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Background: Chordoma is a rare malignant tumor that accounts for 1-4% of primary malignant bone tumors arising primarily in the axial skeleton and primarily affects adults. It seems to have difficulty with diagnosis and treatment.

Case description: A 63-year-old man with a history of diabetes treated with oral antidiabetic medication and hypertension. Presented with two years of bilateral decrease in visual acuity. Neurological examination revealed left ptosis, divergent strabismus, and higher cerebral function disorders. Brain MRI revealed a sellar and suprasellar lesion evokes a sellar meningioma, macro adenoma. Hormonal laboratory tests revealed that the prolactin level was elevated to 140ng/ml without any other hormonal disorder. The patient underwent partial surgical resection of the lesion through endoscopic endonasal. Histopathological examination revealed a chondroid chordoma.

Postoperatively the patient had no endocrinological or neurological complications, but he kept the starbism and the ptosis in the left eye.

Conclusion: In the context of progressive ophthalmological signs by a sellar and suprasellar lesion, the differential diagnosis should include chordoma along with meningioma, macro adenoma and metastasis.

Complete and early surgical removal is the first line treatment.

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