Mathkour, Mansour; Carsky, Katie; Chabot, A. Bert; Werner, Cassidy; Berry, John F.; Carr, Christopher; Lockwood, Joseph D.; Keen, Joseph R.; Bui, Cuong J.; Biro, Erin E.
BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant central nervous system neoplasm classified as an embryonal grade IV neoplasm by the World Health Organization. ATRT generally occurs in children younger than 3 years, with 85 pathologically confirmed cases reported in adults. It is most commonly supratentorial, with only 9 confirmed adult cases localized to the pineal region.; CASE DESCRIPTION: The case is described of a 29-year-old man with a history of chronic migraines and a previously negative computed tomography scan of the head presenting with worsening headaches and new-onset diplopia with upward gaze palsy. Computed tomography and magnetic resonance imaging showed a hemorrhagic pineal mass with extension into the right thalamus. After resection, the immunohistochemical staining and cytogenetic profile proved consistent with ATRT, making it the ninth reported case of pineal ATRT in an adult, which was treated aggressively with good outcome.; CONCLUSIONS: Adult ATRT is rare, especially in the pineal region, with only 9 cases reported. Because of the aggressiveness, ATRT must be considered in the differential diagnosis of pineal region lesions because early diagnosis and aggressive treatment are key to prolonged survival.