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...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.展开更多
2016版《世界卫生组织（World Health Organization,WHO）中枢神经系统肿瘤分类》[1]将黑色素细胞肿瘤分为：脑膜黑色素细胞增多症、脑膜黑色素细胞瘤、脑膜黑色素瘤及脑膜黑色素瘤病。其中,脑膜黑色素细胞增多症定义为良性肿瘤,脑膜黑...2016版《世界卫生组织（World Health Organization,WHO）中枢神经系统肿瘤分类》[1]将黑色素细胞肿瘤分为：脑膜黑色素细胞增多症、脑膜黑色素细胞瘤、脑膜黑色素瘤及脑膜黑色素瘤病。其中,脑膜黑色素细胞增多症定义为良性肿瘤,脑膜黑色素细胞瘤为交界性肿瘤,脑膜黑色素瘤及脑膜黑色素瘤病为恶性肿瘤。展开更多
文摘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.
文摘2016版《世界卫生组织（World Health Organization,WHO）中枢神经系统肿瘤分类》[1]将黑色素细胞肿瘤分为：脑膜黑色素细胞增多症、脑膜黑色素细胞瘤、脑膜黑色素瘤及脑膜黑色素瘤病。其中,脑膜黑色素细胞增多症定义为良性肿瘤,脑膜黑色素细胞瘤为交界性肿瘤,脑膜黑色素瘤及脑膜黑色素瘤病为恶性肿瘤。
