Atypical Teratoid/Rhabdoid Tumors (ATRT): Improved Survival in Children 3 Years of Age and Older With Radiation Therapy and High-Dose Alkylator-Based Chemotherapy

Abstract

Children presenting with ATRT before the age of 3 years have a dismal prognosis. ATRT presenting in older patients can be cured using a combination of radiation and high-dose alkylating therapy. Older patients with relapsed ATRT can have salvage treatment using ICE chemotherapy.

Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis

“…This occurred in the context of 2 retrospective studies that demonstrated an association between adjuvant RT and improved OS. 10,12 We confirmed this modest association between increased survival and RT in a rigorous statistical manner with the use of a landmark analysis to account for immortal time bias, which potentially can limit studies that use observational data. RT was a positive prognostic factor for survival in univariate analysis and remained significant in multivariate models, further supporting a robust association between RT and improved OS in ATRT.…”

“…The benefits of greater tumor resection have not been consistent in other studies, suggesting that resection alone is not sufficient for long-term survivorship in patients with ATRT and establishing the need for adequate adjuvant therapy. 9,10,12,14 Data describing chemotherapeutic regimens were not available for analysis; however, chemotherapy often is administered in the initial postoperative period to patients with ATRT. Because most children aged 3 years receive chemotherapy as a way of postponing RT, chemotherapeutic strategies for ATRT have been among the best studied issues in ATRT management.…”

“…Nevertheless, multiagent chemotherapy containing anthracyclines and alkylating agents in an approach similar to that used to treat rhabdomyosarcoma has been efficacious in the treatment of ATRT. 12,[20][21][22][23] Intrathecal chemotherapy is associated with prolonged survival, and its role in treatment continues to be investigated. 11,14 There is also evolving evidence that dose-intensive chemotherapy and autologous hematopoietic stem cell rescue, particularly with the use of high-dose methotrexate, may be of benefit in young children with ATRT.…”

“…11 Although the use of RT has been controversial given the associated risk of adverse affects on neurocognitive development of young children, there is emerging nonrandomized evidence that RT may be important for the achievement of long-term survival in patients with ATRT. 5,[10][11][12][13] Currently, there is no standard therapeutic regimen for ATRT outside of clinical trials. Given the rarity of ATRT, large retrospective analyses are important sources of information to guide the development of treatment strategies.…”

“…Given the rarity of ATRT, large retrospective analyses are important sources of information to guide the development of treatment strategies. 1,9,10,[12][13][14][15] The objectives of the current study were to identify clinical and prognostic factors for ATRT and to determine whether the use of RT has changed in the last decade in light of information suggesting its importance in the management of ATRT using the National Cancer Institute (NCI) Surveillance, Epidemiology, and End Results (SEER) database. The SEER Program collects cancer survival and incidence information from population-based cancer registries that encompass approximately 28% of the US population, which provides an opportunity to study rare entities like ATRT.…”