Primary malignant melanoma of the pineal gland is a rare disease entity, with only 24 cases reported in the literature to date.1 Given the rarity of this malignancy, management is primarily based on case reports and literature reviews. We present the first reported case of a patient with a subtotally resected pineal melanoma achieving an excellent clinical outcome using a combination of adjuvant radiotherapy (RT) and pembrolizumab, and provide a thorough review of the literature and discussion of current management strategies.
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