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Medulloblastoma

Overview

Medulloblastoma is the most common type of central nervous system (CNS) tumour that forms in embryonic cells, remaining in the brain after birth. Medulloblastomas are typically fast-growing tumours. They form in the brain cells within the cerebellum, which is located at the back of the brain, and controls for balance, body movement and other complex motor functions.

Medulloblastomas can sometimes spread to the spine, bone marrow, lung or other parts of the body.
The survival rate for medulloblastoma ranges from 30% to 80%, depending on factors such as the age of the child, and the ‘risk level’ of their tumour (low, average or high-risk).

Risk Factors

Medulloblastoma is known as a primitive neuroectodermal tumour – it forms within embryonic cells and so preventative research is difficult, but ongoing.

A selection of inherited factors have been identified as risk factors for medulloblastoma, including Li-Fraumeni syndrome (which is characterised by an alteration in the p53 tumour suppressor gene), and Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin syndrome (a genetic condition that causes disorders of the skin, bones, nervous system, eyes and endocrine glands).

Symptoms

The symptoms of medullobastoma in children can include:

• headaches
• vomiting (particularly in the mornings)
• clumsiness or unsteadiness
• difficulty with handwriting and other motor skills
• problems with vision
• developmental delays in sitting, walking, talking in full sentences.

Diagnosis

Medullobatoma is diagnosed through tests that examine the brain and spinal chord. Further tests may be required to investigate the spread of the cancer through the body:

• physical examination
• neurological examinations (usually a series of questions and activities) to check brain function
• imaging of the brain (CT or MRI scan)
• lumbar puncture (using a needle to take a small amount of spinal fluid)
• tumour biopsy (removing and examining part of the tumour)

Treatment

Medulloblastoma is treated with surgery (the tumour is removed if possible), and then usually followed up with chemotherapy and/or radiation therapy to ensure any remaining cancer cells are targeted.

If the disease returns after initial treatment (recurrent medulloblastoma), further treatment may include surgery, radiation therapy, chemotherapy, and/or stem cell/bone marrow transplantation.

Children with medulloblastoma may experience certain negative side-effects after surgery or radiation therapy based on the area of the brain that has been effected. These side effects can include alterations in their ability to learn and pay attention, behavioural or mood swings, loss of balance, and delayed speech ability.

Research is being done to identify specific genes and proteins unique to medulloblastoma, so therapies can be developed that target these genes and proteins to stop the growth and spread of medulloblastoma tumours. Ultimately, the aim is to be able to tailor treatment to each individual patient, based on the specific features of their tumour.

Follow Up

All children treated for brain tumours of any type need follow-up and care. Depending on how their cancer was treated, children may need a variety of different types of examinations and tests to check for any late effects of treatment.

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