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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 24:287-290, February 2003
© 2003 American Society of Neuroradiology

Case Report
PEDIATRICS

Neurocutaneous Melanomatosis with a Rapidly Deteriorating Course

Winnie C.W. Chu^a, Vincent Lee^b, Yu-leung Chan^a, Matthew MK Shing^b, Ki-wai Chik^b, Chi-kong Li^b and Kwok-chiu Ma^b

^a Department of Diagnostic Radiology and Organ Imaging, The Chinese University of Hong Kong, Prince of Wales Hospital, China
^b Department of Pediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, China

Address reprint requests to Dr W.C.W. Chu, Assistant Professor, Department of Diagnostic Radiology and Organ Imaging, The Chinese University of Hong Kong, Prince of Wales Hospital, 30–32 Ngan Shing St, Shatin, Hong Kong, China

Summary: Neurocutaneous melanosis is a rare 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 present serial MR imaging findings in the brain and spine of a child with congenital giant hairy nevi who developed progressive leptomeningeal melanomatosis and whose neurologic condition rapidly deteriorated.

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