The clinical features of primary central nervous melanoma are not typical. Treatment standard is uncertain, and surgical resection is usually the first option. Targeted therapy and immunotherapy might be potential choices for primary CNS melanoma. 1 BACKGROUND The incidence of primary central nervous system (CNS) melanoma is approximately 0.01/100,000 per year, accounting for 1% of all melanomas (Balakrishnan et al., 2015; El Habnouni et al., 2018; Hsieh et al., 2015b; Rosenthal et al., 2003c).
