Background: Sellar and suprasellar primary melanocytic tumors are exceptional occurrences. Besides the difficulty of differential diagnosis between a primary and secondary lesion, treatment of these pathologies is still unclear and controversial.
Case Report: We describe the case of a 36-year-old woman with no relevant previous medical history who presented with 1 month history of diabetes insipidus, blurred vision and generalized weakness; a brain MRI disclosed an atypical pituitary stalk lesion; initially the tumor was biopsied through an endonasal endoscopic approach that revealed a melanocytic tumor; the patient was afterwards managed by a second stage extended endonasal endoscopic approach achieving a subtotal tumor removal. The overall survival was of 14 months due to the multidisciplinary management including surgery, radio and chemotherapy.
Conclusion: If a biopsy is essential to deal with these invasive lesions, treatment including surgical resection should be part of a multidisciplinary approach.
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