Atypical teratoid rhabdoid tumor medical therapy

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.

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