: Attending physician confirmation of submitted information was unavailable. Conclusions: LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths. In contrast, most of the rare persons (N = 17) with MCMN developed symptomatic NCM, and more than a third died.Background： There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis （NCM） in a large cohort of persons with large congenital melanocytic nevi （LMCN） or multiple congenital melanocytic nevi （MCMN） . Objective： The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods： Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results： Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations： Attending physician confirmation of submitted information was unavailable. Conclusions： LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths.

关 键 词：黑色素细胞痣 皮肤黑色素瘤 细胞增多症 先天性 发生率 多发性 患者 神经 

分 类 号：R739.5[医药卫生—肿瘤] R259.573[医药卫生—临床医学]