Treatment of Tumors of the Choroid Plexus Epithelium

Tumors of the choroid plexus epithelium are rare. Participants in this study will have surgery to remove as much of the brain tumor as possible. Taking as much of the tumor out during surgery is generally believed to have the best result for this disease. Some participants may even have a second surgery to remove more tumor if thought necessary. After the tumor surgery, exact treatment for each participant in this study will depend on how much of the tumor is removed during surgery and the way the tumor tissue looks under a microscope. Some participants will not require additional treatment because most or all of the tumor has been taken out. Those participants will still be on study, but they will only have observation and not receive additional treatment. Those that require additional treatment will be randomly assigned (as in the toss of a coin) to one of 2 treatment groups with an equal chance of being assigned to either group. Chemotherapy (treatment with anti-cancer drugs) will be given as part of treatment for participants whose tumors are not completely removed surgically. Participants in one group will receive carboplatin plus etoposide, vincristine, and radiation therapy . Participants in the other group will receive cyclophosphamide plus etoposide, vincristine, and radiation therapy. Among all the known drugs for cancer, etoposide, vincristine, cyclophosphamide, and platinum drugs are the most effective against brain tumors. Carboplatin will be used because fewer side effects related to hearing should occur later on. This study also uses radiation therapy after surgery for children younger than 3 years old. Normally, chemotherapy has been used to delay radiation therapy until the child was older because of concerns about side effects. This change has been made because of the poor results achieved when chemotherapy was used to delay radiation therapy. Chemotherapy is the one and only additional treatment that participants under 3 years of age can receive in this study. After the first 2 cycles, response will be evaluated, including all exams done at screening before you continue on treatment, if needed. Further surgery will be considered after these exams. If both you and your doctor choose to consider further surgery and agree for the procedure to be the next appropriate step, you may undergo a second surgery to remove anymore remaining tumor. After the second surgery, the chemotherapy will be again continued on the same schedule for 4 more cycles. If you did not require further surgery, you will continue on with the chemotherapy as previously planned. For participants older than 3 years of age, radiation therapy will be a part of the treatment. It will be given after the second cycle of chemotherapy. Participants will receive radiation once per day, five days per week, over a period of about 6 weeks. Most of the participants do not need to stay in the hospital during this time. This will be followed by 6 more cycles of chemotherapy. After 6 cycles of chemotherapy, further surgery will again be considered. While on radiation treatment, you will have blood (about 2 teaspoons) drawn for routine tests 2 times a week. Before and after the finish of radiation, another blood test (2-3 teaspoons) will be taken to monitor the kidney and liver function, as well as to measure levels of hormones. You will have a physical exam, and blood (about 2 teaspoons) will be drawn for routine tests before each cycle of treatment. Participants in the carboplatin group will receive etoposide over 1 hour on Days 1-5, carboplatin over 2 hours on Days 2 and 3, and vincristine over 15 minutes on Day 5. This will be repeated every 4 weeks for 24 weeks. Each period of 4 weeks is considered 1 cycle of treatment. Participants in the cyclophosphamide group will receive etoposide over 1 hour on Days 1-5, cyclophosphamide over 1 hour on Days 2 and 3, and vincristine over 15 minutes on Day 5. This will be repeated every 4 weeks for 6 cycles (24 weeks) of treatment. Mesna, a drug to protect the bladder from the effects of cyclophosphamide, will be given 15 minutes before each dose of cyclophosphamide. The chemotherapy given in this study can cause your white blood cell count to be too low. White blood cells are infection-fighting cells. If the white blood cell count is low for too long, participants in both groups may be given a drug called G-CSF (filgrastim). Filgrastim is a growth factor naturally produced in the body to increase the production of white blood cells. Filgrastim will be given as a shot under the skin starting at Day 9 after starting the chemotherapy. The total length of treatment that you can receive will be 7 months, if you are able to complete all the cycles of the additional treatment. You will be taken off study if the disease gets worse or intolerable side effects occur, and your doctor will discuss other treatment options with you. After completing treatment, there will be follow-up visits every 3 months for the first year. Every 6 months there will still be visits with the doctor until 4 years after completing treatment. You will continue to have follow-up visits every year after that to monitor for any signs of the disease coming back, or for as long as you would agree to allow follow-up visits. At each visit, you will have routine blood tests (about 2-3 teaspoons), checking of hormone levels, measurement of growth, and a hearing test. The effects of radiation/chemotherapy on your brain function, your ability to learn, and your quality of life will be measured. You will have a MRI of the brain, all known metastatic sites, and of the spine. Your body height and body weight will be measured. About 2 teaspoons of blood will be drawn for routine tests. Your urine will be tested for the presence of blood. You will have a spinal tap to look for cancer cells in the spinal fluid. Your hormone levels will also be taken to see if there are any signs of metabolic disorder and growth deficiency. This will be done with the other blood test and will not require any more extra blood samples from you. This is an investigational study. All of the drugs used in this study are FDA approved and are commercially available. Their use together in this study is experimental. A total of up to 100 patients will take part in this multicenter study. Up to 5 will be enrolled here at M. D. Anderson.