Background: Atypical teratoid/rhabdoid tumor (AT/RT) is well documented in children but less so in adults because of its rarity.
Method: We report a series of five cases, a literature review and quantitative analysis of the survival outcome of adult AT/RT patients.
Results: Seventy-four patients including our five cases (male: female = 16: 58) were evaluated, whose median age was 32.5 years (18-80 years). The commonest location was the sellar region. Median overall survival (OS) was 12.5 months (0.5-204.00 months). Chemotherapy was associated with OS (HR: 0.349, 95%CI: 0.176-0.694, = 0.003), while other factors did not influence OS. From Kaplan-Meier analysis, surgery combining postoperative chemotherapy and radiotherapy was associated with better prognosis (Log Rank: = 14.662, = 0.001).
Conclusions: Adult AT/RT is commoner in females and tends to recur rapidly after surgical resection. Chemotherapy may provide a survival benefit. Surgery combined with postoperative chemotherapy and radiotherapy was associated with better prognosis for adult AT/RT patients, though the overall prognosis was still poor.
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Atypical teratoid/rhabdoid tumors (AT/RTs) of the central nervous system (CNS) are rare and aggressive, typically occurring in early childhood or infancy, with adult cases being extremely rare. These tumors are associated with the inactivation of the integrase interactor 1 (INI1) gene. The prognosis is poor, worsening significantly if metastasis is detected at diagnosis.
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