AT/RT is a lethal disease, with initial retrospective studies showing a time-course from diagnosis to death of about 12-months with standard therapy. There are, still reports of long-term survivors. Various treatment options are available for atypical teratoid rhabdoid tumor depending upon the severity of the disease.
No potential data exist about the effect of surgical resection on treatment outcome. Data obtained from the AT/RT registry advocates that patients having a gross total resection have a longer median survival. 10 out of the 14 long-term survivors in the registry who had total surgical resection remained free from the disease 9.5–96 months from diagnosis.
For young age patients with AT/RT, chemotherapy has been found to be the major form of post-surgical adjuvant therapy. A range of chemotherapeutic moves has been exploited and the majority patients have received multi-agent treatment.
An additional therapeutic move towards patients with AT/RT is based on the IRS-III Study. This approach employs radiation therapy, hydrocortisone, intrathecal methotrexate, cytarabine and systemic chemotherapy with several agents.
Radiation therapy appears to have a rapid impact on survival for AT/RT patients. However, it is not clear whether early radiation followed by chemotherapy is more effective or not.