Neurocutaneous Melanosis with Intraspinal Malignant Melanoma in Adults — An Extremely Rare Clinical Entity: A Case Report with Review of Literature

Abstract

Objectives Neurocutaneous melanosis (NCM) in adult patients was rare, and few cases had been reported. In this report, we summarized the features of NCM in adult patients for providing useful information about this rare clinical entity. Methods In this report, we present a case of a 41-year-old woman, who was eventually diagnosed with NCM with intraspinal malignant melanoma. The clinical features, treatments, and prognosis of cases of NCM in adults were thoroughly reviewed and discussed. Results A 41-year-old woman was eventually diagnosed with NCM with intraspinal malignant melanoma. The postoperative course was uneventful and her symptoms significantly improved (VAS: 3 points). However, she developed symptoms of intracranial hypertension and deteriorated rapidly after 11 months of operation, and died 1 month later. To date, only 15 cases of NCM in adults have been reported in the English literature. Analyses showed that the average age was 33.8 years (range 19–65 years), and 11 patients (68.8%) were between 20 and 40 years. A slight male predominance (M:F/11:5) was found. For the 12 cases which detailed follow-up information was available, 9 patients (75%) died, with a survival time ranging from 1 day to 29 months post-operation. Death occurred within the first month of operation in 6 cases (46. 2%). The median survival time was 3 months. Conclusions We presented a rare case of NCM with intraspinal malignant melanoma in an adult patient. Although rare, this clinical entity causes significant mortality and has poor prognosis.