Medulloblastoma

Overview


Tumour Group: Pediatric Brain Tumours
WHO Grade: Grade IV
Prevalence/Incidence: Medulloblastoma is relatively rare and accounts for less than 2% of all primary brain tumours and 18% of all pediatric brain tumours. Medulloblastoma occurs more often in men than in women.
Typical Age Range: More than 70% of all pediatric medulloblastomas are diagnosed in children under age 10; very few occur in children up to age one.

Medulloblastoma in adults is far less common, but does occur. About one-third of all medulloblastomas diagnosed in the United States are found in adults between the ages of 20-44. The incidence in adults sharply decreases in frequency after age 45, with very few older adults having this tumour.

Contents
Description of Tumour
Symptoms
Treatment / Standard of Care
Prognosis
References

Description of Tumour


Medulloblastoma is a fast-growing, high-grade brain tumour. Like many tumour types, the exact cause of medulloblastoma is not known. However, scientists are making significant strides in understanding its biology.

Changes have been identified in genes and chromosomes (the cell’s DNA blueprints) that may play a role in the development of this tumour. There are also a few rare, genetic health syndromes that are associated with increased risk for developing this tumour. These include Gorlin syndrome and Turcot syndrome.

Different subtypes of medulloblastoma tumours have recently been identified and described using "molecular classification." Each subtype has different characteristics and may:

  • Be more likely to occur in a specific patient age range
  • Have different pathological, genetic and molecular biology characteristics
  • Have different clinical outcomes (some subtypes have a much better prognosis than others)

This classification was outlined in a manuscript produced as a result of a consensus conference held by experts in Boston, MA in 2010. The four main subtypes of medulloblastoma are:

  1. Wnt
  2. Shh (Sonic Hedgehog subgroup)
  3. Group 3 tumours
  4. Group 4 tumours

Symptoms


Common symptoms are usually those of raised intracranial pressure and include:

  • Behavioural or personality changes
  • Double vision
  • Headaches (which are worse first thing in the morning)
  • Lack of coordination
  • Lethargy or sleepiness
  • Vomiting

Treatment / Standard of Care


The management of medulloblastoma involves a multidisciplinary team:

  • Surgery is the most important first line treatment and involves removing as much of the tumour as possible.
  • Sometimes the CSF pathways do not re-establish themselves after surgery and then a shunt may be necessary to treat the raised intracranial pressure (hydrocephalus).
  • Chemotherapy is usually an important part of the treatment plan. In very young children aged 3 years or less, it is standard to treat children with medulloblastoma using chemotherapy alone as radiation therapy can be associated with significant long-term health problems (late effects).
  • Radiation therapy is used for older children and involves treatment of the brain and spine followed by a “boost” of extra radiation therapy to the area where the tumour started in the posterior fossa.

Prognosis  


A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. Since every person is different, please take time to talk with your health care team about how this information applies to you.

By clicking on 'Expand,' a statistic on the prognosis for medulloblastoma will be shown.

Expand for Prognosis Information

For brain tumour patients, a prognosis depends on several factors, which can include age and other health issues, the size of the tumour, its molecular profile, the type of tumour, how much can be removed and its response to treatments such as chemotherapy and radiation therapy.

The outcome for patients with medulloblastoma depends on many different factors which include:

  • Extent of disease at presentation. Children who have tumour that has already spread through the CSF pathways when they are first seen (high-risk disease) have a much worse prognosis than those where the tumour is localised (average-risk disease)
  • If all of the tumour can be surgically resected, then there is less chance of the tumour recurring later
  • The molecular classification of the tumour
  • The age of the child (younger children have a poorer prognosis)

With current therapies, at least 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 high-risk disease, effective therapy is possible and results in long-term disease control in as many as 70% 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.

References


 Adult Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

Louis, David N. WHO Classification of Tumours of the Central Nervous System. Lyon: International Agency for Research on Cancer, 2007. Print

"Medullobastoma." American Brain Tumour Association. American Brain Tumour Association, n.d. Web. July 4, 2013.

“Medulloblastoma.” The National Brain Tumour Society, n.d. Web. July 4, 2013.

"Molecular Subgroups of Medulloblastoma: The Current Consensus - Springer." Molecular Subgroups of Medulloblastoma: The Current Consensus - Springer. N.p., 01 Apr. 2012. Web. 21 Sept. 2013.

"National Cancer Institute." Childhood Central Nervous System Embryonal Tumors Treatment (PDQ -. N.p., n.d. Web. 21 Sept. 2013.

Image credited to The Armed Forces Institute of Pathology [Public domain], via Wikimedia Commons

  
You can also download this information as a Information Sheet on Medulloblastoma (pdf).

 

Riley was just six-and-a-half when he was diagnosed with a fast-growing medulloblastoma, one of the most common pediatric brain tumours.

He had to have several surgeries, plus chemotherapy and radiation, to treat the cancer. Riley had to learn how to walk and talk again. He was fed through a tube for two years. But despite all of the challenges that came with Riley’s diagnosis and recovery, his family never lost hope. Today Riley is a recent high school graduate and looking forward to a bright future.

Watch this inspiring video of Riley and his mom, Coral-Ann >>

 

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