Neurocutaneous Melanosis in an Adult Patient with Intracranial Primary Malignant Melanoma: Case Report and Review of the Literature

To explore the clinical characteristics of neurocutaneous melanosis (NCM) in adult patients to help improve diagnosis and treatment of this disease, we present a rare case of an adult patient suffering from NCM with malignant melanoma, as well as a review of the relevant Chinese and English literature. The patient reported here plus the patients identified in our literature review total 30 adults with NCM (20 males [66.7%] and 10 females [33.3%]), age 19–65 years (average, 27.9 years). These include 24 cases of malignant melanoma (80.0%), 3 cases of melanocytoma (10.0%), 2 cases of diffuse melanocytosis (6.7%), and 1 case of unknown pathology (3.3%). Satellite nevi were reported in 25 cases (83.3%) and in 5 cases their presence was unknown (16.7%). Intracranial lesions were present in 28 cases (93.3%), and intraspinal lesions were present in 2 cases (6.7%). There are 4 cases of combined hydrocephalus (13.3%), and 2 cases of combined Dandy–Walker deformity (6.7%). NCM is a rare disease, especially in adults. With the onset of symptoms, the diagnosis is generally confirmed. In children with congenital giant nevus, regular periodic surveys of the central nervous system (brain and spinal cord) with magnetic resonance imaging or cerebrospinal fluid analysis should be performed to diagnose NCM. Active treatment should be undertaken to improve the prognosis. •The neurocutaneous melanosis (NCM) is a rare congenital disease,We through a rare case of an adult patient suffering from Neurocutaneous melanosis with malignant melanoma combined with those reported in Chinese and English literature, a total of 30 cases are analyzed.To explore the clinical characteristics of neurocutaneous melanosis in adult patients can help improve the level of diagnosis and the treatment of this disease.•When the congenital giant nevus in the head, the neck, back and buttocks have a large number of congenital melanoma cell nevus, especially when the melanin cell “satellite nevi” appear, the development of malignant lesions of the risk are larger than those congenital melanoma is limited to the hand-foot or lack of “satellite nevi” of the patients.•The early high mortality rate of child NCM patients, that may result in a paucity of genetic studies for patients and their children. The adult NCM patients who have children provided data samples for the study. The patient we reported was born with congenital giant nevus, but the patient's parents and daughter are both healthy and have no obvi