Neurocutaneous melanosis in an adult patient with intracranial primary malignant melanoma: case report and review of the literature.

Neurocutaneous melanosis in an adult patient with intracranial primary malignant melanoma: case report and review of the literature.

World Neurosurg. 2018 Mar 09;:

Authors: Ma M, Ding ZL, Cheng ZQ, Wu G, Tang XY, Deng P, Wu JD

Abstract
OBJECTIVE: To explore the clinical characteristics of neurocutaneous melanosis (NCM) in adult patients in order to help improve diagnosis and treatment of this disease.
METHODS: We present a rare case of an adult patient suffering from neurocutaneous melanosis with malignant melanoma as well as a review Chinese and English literature, and analyze their clinical features.
RESULTS: There were thirty adult NCM patients, aged 19 to 65 years old, average 27.9 years old, twenty males (66.7%), ten females (33.3%); Twenty-four cases of malignant melanoma (80.0%), three cases of melanocytoma (10.0%), two cases of diffuse melanocytosis (6.7%), and one case pathology unknown (3.3%). Twenty-five cases showed satellite nevi (83.3%), five cases unknown (16.7%). Twenty-eight cases had intracranial lesions (93.3%) and two cases had intraspinal lesions (6.7%). Four cases combined hydrocephalus (13.3%), and two cases combined Dandy-Walker deformity (6.7%).
CONCLUSION: NCM is a rare disease, especially in adults. With the onset symptoms, the diagnosis is generally confirmed. For children with congenital giant nevus, there should be regular periodic surveys of the central nervous system (brain and spinal cord) MRI or cerebrospinal fluid examination to diagnosis. Active treatment should be undertaken to improve the prognosis.

PMID: 29530698 [PubMed - as supplied by publisher]

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