title: Neurocutaneous melanomatosis with a rapidly deteriorating course.
abstract: 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.
authors.affiliation: 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.
authors.forename: Winnie C W
authors.initials: WC
authors.lastname: Chu
authors.forename: Vincent
authors.initials: V
authors.lastname: Lee
authors.forename: Yu-leung
authors.initials: YL
authors.lastname: Chan
authors.forename: Matthew M K
authors.initials: MM
authors.lastname: Shing
authors.forename: Ki-wai
authors.initials: KW
authors.lastname: Chik
authors.forename: Chi-kong
authors.initials: CK
authors.lastname: Li
authors.forename: Kwok-chiu
authors.initials: KC
authors.lastname: Ma
mesh_terms: 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