Specific treatment for a rhabdoid tumor will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- type, location, and size of the tumor
- extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- how your child's doctors expect the disease to progress
- your opinion or preference
A multidisciplinary approach, involving the neurosurgeon, pediatric oncologist, neurologist, and radiation oncologist is needed for these patients.
Treatment may include (alone or in combination):
- surgery - aggressive surgical removal followed by chemotherapy is standard treatment
- radiation therapy - children over 3 years of age will receive craniospinal radiotherapy with a boost to the primary tumor. For children under 3, radiotherapy is generally deferred, however, focal radiation therapy has been incorporated into some treatment protocols designed for this age group. Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.
- chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.
While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
- as a pill to swallow
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
Chemotherapy regimens have varied for patients with rhabdoid tumors, although none has been considered curative. Protocols have used cis-platin, VP-16, cyclophosphamide, and vincristine. Regimens for extracranial rhabdoid tumors have employed ifosfamide, doxorubicin, vincristine, and cyclophosphamide. The prognosis is very poor although objective responses have been observed but the duration is 2 to 5 months from diagnosis. Recurrent disease is managed symptomatically with consideration for palliative surgery and/or radiotherapy.