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 Medulloblastoma

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Medulloblastoma Brain Tumours
Definition, Prognosis and Treatment

 

William had Medulloblastoma Brain tumours.


Medulloblastoma is the most common malignant or high-grade paediatric brain tumour. At least 75% of medulloblastomas occur in the cerebellum at the back and base of the brain.


Medulloblastoma brain cancer is usually diagnosed as a grade 3 or grade 4 tumour. It is more common in boys than girls and is rarer in adults. It originates in embryonic (foetal) tissue hence is classified as an embryonal tumour and is typically discovered during the first five years of life.


This type of tumour is rare in adults


What is the survival rate for Medulloblastoma?
Survival rates in children with medulloblastoma depend on the patient's age and if the tumour has spread. 
If the disease has not spread, survival rates are above 60%, but if it has spread to the spinal cord, the survival rate is reduced.


Because medulloblastoma is such a rare disease in adults, prognosis is very much determined on a case by case basis. Like any high-grade brain tumour, the survival rate statistics will be lower than for patients with a low-grade brain tumour, for example.
 

The exact type of medulloblastoma makes a difference to the prognosis. For example, a WNT-activated medulloblastoma is considered relatively low-risk with a better prognosis than those classified as Sonic Hedgehog (SHH) activated, grade 3 or grade 4 (see below). 


For children and adults with a recurrent medulloblastoma (where the tumour grows back quickly despite having treatment to remove it), the prognosis is shorter, with a five-year survival rate of less than 40%.


Advances in surgery, radiation, and chemotherapy are contributing to an increase in survival rates, but side effects of treatment can produce long-term challenges for patients, including cognitive difficulties along with psychological and social problems. Longer term support services may be required in order to maximise quality of life. 
Research around the world is now focused on developing more personalised treatments that are more effective, with fewer side effects.

Are there different types of medulloblastoma?
Medulloblastomas are classified into four main groups using molecular and genetic analysis of biopsy tissue, and then into further subgroups using histology – variations identified on examination of tumour cells under a microscope. Tumours are thus given detailed, integrated classifications that are used to indicate a potential prognosis and guide treatment.

 

What do the different types of medulloblastoma mean?
The size, shape and physical characteristics of tumour cells when viewed through a microscope are reflected in names such as ‘large cell’ or ‘nodular’ medulloblastoma, but the four main groups of this tumour type are defined by how they act rather than how they look.

What are the symptoms of medulloblastoma?
Medulloblastoma often occurs in the cerebellum, the area governing motor functions such as movement, balance and co-ordination. 
Symptoms resulting from medulloblastoma growth can include:
•    Problems with walking
•    Increased stumbling and falling 
•    General co-ordination issues, with increasing clumsiness for         example


What causes a medulloblastoma?
The causes of a medulloblastoma are not yet clear, although SHH-activated medulloblastomas can sometimes be associated with a pre-existing condition called Gorlin Syndrome, also known as nevoid basal cell carcinoma syndrome. Gorlin Syndrome can lead to various types of cancer, including basal cell carcinoma, the most common form of skin cancer.

What treatments are used for medulloblastoma?
Surgery to remove all or as much of the tumour as possible usually comes first. This may include the insertion of a shunt in order to divert or regulate the flow of cerebral spinal fluid and / or a biopsy to extract some brain tumour tissue for classification and prognosis.
Surgery is likely to be followed by radiotherapy and chemotherapy.

How can we find a cure for medulloblastoma?
Research we are funding will help lead towards finding a cure for a wide range of brain tumours.
Pioneering researchers at our Brain Tumour Research Centre of Excellence at Queen Mary University of London are learning more about the molecular and genetic make-up of these challenging tumours in order to identify new ways to treat them. The William Low Trust are funding £143,657 to enable Thomas Willot’s to research into Medulloblastoma. Our aim to find a cure so that other people and families do not have to suffer as William did and his family have from this impact of this devastating disease. 

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