A prospective study of congenital melanocytic naevi: progress report and evaluation after 6 years

Abstract

A prospective study of children with large congenital melanocytic naevi (CMNs) is in progress. Its aims are to observe the natural history and inheritance of CMNs, to record associated pathologies, and eventually to evaluate the effects of treatment. Information is obtained by patient-completed questionnaires and clinical assessment. One hundred and thirty-three children have been followed for a total of 452 patient years. The female/male ratio is 3:2. The characteristics, size and distribution at birth of the CMNs in this group of patients, and their development and associated abnormalities, are documented. After 5 years, affected children were compared with a matched cohort of normal children. Although the prevalence of neurological and developmental defects was higher in affected children, no malignant melanomas have been reported to date. The families of affected children had higher numbers of naevi and café-au-lait spots than those of normal children, suggesting a possible hereditary component. The mothers of affected children also reported an increased incidence of influenza-like illness and of severe nausea and vomiting in pregnancy. This study will continue for a further 4 years, in the first instance, after which we will seek relationships between characteristics of the naevi and outcome.

c-Kit-Targeting Immunotherapy for Hereditary Melanoma in a Mouse Model

“…Human congenital melanocytic nevus, which is present from birth, is linked to hereditary hyper melanocyte stimulation, a trait frequently giving rise to melanoma during aging. It has been reported that families of affected children had higher numbers of nevi and cafe-au-lait spots than did those of normal children, suggesting a possible hereditary component (3). Little progress has been made toward the establishment of effective therapy for melanoma with a hereditary load, despite recent progress in study on melanoma in general (4).…”

Estudo clínico dos nevos melanocíticos congênitos na criança e no adolescente

“…Poucos utilizaram a metodologia desta investigação, isto é, incluindo NMCs gigantes, grandes, médios e pequenos. 11,13,[15][16][17][18] Alguns autores discutiram risco de malignidade e conduta dos NMCs baseando-se em casuísticas exclusivas de NMCs grandes/gigantes. As controvérsias na classificação dos NMCs não sanadas ao longo do tempo e a informação sobre a época de apresentação (principalmente do NMC pequeno) são tropeços na abordagem de tais lesões.…”

“…Na literatura, entre 133 NMCs de diferentes tamanhos, relata-se uma relação 3:2 em relação a sexo feminino/masculino, sendo a maioria das crianças caucasianas e apenas um negro. 15 Para outros autores, 16,18 nenhuma diferença de significância foi observada em relação ao sexo. Em acompanhamento de 46…”

“…19 Quanto ao tempo de seguimento ambulatorial, nosso estudo teve uma média de 5,7 anos, enquanto na literatura são relatados períodos médios compreendidos dentre 3,4 e 23,7 anos. 13,15,16,17 Na presente casuística, dentre os quatro NMCs grandes, um apresentou clareamento parcial e, entre os cinco NMCs gigantes, dois apresentaram clareamento parcial. Um estudo 15 evidenciou clareamento em 17% dos NMCs grandes.…”

“…13,15,16,17 Na presente casuística, dentre os quatro NMCs grandes, um apresentou clareamento parcial e, entre os cinco NMCs gigantes, dois apresentaram clareamento parcial. Um estudo 15 evidenciou clareamento em 17% dos NMCs grandes. Foi publicado um caso de NMC gigante 20 e dois casos de nevos médios 21 com regressão espontânea.…”