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
Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was de...Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was designed to see if neurofibromas in patients with neurofibromatosis, type 1 (NF-1) differ from sporadic neurofibromas (SN) in their incidence of associated melanocytic nevi and other histologic features. Slides from 114 NF-1 were compared with 112 SN and 300 intradermal melanocytic nevi (IDN). Results: Small lentiginous melanocytic nevi were identified over 13 NF-1 (11%) but no SN (P=0.0002). Compared with other NF-1, NF-1 with nevi were more frequently associated with melanocytic hyperplasia, giantmelanosomes and diffuse neurofibroma (P < 0.03). Compared with SN, NF-1 were also more frequently assoc iated with melanocytic hyperplasia, lentigo simplex-like changes, diffuse neuro fibroma and plexiform neurofibroma (P < 0.001). Sebaceous hyperplasia (14%), de rmal elastosis (9%), lipomatous change (8%), epithelial cysts (4%) and kerati n granulomas or folliculitis (3%) were not significantly different in prevalenc e between NF-1, SN and the control group of IDN. Conclusions: This study sugges ts that there is a difference in the potential for melanocytic proliferation in NF-1 compared with SN. NF-1, SN and IDN are associated with a similar range of incidental histologic changes.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Background: Neurofibromatosis, type 1, is associated with cutaneous melanin pigmentation, but an association with ordinary melanocytic nevi has not been described. Methods: This retrospective case-control study was designed to see if neurofibromas in patients with neurofibromatosis, type 1 (NF-1) differ from sporadic neurofibromas (SN) in their incidence of associated melanocytic nevi and other histologic features. Slides from 114 NF-1 were compared with 112 SN and 300 intradermal melanocytic nevi (IDN). Results: Small lentiginous melanocytic nevi were identified over 13 NF-1 (11%) but no SN (P=0.0002). Compared with other NF-1, NF-1 with nevi were more frequently associated with melanocytic hyperplasia, giantmelanosomes and diffuse neurofibroma (P < 0.03). Compared with SN, NF-1 were also more frequently assoc iated with melanocytic hyperplasia, lentigo simplex-like changes, diffuse neuro fibroma and plexiform neurofibroma (P < 0.001). Sebaceous hyperplasia (14%), de rmal elastosis (9%), lipomatous change (8%), epithelial cysts (4%) and kerati n granulomas or folliculitis (3%) were not significantly different in prevalenc e between NF-1, SN and the control group of IDN. Conclusions: This study sugges ts that there is a difference in the potential for melanocytic proliferation in NF-1 compared with SN. NF-1, SN and IDN are associated with a similar range of incidental histologic changes.
