A Case of Neurocutaneous Melanosis in a Child with Congenital Giant Nevi.

Kim,  Dong Hyun; Kwon,  Young Se; Kim,  Soon Ki; Son,  Byong Kwan; Park,  In Suh; Kim,  Eun Young; Lee,  Choong Jae

Case Report

Journal of the Korean Child Neurology Society 2005;13(2):271-275.
Published online November 30, 2005.

A Case of Neurocutaneous Melanosis in a Child with Congenital Giant Nevi.

Dong Hyun Kim, Young Se Kwon, Soon Ki Kim, Byong Kwan Son, In Suh Park, Eun Young Kim, Choong Jae Lee

¹Department of Pediatrics, College of Medicine, Inha University, Incheon, Korea.
²Department of Pathology, College of Medicine, Inha University, Incheon, Korea.
³Department of Neurosurgery, College of Medicine, Inha University, Incheon, Korea.
⁴Department of Plastic surgery, College of Medicine, Inha University, Incheon, Korea.

Abstract

Patients with giant congenital melanocytic nevi may grow up to have melanotic tumors characterized by central nervous system involvement, termed neucutaneous melanosis. Neurocutaneous melanosis is a rare but a significant congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We experienced a case of a neurocutaneous melanosis with congenital giant hairy nevi, who grew up to have progressive leptomeningeal melanomatosis and whose neurologic conditions were rapidly deteriorated to death. So we report the case with a brief review of related literature.

Key Words: Congenital giant nevi, Neurocutaneous melanosis, Malignant melanoma