By Gary M. White, MD
Neurocutaneous melanosis (NCM) is the combination of large or multiple (3 or more) congenital melanocytic nevi in association with meningeal melanosis or melanoma.
One study however, found NCM more likely in patients with skin changes on the anterior part of the body [Clinical Imaging 2014;38;79].
In one study of NCM [Pediatr Dev Pathol. 2015 Jan-Feb;18:1-9], NRAS Q61 mutation was found in 12/16 (75.0%), and BRAF V600E in 2/16 (12.5%).
In the perfect world, all patients with LCMN would have a screening head MRI although, as of this writing, there is no specific treatment for CNS melanosis. Certainly, any child with symptoms, e.g. seizures, hydrocephalus should undergo such an exam. Cranial ultrasound has been used as a screening tool as well. Workup and treatment should be done by multiple specialists.
A baby girl was born with a large pigmented nevus of the body and multiple satellite lesions on the skull, extremities and body. Although the patient was asymptomatic, cranial ultrasound was performed and showed multiple echogenic intra-parenchymal lesions with smooth margins. MRI confirmed neural melanosis. [Iran J Radiol. 2014 Dec; 11(4): e10107].
A 30-year-old woman presented with impaired vision, dizziness, and headache. Physical examination revealed many brownish-black nevi on the trunk, including a giant one covering nearly the entire right thoracodorsal region and another on the left thigh. MRI of the brain revealed a large expansive lesion in the left frontal region. Surgery revealed melanoma. [Case Rep Med. 2015; 2015: 545603]
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