[Neurocutaneous melanosis]

Abstract:

:OBJECTIVE: Describe the morbidity associated with a rare disease due to an embryological defect. METHODS: Retrospective revision of medical and necropsy reports. Bibliographic research using MEDLINE, LILACS and Index Medicus databases. RESULTS: 1 year-old male patient, admitted with generalized tonic-clonic seizures, evolving to deep coma and death in a few hours. Necropsy showed diffuse leptomeningeal malignant melanoma in brain stem, cerebellum, spinal cord and temporal lobe associated with a giant melanocytic nevus and satellite lesions. CONCLUSIONS: Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large and/or multiple melanocytic nevi and pigmented tumors of the leptomeninges. It has a poor prognosis as demonstrated by the present report. It's physiopathology is believed to be due to a migration defect of the cells arising from the primitive neural crest. In these cases, an early diagnosis may improve the survival time.

journal_name

J Pediatr (Rio J)

journal_title

Jornal de pediatria

authors

Noronha L,Sampaio G,Netto MR,Reis-Filho JS,Faoro LN,Raskin S,Bleggi-Torres LF

doi

10.2223/jped.320

keywords:

subject

Has Abstract

pub_date

1999-07-01 00:00:00

pages

277-80

issue

4

eissn

0021-7557

issn

1678-4782

journal_volume

75

pub_type

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