Medulloblastoma

Medulloblastomas are characterized by a high degree of malignancy and a tendency to relapse. Standard treatments for these tumors have several limitations and are associated with serious side effects. Therefore, in recent years, there has been an active search for new, safer and more effective methods of therapy. In this article, we have collected all the most up-to-date information about medulloblastomas and innovative methods of their treatment.

What is medulloblastoma?

Medulloblastoma is an aggressive tumor that affects the cerebellum in the brain and spreads through the cerebrospinal fluid. During the first few years after diagnosis, the mortality rate from this disease is 15%. However, the use of modern therapeutic methods can achieve a cure rate of 60%. Surgical resection with prior or subsequent chemotherapy and radiation therapy is the standard therapeutic protocol. However, in recent years, other methods have been actively used, such as immunotherapy, vaccine therapy, and targeted therapy.

Medulloblastoma in children

Medulloblastoma is the most commonly diagnosed malignant brain tumor in children. It accounts for about 20% of all brain tumors in pediatric patients. The most common age of diagnosis is between 5 and 9 years of age. The disease is more common in boys than girls.

Medulloblastoma in adults

This type of cancer is extremely rare in adults. The incidence rate is approximately 0.5 cases per million per year and decreases with age. The tumor is diagnosed mainly in young people from 20 to 40 years old. As with pediatric patients, medulloblastomas are more common in men than in women.

Causes of medulloblastoma

The exact causes of tumor development are unknown. Scientists suggest a link to certain genetic disorders, such as:

• Li-Fraumeni syndrome (hereditary high-risk tumor syndrome);
• Gorlin’s syndrome (basal cell nevus syndrome);
• Turcot syndrome (a rare hereditary syndrome accompanied by the development of brain tumors and colorectal cancer).

Some scientists have found a link between medulloblastoma and maternal diet and immune disorders during pregnancy. There are also studies pointing to an increased risk of developing a tumor in children who have had a cytomegalovirus infection in childhood. However, there is not yet enough scientific evidence to support these theories.

Symptoms and signs of medulloblastoma

The most common manifestations of medulloblastoma include:

• headache (especially severe when waking up in the morning);
• nausea and vomiting;
• general deterioration of well-being, chronic fatigue, drowsiness;
• impaired coordination of movements;
• violation of fine motor skills;
• diplopia (double vision);
• stiffness of the occipital muscles of the neck, tilt of the head;
• sudden mood swings;
• convulsive seizures.

If the medulloblastoma has spread to the CNS or spinal canal, the following symptoms may be present:

• weakness in the legs;
• backache;
• impaired bladder and bowel control;
• gait disturbance.

When anxiety symptoms appear, it is important to immediately consult a doctor. Call or write to us. The doctor-coordinator of the MedTour platform will help you choose a clinic and an experienced oncologist specializing in the diagnosis and treatment of medulloblastoma for free.

Types of medulloblastoma

Tumors of this type are classified according to the histological characteristics of the cells of which they are composed. There are the following types of neoplasms:

• Classical medulloblastoma. It consists of small round cells with dark nuclei. Cells are located close to each other.
• Desmoplastic or nodular medulloblastoma. It has a nodular structure. In this case, the tumor is permeated with thin collagen fibers, giving it a dense texture.
• Medulloblastoma with extensive nodularity. Histologically similar to desmoplastic medulloblastoma, but with a more nodular structure.
• Large cell/anaplastic medulloblastoma. Consists of cells of irregular shape with large nuclei.

To determine the prognosis and select the optimal therapeutic tactics, doctors also classify medulloblastoma according to molecular characteristics. There are 4 groups of tumors:

• SHH group. The most common form of medulloblastoma in patients under the age of 3 years (diagnosed in about two-thirds of cases). It also occurs in adults over 17 years of age. This form accounts for about 30% of all cases of the disease. It is named after the Sonic Hedgehog, a gene whose overactivation leads to the development of medulloblastoma.
• WNT group. It occurs mainly in children over 4 years of age. This subgroup accounts for about 10% of all cases. It occurs equally in both women and men. The name comes from the term WNT, which refers to the signaling pathway that controls cell division, growth, and differentiation. Mutations of proteins in the WNT signaling pathway lead to the development of medulloblastoma.
• 3rd group. It mostly occurs in infants and children. Tumors of this group account for about 25% of all cases of medulloblastoma. Men get sick twice as often as women. Patients suffering from this form of the disease are at high or very high risk, depending on their age and the molecular features of the neoplasm. As a rule, patients need more intensive treatment, including radiation therapy.
• 4th group. The most common form of medulloblastoma in patients of all ages. It accounts for 35-40% of all cases. This type of tumor occurs three times more often in men than in women. Neoplasms of the 4th group have several gene mutations. By the time it is discovered, the cancer has usually already spread. Patients with this form of the disease are at intermediate to high risk.

Determination of the molecular form of medulloblastoma is of great importance when choosing a method of treatment, therefore, the diagnosis should be carried out by an experienced doctor, and the clinic should have modern laboratory and instrumental equipment.

Stages of medulloblastoma

The stages of the disease depend on the size and extent of the tumor in the brain:

• T1 – tumor less than 3 cm in diameter;
• T2 – neoplasm greater than or equal to 3 cm;
• T3a – the tumor is larger than 3 cm and has spread to the aqueduct of Sylvius or Luschka’s foramen;
• N3b – neoplasm larger than 3 cm with infiltration of the floor of the 4th ventricle and brainstem;
• T4 – a tumor larger than 3 cm, growing into the 3rd ventricle through the aqueduct or below the foramen magnum.

