Atypical teratoid/rhabdoid tumor in adult: case series and an integrated survival analysis.

ABSTRACT

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, p = 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 χ2 = 14.662, p = 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.