Cerebral mass due to neurocutaneous melanosis: Eight years later

Citation
Jv. Schaffer et al., Cerebral mass due to neurocutaneous melanosis: Eight years later, PEDIAT DERM, 18(5), 2001, pp. 369-377
Citations number
75
Categorie Soggetti
Pediatrics
Journal title
PEDIATRIC DERMATOLOGY
ISSN journal
07368046 → ACNP
Volume
18
Issue
5
Year of publication
2001
Pages
369 - 377
Database
ISI
SICI code
0736-8046(200109/10)18:5<369:CMDTNM>2.0.ZU;2-J
Abstract
Neurocutaneous melanosis (NCM) is associated most commonly with giant conge nital melanocytic nevi (CMN), in particular those on the scalp or In a post erior axial location that are accompanied by satellite congenital nevi. It also can occur in patients with multiple medium-sized CMN. In general, the prognosis of those with symptomatic NCM is poor, even in the absence of mal ignancy, while the prognosis of those with asymptomatic NCM detected via sc reening varies and is more difficult to predict. Herein we report an asympt omatic patient with a giant CMN and multiple satellite nevi who had a scree ning magnetic resonance Imaging (MRI) study at age 5 months that showed a r ounded area of increased signal In the right temporal lobe on T1-weighted i mages, suggestive of parenchymal melanosis. This melanotic mass was resecte d at age 10 months, and histologic examination of the surgical specimen sho wed prominent perivascular collections of benign, pigment-containing melano cytes within cerebral tissue. The patient remains healthy 8 years later. Hi s excellent long-term outcome and other reports of NCM with localized centr al nervous system (CNS) involvement apparent on MRI may have implications f or management, including early imaging of patients with high-risk CMN and p otential surgical intervention for NCM.