The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.

The mainstay of therapy for atypical teratoid rhabdoid tumor includes:^[1]

• Chemotherapy is given intrathecally for the direct action of the drug on the central nervous system.^[2]
• Various chemotherapeutic drugs used in the treatment of atypical teratoid rhabdoid tumor include cisplatin, cyclophosphamide, vincristine, and etoposide.^[3]
• Approximately 50% of the atypical teratoid rhabdoid tumors will transiently respond, but chemotherapy by itself is rarely curative.

• Radiation is an effective component of therapy for atypical teratoid rhabdoid tumor, but is avoided in patients younger than 3 years of age due to the long term neurocognitive sequelae.^[3]
• The traditional practice for childhood brain tumors has been to use chemotherapy to defer radiation therapy, until a child is older than three years.
• However, the long term outcomes of atypical teratoid rhabdoid tumor are so poor that protocols call for upfront radiation therapy, often in spite of young age.^[4]
• The dose and volume of radiation have not been standardized, however, radiation appears to improve survival.
• External beam (conformal) radiation uses several fields that intersects the beam at the tumor location; the normal brain tissue receives less radiation and has less impact on the cognitive function.