Descriptive epidemiology and prognostic factors of atypical teratoid/rhabdoid tumors in the United States, 2001-2021.

Atypical teratoid rhabdoid tumor (AT/RT) is a rare embryonal central nervous system tumor with a dismal prognosis that occurs mostly in early childhood. Since recent epidemiological and prognostic information is limited, we aimed to describe and analyze AT/RT-related incidences, temporal trends and prognostic factors. Incidence and survival data between 2001 and 2021 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Descriptive epidemiology was utilized to assess incidences and rate ratios. Temporal trends were assessed by Joinpoint analysis. Prognostic factors were evaluated by survival analysis. A total of 393 patients were included in our analysis. Most patients developed tumors at less than 1 year of age (33.6%). The overall incidence was 0.084 per 100,000 population. According to the SEER 17 data, AT/RT represented 18.3% of all CNS malignant tumors diagnosed in infants aged < 1 year, with a relatively high incidence of 0.583. The overall incidence trend increased nonsignificantly from 2004 to 2021 (P = 0.572). Older age, more aggressive tumor resection, radiotherapy and chemotherapy administration, and nonmetastasis status were related to improved overall survival (OS) and cancer-specific survival (CSS) via Kaplan-Meier analysis. Multivariable Cox regression analysis indicated that age, extent of resection, radiotherapy, chemotherapy and metastasis status were independent prognostic factors for OS and CSS. AT/RT, particularly in infants under 1 year of age, should receive increased attention from epidemiologists and clinicians. An aggressive multimodal regimen including surgery combined with chemotherapy and radiotherapy is necessary.