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Medulloblastoma is a type of brain tumor that forms primary in the central nervous system, usually in the cerebellum. The function of the cerebellum is controlling coordination movement and balance. It is generally classified as a Grade IV tumor which means it is malignant (cancerous) and spreads rapidly. Medulloblastoma is commonly diagnosed in children.
Medulloblastoma usually begins in embryonic cells of the cerebellum. Although it rarely spreads in different parts of the body, it can spread through the cerebrospinal fluid (CFS) that surrounds the brain and spinal cord.
The treatment for medulloblastoma is based on the tumor subtype, tumor extent and other medical related factors. Treatment options typically consists of surgery, radiation therapy and chemotherapy.
Symptoms
The signs and symptoms of medulloblastoma may vary depending on the tumor’s location or increased in pressure inside the brain. Common symptoms may include:
- Nausea and vomiting
- Headaches
- Fatigue
- Dizziness
- Double vision
- Poor coordination and balance
- Walking problems
- Urinary or bowel incontinence
- Back pain
- Seizures or fainting
- Confusion
Medulloblastoma is a serious condition and therefore children or adults who constantly experiencing any of the signs and symptoms should consult a healthcare provider for immediate assessment.
Causes
There is unknown cause for most medulloblastoma in adults and children. However, a small percentage of medulloblastoma in children is linked to genetic changes that can be inherited from parents such as Breast cancer gene (BRCA) mutation, Gorlin syndrome, and Turcot syndrome. Genes mutation can transform in many types of cancer, which can promote the growth and spread of cancer cells.
Risk factors
The underlying cause for primary brain tumors like medulloblastoma, is often unknown. However, several risk factors can contribute to its development, such as:
- Genetic: There are some genetic conditions that may increase the risk for developing medulloblastoma including:
- Breast cancer gene (BRCA) mutations: People who inherit specific versions of these genes are more susceptible to a wide range of cancers and have a younger age-onset cancer risk. BRCA1 (breast cancer gene 1) and BRCA2 (breast cancer gene 2) produce proteins that act as a tumor suppressor. Tumor suppressor protein help to prevent cell form uncontrolled cell growth.
- Gorlin syndrome: Also known as nevoid basal cell carcinoma syndrome (NBCCS). People with this condition have a 5% chance of having medulloblastoma. This is a rare genetic disorder that increase the risk for certain type of skin cancers (basal cell carcinoma) or noncancerous tumors.
- Turcot syndrome: A central nervous system tumor, such as a medulloblastoma or pituitary adenoma, as well as colorectal cancer are both present in people with Turcot syndrome, which is an uncommon genetic condition classified as colorectal (colon) cancer with primary brain tumors.
- Exposure to radiation: Ionizing radiation is a category of radiation that increases the risk of brain tumors. This is often found in radiation therapy for curing cancer and atomic bomb radiation exposure.
- Family history of brain tumors: Family history of genetic disorders or immediate family who had brain tumors before, although rare, can enhance one’s risk of having brain tumor.
Diagnosis
The diagnosis for medulloblastoma often involves discussing the symptoms experienced by the patient, evaluating the medical history, and conducting different tests and investigations, such as:
- Neurological exam: A neurological exam is an assessment tool to identify sign of disorder affecting the brain, spinal cord, and nerve. The neurological exam has many aspect of testing including coordination, balance, vision, muscle strength, and sensation.
- Imaging tests: Computerized tomography (CT) scan or magnetic resonance imaging (MRI) are commonly used to diagnose brain tumors. In some cases, perfusion MRI and magnetic resonance spectroscopy may be used. Through high-grade X-rays and radio waves, a detailed image which can aid in determining the location and size of a brain tumor is produced. These tests are also critical for detecting pressure or obstruction in the CSF pathways.
- Biopsy: The doctor may collect a tissue sample and send it to a pathology lab for testing and analysis. A biopsy is usually not performed, but it may be advised if the imaging tests are not typical pointed for medulloblastoma or failed to confirm the condition.
- Spinal tap (lumbar puncture): This test is usually performed after the pressure in the brain has been relieved or the tumor has been removed. It involves placing a needle between two bones in the lower spine to extract cerebrospinal fluid from surrounding the spinal cord. The fluid is examined for cancer cells or other abnormalities.
Treatment
The treatment plan for medulloblastoma requires careful consideration of many factors such as tumor grade and type, cancer characteristics, the patient’s age, and health at the time of diagnosis, and potential response to treatment. Several treatment options include:
- Surgical removal of medulloblastoma: Surgery is usually the first line of treatment for medulloblastoma. During the surgery, it is important not to damage nearby tissue while the tumor is being removed. However, because medulloblastoma grows near key structures deep within the brain, it is not always possible to completely remove the tumor. The surgery may be followed by chemotherapy, radiation therapy or both.
- Surgery to relieve fluid buildup in the brain: This surgery can be done with the removal of medulloblastoma. The goal is to create a passageway for the fluid to exit the brain, typically an external ventricular drain or ventriculoperitoneal shunt, may be advised. This occurs because when a medulloblastoma grows enough to impede the flow of cerebrospinal fluid, causing an accumulation of fluid that exerts pressure on the brain.
- Radiation therapy: Surgery is usually followed by radiation to the brain and spine because the disease can spread in these areas. This treatment uses high-powered X-rays to destroy cancer cells. In some cases, a proton beam radiation therapy is recommended as it focuses on killing cancer cells while preserving healthy tissues. It minimizes the possible complication with bone marrow and nearby organs during a standard brain and spine radiation.
- Chemotherapy: Chemotherapy may be part of the post-surgery treatment plan alongside or after radiation depending on the amount of tumor left. Following chemotherapy, a stem cell transplant may be done. The drugs used in this treatment are either ingested in pill form or injected into a vein (intravenous chemotherapy) to destroy cancer cells and prevent tumor growth.
- Clinical trials: These trials allow an opportunity to explore the latest medical methods such as new chemotherapy, targeted therapy, or immunotherapy medications that may treat medulloblastoma. The patient’s medical team decides on possible treatments based on the patient’s age, remaining tumor after surgery, tumor type, tumor location and danger of unknown side effects.
