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Prognosis

How well a patient responds to treatment is affected by their age at the time of diagnosis; the size and extent of the tumor; the amount of mass that can be removed safely; and the level of metastatic disease (the M stage). Overall, the Central Brain Tumor Registry of the United States reports about 65% – 70% of adults (age 20+) with medulloblastoma are alive at 5 years following diagnosis. It is important to realize these statistics do not reflect differences in outcome between low risk and high risks groups (since high risk groups may not do as well), differences in patient characteristics, nor differences between patient responses to treatment.

With current therapies, 70% – 80% of children with average-risk medulloblastoma can be expected to be alive and free of disease five years from diagnosis. Even in those children with highrisk disease, effective therapy is possible and results in long-term disease control in as high as 60% – 65% of patients. Outcome for infants is poorer, but for those infants with localized disease at the time of diagnosis, survival rates in the 30% – 50% range are being seen.

Take the opportunity to speak with the healthcare team treating you or your child to learn how these statistics apply to your individual situation.

Medulloblastoma patients have a significantly increased chance of survival, thanks to improved treatment techniques. The best results, however, occur when patients are cared for by an experienced multi-disciplinary team of medical professionals at an established pediatric or adult cancer center.

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Recurrence

Tumors recur when all the tumor cells cannot be removed by surgery or killed by other treatments. In children, medulloblastoma tends to “seed” or drop tumor cells into the spinal fluid. These cells can give rise to tumor growth in the spine. This type

of spread may or may not be accompanied by tumor regrowth in the cerebellum. In adults, the tumor tends to first regrow in the cerebellum. On very rare occasions, the tumor may spread elsewhere in, and outside, of the central nervous system.

Recurring medulloblastoma is treated aggressively with repeated surgery, re-irradiation if possible, and chemotherapy. Recurrences limited to the cerebellum (the posterior fossa) offer the best chance of long-term survival since treatment can

be aimed at the “local” site. Surgery or radiation therapy focused on the regrowth may be a choice.

Chemotherapy may be of benefit if the tumor spreads beyond the local area. If chemotherapy was not used for the initial tumor, it may now be a consideration. Patients who previously received chemotherapy can be given different drugs for the recurrence. High-dose chemotherapy may be considered, as might a clinical trial investigating new therapies.

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Follow-up

MRI scanning of the brain will be done every 2-3 months and spinal MRI every 4-6 months for the first two years following surgery. The scans help determine the effectiveness of treatment, and are used to monitor for early evidence of a recurrence. Scans will be done less frequently thereafter, unless specific symptoms develop that might indicate further growth. Your doctor will determine the appropriate schedule.

Children should be carefully evaluated for long-term cognitive problems, and should

receive early aggressive learning support. Neuropsychological testing before treatment can serve as a baseline for follow up evaluations. If learning concerns arise after treatment, these baseline results can be used as a tool for comparison. Children should be carefully evaluated for long-term cognitive problems, and should receive early aggressive learning support. In addition, your doctor may refer you to other specialists, such as an endocrinologist (a physician specially trained in treating growth or hormone imbalances), or an oncologist (a physician trained in treating cancer, particularly with chemotherapy drugs). Rehabilitation and special education programs will play a vital role in returning children to school.

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Side Effects

Despite its impact on increasing survival, the tumor and its treatment can cause significant side effects. Your healthcare team can speak with you about the potential side effects of your/your child’s personalized treatment plan, and help you weigh risks against the benefits. Some of the more common effects are discussed here. In a recent study, about 25% of children undergoing surgery for their tumor developed delayed onset (usually 6 to 24 hours after awakening) loss of speech which was often associated with decreased muscle tone, unsteadiness, emotional lability, and irritability. This syndrome, called “posterior fossa mutism syndrome” or “cerebellar mutism” seems to occur predominantly after surgery in children with medulloblastoma, and has not been clearly related to tumor size or surgical approach. Many of these children recover, but the study noted that some children still have significant neurologic problems – such as abnormal speech and unsteadiness — a year after surgery.

If mutism occurs, a speech pathologist can help outline a temporary communication plan for your child, and help initiate a rehabilitation evaluation. The rehab team can plan a program specialized to your child’s needs and strengths.

Understandably so, parents and adult patients often express concern about the effects of radiation therapy. In the short-term, fatigue, lack of appetite, nausea, sore throat, difficulty swallowing, and hair loss in the path of the radiation beams are the most common acute effects of this treatment. Adults seem to experience these temporary, short term effects to a greater degree than children.