There are also stages describing the absence or presence of metastases:

• M0 – no metastases;
• M1 – tumor cells are found in the liquor (cerebrospinal fluid);
• M2 – the tumor has spread to other parts of the brain;
• M3 – the tumor has spread through the spinal subarachnoid space (into the spine);
• M4 – the malignant process has spread beyond the central nervous system (bones, lungs, liver).

Accurate tumor staging is of great importance in determining the prognosis and choosing a therapeutic strategy.

Diagnosis of medulloblastoma

As a rule, the diagnostic process begins with a neurological examination. After that, an MRI is performed to determine the presence of a tumor. Medulloblastoma may be similar to other types of brain tumors. Therefore, after the operation, a sample of pathological tissue is sent for histological analysis. According to its results, the doctor makes a final diagnosis and selects a therapeutic tactic. If there is a suspicion that the malignant process has spread beyond the brain, a lumbar puncture is performed (cerebrospinal fluid sampling for subsequent analysis).

Treatment of medulloblastoma

The first line treatment is surgery to remove the tumor. Most patients with medulloblastoma require follow-up therapy. The protocol may include chemotherapy, radiotherapy, or innovative techniques that are carried out as part of clinical trials.

After surgery, doctors usually prescribe radiation therapy, as medulloblastomas are prone to metastasis. Since there is a risk of tissue damage to the brain and spinal cord, many patients are recommended more gentle proton beam therapy.

Chemotherapy, as well as the use of targeted and immunotherapeutic drugs, may be part of the treatment protocol for some types of medulloblastoma. It is important that the therapeutic plan is developed by an experienced neurooncologist, and that the clinic has access to modern methods of treatment. Call or write to us, and the MedTour coordinator will select the best specialist and medical center, taking into account your personal needs and wishes.

How is medulloblastoma removed?

The goal of surgical treatment of medulloblastoma is to resect (remove) as much of the tumor as possible without damaging nearby healthy tissue, and to reduce intracerebral pressure caused by blockage of cerebrospinal fluid (CSF).

The use of modern surgical techniques allows doctors specializing in the treatment of brain tumors to remove the tumor completely or almost completely. To destroy malignant cells that could remain after the operation, chemotherapy or radiation therapy is prescribed.

Children with medulloblastoma often require treatment for hydrocephalus. For this purpose, either ventriculoperitoneal shunting or endoscopic ventriculostomy of the third ventricle is performed. Both surgeries are performed to reduce pressure in the brain and relieve the symptoms of hydrocephalus, which are present in a third of patients with medulloblastoma.

Where does medulloblastoma metastasize?

The tumor usually metastasizes along the cerebrospinal fluid pathway. Extracranial (outside the brain) spread is rare, and is more common in children. The most common sites of metastasis are bones and bone marrow, lymph nodes, and, to a lesser extent, the liver, lungs, and peritoneum.

Treatment of medulloblastoma with cancer vaccines

Malignant brain tumors are the most common cause of cancer death in children. Despite aggressive and highly toxic multimodal therapy, including surgery, craniospinal irradiation, and high-dose chemotherapy, nearly half of children with medulloblastomas die of relapse.

In addition, aggressive treatment often leads to severe, lifelong cognitive and motor impairment. Therefore, more effective and targeted treatments are being developed. The use of antitumor vaccines is one of the most promising of them.

Conventional treatment often fails as cancerous cells develop mechanisms that make them invisible to the immune system and resistant to therapy. For the manufacture of cancer vaccines, either the patient's own tumor sample obtained during surgery, or animal proteins, which are structurally similar to tumor antigens, are used.

Once in the body, the vaccine trains the immune system to recognize malignant cells, attack and destroy tumor tissue. Thus, vaccine therapy activates the patient’s own defense system to fight the tumor. Treatment helps improve the outcome of standard therapies and reduce the risk of recurrence of medulloblastoma.

One of the main objectives of the company MedTour is to make innovative methods of treatment more accessible to patients. Currently, we are collaborating with the best clinics in the world that treat brain tumors with antitumor vaccines. At the request of the patient, we will help organize treatment in Ukraine, Turkey, Germany and other countries. To get a free consultation with a MedTour coordinating doctor, call us or fill out the feedback form.

Prevention of medulloblastoma

Unfortunately, at the moment there are no known specific methods for preventing the disease, since the exact causes of its occurrence are not yet fully understood. However, there are some general measures that can help promote health and prevent the development of brain tumors:

• Healthy lifestyle. Proper nutrition, regular physical activity and healthy habits help support the immune system and reduce the risk of cancer.
• Healthy environment. Avoiding exposure to harmful chemicals and toxins reduces the chance of developing cancer.
• Genetic counseling. If there have been cases of brain tumors in the family, it is important to be tested for hereditary diseases that can provoke their development.

In addition, it is necessary to undergo regular medical examinations. Early detection of the tumor is the key to a successful cure.

How long do people live with medulloblastoma

The prognosis largely depends on the type and stage of the neoplasm, as well as on the treatment being performed. With timely initiation of therapy, including surgical resection in combination with chemotherapy or radiation therapy, the 5-year survival rate for children with medulloblastoma is from 50% to 90%. In adult patients, this figure is almost the same, ranging from 58% to 84%.

Best clinics for medulloblastoma treatment

The MedTour company cooperates with the best oncology centers for the treatment of brain tumors. These are clinics that have modern equipment and have access to modern therapeutic protocols. Write or call us and we will select the best oncology center according to your personal needs and wishes.

Best doctors for medulloblastoma treatment

The MedTour platform contains information about the best neurooncologists and neurosurgeons specializing in the treatment of medulloblastoma. Contact our medical coordinator in any way convenient for you (by phone or using the feedback form) to find the best available specialist in this field.

Published: 16.08.2023

Updated: 03.09.2023

Medical author, Medical editor:

Olena Kursabaieva

Medical author, Medical editor:

Natalia Segen