Background and importance: Different types of tumor have been reported in the pineal gland, but pleomorphic xanthoastrocytoma (PXA) in this region is extremely rare.
Clinical presentations: A 61-year-old man had gait disturbance and dementia for 1 month. Radiological examination revealed a 22 × 26 × 22-mm-diameter mass in the pineal gland and remarkable hydrocephalus. Biopsy of the tumor was performed and histological examination confirmed diagnosis of PXA with anaplastic features. Radiation therapy with concomitant temozolomide was performed, and tumor reduction was achieved.
Conclusion: We report the first case of PXA with anaplastic features in the pineal gland. This case indicates that temozolomide and radiation therapy are effective for treating PXA with anaplastic features.