Children appear to experience greater intensity of the long-term effects. Radiation may trigger a decrease in IQ or intellectual ability, accompanied by learning disabilities, attention deficit and memory loss. Most of this research has focused on

children: The younger the child during treatment, the greater the potential subsequent learning challenges. Infants and children less than 3 years of age are particularly vulnerable because the brain is maturing rapidly during this time. For any age group, however, the radiation oncologist will be able to talk with you about what you can expect based on age and the planned dose of radiation.

Radiation can also have long-term effects on the hypothalamus and pituitary, two glands that contribute important hormones for bodily function and growth. Since these glands are directly in the pathway of the radiation beam, their normal function may be disturbed by the treatment. As a consequence, patients can have problems with obesity and hypothyroidism (thyroid deficiencies). They also may experience short stature and scoliosis (curvature of the spine) if the spinal cord is irradiated.

Patients should be evaluated carefully for hypothalamic or pituitary dysfunction and receive replacement therapy. Studies have not shown that children treated with growth hormone replacement are at a higher risk for tumor recurrence. Hearing loss may accompany the use of the drug cisplatin in children. Because this drug has an important role in treating childhood medulloblastoma, scientists are testing “protective” drugs that may be able to defend a child’s hearing mechanisms from cisplatin. This research is ongoing. Hearing may also be affected if radiation beams pass near the ears; an otolaryngologist (an ear, nose and throat doctor) can be of help in diagnosing and treating this effect.

The short-term effects of chemotherapy are similar to those of radiation: Hair loss, nausea, vomiting, fatigue and weakness. But chemotherapy can also lead to reduced blood counts and kidney problems. As patients live longer, there’s the added risk of secondary malignancies, such as leukemia.

Doctors continue to study the long-term effects of both radiation and chemotherapy in hopes of developing new agents and combinations of agents. Discoveries continue to emerge about the molecular mechanisms used by tumor cells to evade the body’s normal growth controls, and the methods by which tumor cells move through the brain or spine.

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Chemotherapy

Chemotherapy uses powerful drugs to kill cancer cells. For children with medulloblastoma, chemotherapy is used to reduce the risk of tumor cells spreading through the spinal fluid. For adults, this benefit is not quite as clear since their tumors tend to regrow in the cerebellum. Because different drugs are effective during different phases of a cell’s life cycle, a combination of drug may be given. The combination increases the likelihood of more tumor cells being destroyed.

Chemotherapy is now a standard part of treatment for children with medulloblastoma. Most children are treated in clinical trials — organized studies that are helping determine which treatments are most effective. Clinical trials also offer a formal way to test new therapies against existing therapies to learn which is better.

In children at average-risk of recurrence, current studies are exploring the use of chemotherapy as a way to reduce the total amount of craniospinal radiation. There are several treatment plans in use, but most focus on a combination of vincristine, cisplatin, lomustine, and/or cyclophosphamide.

For children at high-risk, the drugs vincristine, cisplatin, and cyclophosphamide tend to be the main focus, but others are being tested in clinical trials. Researchers are also looking at the use of chemotherapy as a radiation sensitizer, and at “post-radiation” high-dose chemotherapy accompanied by a stem cell transplant.

For infants under the age of 3, chemotherapy is used to delay or even eliminate radiation therapy. Cyclophosphamide, vincristine, cisplatin, etoposide, carmustine, procarbazine, cytarabine, and/or hydroxyurea may be found in these treatment plans. New drugs are under consideration, but their effectiveness is generally determined in older children prior to use in infants. Some treatment plans use higher doses of chemotherapy, supported by peripheral stem cell rescue, for infants. There is also interest in instilling chemotherapy directly into the cerebrospinal fluid (either “intrathecally” — into the lumbar spine by spinal taps, or “intraventricularly”—

into the ventricular fluids of the brain via an Ommaya reservoir). This is being done in attempts to deliver high doses of therapy to the coating regions of the brain and reduce disease relapse in these areas. In addition, studies are underway evaluating the efficacy and safety of utilizing local radiation therapy (radiation therapy only to the primary tumor site) after chemotherapy in infants whose initial disease was limited to the posterior fossa.

In adults, the usefulness of chemotherapy is less clear. Although large scale studies have not been done, some smaller studies indicate adult tumors may likewise respond to some of the above combinations. But adults seem less able to withstand potential side effects, especially those of the lomustine and cisplatin used in some treatment plans. Studies are exploring the use of cyclophosphamide, ifosfamide, etoposide, or carboplatin in adults, and other studies are exploring pre-radiation chemotherapy plans as alternatives.

Research continues into defining the best use of chemotherapy in average-risk patients; the best tolerated drugs in adults: and new drugs targeted to specific genetic changes found in medulloblastomas. Your doctor will outline a treatment plan based on current studies, the patient’s age, the amount of remaining tumor, and the risk of further disease.