| Summary: | <p>Abstract</p> <p>Introduction</p> <p>Primary malignant melanoma of the pineal region is exceedingly rare. We report a case of primary pineal malignant melanoma and review the literature.</p> <p>Case presentation</p> <p>Our patient was a 22-year-old Iranian woman without any significant past medical history, who was referred to our center with a four-week history of headache and gait disturbance. A magnetic resonance imaging study showed a solid mass in the pineal region causing obstructive hydrocephalus. A brain biopsy was performed and the histological examination indicated melanoma. No other additional melanocytic lesions were found elsewhere. Our patient underwent gross total resection. At the time of discharge she had fully recovered without any neurological deficits. Three weeks after discharge, she was readmitted to hospital with the diagnosis of distal deep vein thrombosis and pulmonary embolism; 12 weeks after the onset of her illness she died of cardiopulmonary arrest.</p> <p>Conclusion</p> <p>We have presented here a rare tumor, a primary malignant melanoma of the pineal region. To the best of our knowledge, this is the second-youngest patient with such a tumor reported in the literature.</p>
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