RESUMO
INTRODUCTION AND IMPORTANCE: Primary central nervous system (CNS) melanoma is a rare entity. Primary CNS malignant melanomas account for 1 % of melanomas and 0.07 % of intracranial tumours. These are highly aggressive and are associated with poor prognosis. Herein, we have discussed one such rare case of PIMM. CASE PRESENTATION: 62-year-old man with primary CNS melanoma underwent craniotomy and resection of left temporal lesion. Postoperative MRI showed no evidence of residual disease. He received 28 fractions of radiation. Follow-up MRI showed no evidence of disease. However, he later developed worsening symptoms and repeat imaging revealed disease progression with hydrocephalus and drop metastasis to spine. He underwent VP shunting and was started on Temozolomide. He progressively declined functionally and eventually died from his disease. CLINICAL DISCUSSION: Primary CNS melanoma is characterized by its rarity, challenging diagnosis, and aggressive behaviour. Current literature suggests limited treatment options, which depend on complete resection of the primary tumour. Molecular analysis may play a key role in deciding future treatment options, including immune checkpoint inhibitors and targeted therapies targeting the BRAFV600E mutation. CONCLUSION: Primary intracranial malignant melanoma (PIMM) is an extremely rare tumour of CNS, and its treatment paradigm is very limited based on available literature. Currently any long-term survival depends on the complete resection of tumour. Our case is unique as it talks about the limited therapeutic options in case of rapidly declining performance status in a resource constraint setting.