Neurocutaneous melanomatosis with a rapidly deteriorating course.
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.
Chu
Winnie C W
WC
Department of Diagnostic Radiology and Organ Imaging, The Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, Hong Kong, China.
Lee
Vincent
V
Chan
Yu-leung
YL
Shing
Matthew M K
MM
Chik
Ki-wai
KW
Li
Chi-kong
CK
Ma
Kwok-chiu
KC
D002675:Child, Preschool; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008545:Melanoma / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008548:Melanosis / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008577:Meningeal Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D008578:Meninges / Q000473:pathology; D008581:Meningitis / Q000175:diagnosis / Q000473:pathology; D020752:Neurocutaneous Syndromes / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D009508:Nevus, Pigmented / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D013226:Status Epilepticus / Q000175:diagnosis / Q000473:pathology; D013702:Temporal Lobe / Q000473:pathology