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Review
. 2013 Nov-Dec;88(6):863-78.
doi: 10.1590/abd1806-4841.20132233.

Giant congenital melanocytic nevus

Ana Carolina Leite Viana  1 , Bernardo Gontijo  2 , Flávia Vasques Bittencourt  3
Affiliations
Review

Giant congenital melanocytic nevus

Ana Carolina Leite Viana et al. An Bras Dermatol. 2013 Nov-Dec.

Erratum in

  • An Bras Dermatol. 2014 Jan-Feb;89(1):190

Abstract
in English, Portuguese

Giant congenital melanocytic nevus is usually defined as a melanocytic lesion present at birth that will reach a diameter ≥ 20 cm in adulthood. Its incidence is estimated in <1:20,000 newborns. Despite its rarity, this lesion is important because it may associate with severe complications such as malignant melanoma, affect the central nervous system (neurocutaneous melanosis), and have major psychosocial impact on the patient and his family due to its unsightly appearance. Giant congenital melanocytic nevus generally presents as a brown lesion, with flat or mammilated surface, well-demarcated borders and hypertrichosis. Congenital melanocytic nevus is primarily a clinical diagnosis. However, congenital nevi are histologically distinguished from acquired nevi mainly by their larger size, the spread of the nevus cells to the deep layers of the skin and by their more varied architecture and morphology. Although giant congenital melanocytic nevus is recognized as a risk factor for the development of melanoma, the precise magnitude of this risk is still controversial. The estimated lifetime risk of developing melanoma varies from 5 to 10%. On account of these uncertainties and the size of the lesions, the management of giant congenital melanocytic nevus needs individualization. Treatment may include surgical and non-surgical procedures, psychological intervention and/or clinical follow-up, with special attention to changes in color, texture or on the surface of the lesion. The only absolute indication for surgery in giant congenital melanocytic nevus is the development of a malignant neoplasm on the lesion.

O nevo melanocítico congênito gigante é, geralmente, definido como lesão melanocítica presente ao nascimento e que atinge, no mínimo, 20 cm de diâmetro na vida adulta. Sua incidência é estimada em menos de 1:20.000 recém-nascidos. Contudo, apesar de sua raridade, possui importância tanto por estar associado a complicações graves, como o melanoma maligno e o acometimento do sistema nervoso central (melanose neurocutânea), quanto pelo grande impacto psicossocial que ocasiona no paciente e nos familiares, devido a seu aspecto comumente inestético. O nevo congênito gigante, geralmente, apresenta-se como lesão acastanhada, plana ou elevada, de bordas bem definidas e com hipertricose, e seu diagnóstico é eminentemente clínico. Do ponto de vista histológico, porém, os nevos melanocíticos congênitos são diferenciados dos nevos adquiridos, principalmente pelo seu tamanho maior, pela disseminação das células névicas para as camadas mais profundas da pele e pela sua arquitetura e morfologia mais variadas. O nevo congênito gigante é considerado fator de risco para o desenvolvimento do melanoma. Todavia, a real incidência de malignização ainda é controversa. Estima-se que o risco de melanoma ao longo da vida esteja entre 5 e 10%. Diante dessas incertezas e do tamanho das lesões, a abordagem do nevo gigante representa um desafio e deve ser individualizada. O tratamento pode incluir procedimentos cirúrgicos ou não cirúrgicos, intervenções psicológicas e/ou acompanhamento clínico, com atenção a mudanças de coloração, superfície ou textura do nevo. Considera-se que a única indicação absoluta para a intervenção cirúrgica é o surgimento de uma neoplasia maligna sobre a lesão.

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Conflict of interest statement

Conflict of Interest: None.

Figures

FIGURE 1
FIGURE 1
Giant congenital melanocytic nevus in "bathing trunk" with evident hypertrichosis, irregular surface and nodular areas
FIGURE 2
FIGURE 2
A. Giant congenital melanocytic nevi "in garment": A) "in bathing trunk"; (B) "in coat sleeve"; (C) type "shirt"
FIGURE 3
FIGURE 3
Child with multiple satellite lesions associated to giant nevus, affecting even the oral mucosa (A) and plantar areas (B)
FIGURE 4
FIGURE 4
Size of giant congenital melanocytic nevus located on the head, according to the age
FIGURE 5
FIGURE 5
Size of giant congenital melanocytic nevus located on the body, according to the age
FIGURE 6
FIGURE 6
Giant congenital melanocytic nevus "in bathing trunk" submitted to partial surgical removal
See this image and copyright information in PMC

References

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