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Medulloblastoma

Medulloblastoma Overview

Medulloblastoma is a malignant type of brain tumor that first originates in the cerebellum, the lower back part of the brain. This area of the brain has various functions and processes, including controlling balance, muscle coordination and movement.

This tumor can sometimes affect adults, but it’s primarily found in children and is the most common pediatric malignant brain tumor. Medulloblastomas are very rare overall, but still represent a very serious condition, and getting familiarized with it is extremely important.

Let’s go over all of the different symptoms, causes and risk factors for medulloblastoma.

We treat medulloblastoma with a holistic approach that considers quality of life and how the whole body is functioning. From the first evaluation of your child’s case through our comprehensive follow-up services, you and your child will benefit from the expertise of compassionate medical professionals. Cancer can have devastating effects on the physical and emotional well-being of your child and his/her entire family, and we offer a variety of support services to address your needs.

Medulloblastoma - Sheba Medical Center

Medulloblastoma Causes and Risk Factors

As with most other tumors and cancers, medical professionals aren’t exactly sure what causes medulloblastoma. However, there are certain risk factors that increase someone’s risk of developing this tumor, and they are mostly genetic.

Some genetic conditions are associated with a higher risk of developing different brain tumors, including medulloblastoma. These conditions include:

BRCA1 gene mutations.

Lynch syndrome.

Turcot syndrome.

Nevoid basal cell carcinoma syndrome (NBCCS).

Other risk factors associated with medulloblastoma are age and gender because it’s mostly diagnosed in boys between the ages of 3 and 8. Of course, there are cases of medulloblastoma found in girls and in adults, but this is extremely rare. 

hemato-oncology

Medulloblastoma Symptoms

When a medulloblastoma develops, it can cause some or all of the following symptoms:

  • Persistent headaches, especially in the mornings or at night. 
  • Nausea and vomiting.
  • Head bobbing.
  • Poor coordination and trouble walking.
  • Abnormal side-to-side eye movements.
  • Double vision.
  • Feeling confused and lethargic.
  • A constant feeling of tiredness and dizziness.

Most brain tumors have similar if not identical symptoms, and the bigger the tumor gets, the more serious the symptoms are. 

Medulloblastoma Classification

Understanding medulloblastoma is much more complicated than just knowing it’s a brain tumor. There are many different types and subtypes of medulloblastoma, so let’s go through the classification of this tumor.

Medulloblastoma Types

There are four different types of medulloblastoma, and the main thing that sets them apart is their structure. These types are:

  • Anaplastic (large cell) medulloblastoma. Anaplastic medulloblastoma is characterized by large, abnormal cells that, when observed under a microscope, have anaplastic features. This means that these cells divide rapidly and are highly disorganized. This type of medulloblastoma has a very poor prognosis and is very aggressive. 
  • Classic medulloblastoma. This is the most common type of medulloblastoma and it is characterized by densely packed small, round, blue cells. The tumor cells for classic medulloblastoma are typically uniform and similar to primitive neural cells. 
  • Desmoplastic (nodular) medulloblastoma. Desmoplastic medulloblastoma is a type of medulloblastoma that is typically found in adults and is characterized by nodules or small masses of densely packed cells. 
  • Medulloblastoma with extensive nodularity (MBEN). MBEN is one of the more favorable types of medulloblastoma and is characterized by well-defined nodules. This is a very rare subtype and is characterized by the presence of extensive nodules within the tumor.

Medulloblastoma Subtypes

As for the different subtypes of medulloblastoma, there are also 4 that you need to be aware of:

  • Wingless (WNT) medulloblastoma. This subtype is characterized by mutations in the WNT pathway, which plays a crucial role in regulating the growth, differentiation, and mortality of cells. This subtype has a favorable prognosis and typically affects young children.
  • Sonic hedgehog (SHH) medulloblastoma. The SHH subtype of medulloblastoma is the second most common subtype of this tumor and it is characterized by alterations in the Sonic Hedgehog signaling pathway. It can occur in patients of all ages but is more commonly found in adults. 
  • Group 3 medulloblastoma. This subtype is characterized by a distinct gene expression pattern and a set of genes that easily mutate into tumor cells. Group 3 medulloblastoma is mostly found in young children and usually has a very poor prognosis, meaning it requires more intensive treatment.
  • Group 4 medulloblastoma. This is the most common subtype of medulloblastoma and is characterized by very few gene mutations. It’s fast-spreading and patients who get diagnosed with it are often considered to be high-risk.  

Understanding the types and subtypes of medulloblastoma is important because all of them have different characteristics and therefore require different approaches when treating them.

Medulloblastoma Diagnosis

If someone experiences the symptoms of a brain tumor, it’s essential to go for a check-up because only a medical professional can make the correct diagnosis.

These are all the steps a doctor will take if they suspect the presence of medulloblastoma:

  • Physical exam and medical history. Doing a physical exam and going over the patient’s health history to see if there are any reasons why a tumor could have formed (such as previous exposure to radiation and potential risk factors) is typically the first step in making the diagnosis. This will allow the doctor to make an initial assessment and decide what steps to take next in the diagnostic process.
  • Neurological exam. Those who suffer from a brain tumor also have neurological issues, which is why a neurological exam is performed to see if there are any side effects that are directly related to the tumor.
  • Imaging tests. Since a tumor is a mass that can be clearly observed with an imaging test, the doctor will order an MRI or a CT scan, both of which produce cross-sectional 3D images of a patient’s brain and spinal cord.
  • Lumbar puncture. Since brain tumors such as medulloblastoma spread to the cerebrospinal fluid (CSF), once the doctor confirms the presence of the tumor with an imaging test, they need to check if it’s spread. This is done by performing a lumbar puncture, also known as a spinal tap.
  • Biopsy. As we already mentioned, there are many types and subtypes of medulloblastoma. For the doctor to be able to understand which type the patient is suffering from, they will have to perform a biopsy. This is a procedure that requires removing a small piece of tissue from the tumor and examining it under a microscope to determine its molecular structure.

Medulloblastoma Treatment

When the doctor knows for certain that the patient is suffering from medulloblastoma and from what subtype, they can plan out the best treatment plan:

  • Surgery. A surgical intervention for medulloblastoma is performed to remove the entire tumor or at least part of it, depending on the location and size of the tumor. In some cases, surgery can’t be performed because if it was, it would cause serious neurological damage.
  • Chemotherapy. If there are still cancerous tumor cells left in the patient’s brain after surgery, the doctor will administer multiple rounds of chemotherapy. If there isn’t much of the tumor left, it will probably be high-dose chemotherapy.
  • Radiotherapy. Radiation therapy destroys cancerous cells with the use of high-energy rays. This can be a very effective treatment but when it comes to children under the age of 3, doctors try to avoid radiotherapy because it could potentially stunt their growth. 
  • Targeted therapy. This treatment option doesn’t cause damage to healthy cells since it specifically targets cancerous cells and destroys them. Targeted therapy kills cancerous cells by blocking the signals that are conveyed from one molecule to another inside that cell.
  • Bone marrow transplant. If a patient has recurrent medulloblastoma, they will most likely be treated with high-dose chemotherapy followed by a bone marrow transplant. This transplant will allow the patient’s body to create healthy stem cells and fight off the disease quickly.

 

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