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J Pediatr (Rio J). 1999 Jul-Aug;75(4):277-80.

[Neurocutaneous melanosis]

[Article in Portuguese]

Author information

1
Universidade Federal do ParanĂ¡ (UFPR), Curitiba, PR, Brazil.

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.

PMID:
14685530
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