Cancer in children Flashcards
(47 cards)
How many children are affected by cancer in the UK
1/500
What % of childhood cancers are brain tumours
25
What are the effects of cancer treatment exaggerated in childhood
Chemotherapy gets incorporated into dividing cells, there are lots of dividing cells in childhood as they’re growing and developing
What is an embryological tumour
Malignancies originating in developing tissues and organ systems
What does cancer arise from
Accumulation of genetic aberrations in somatic cells
What do aberrations consist of
Mutations and chromosome defects
How do oncogenes act
By gain of function
Does one or both allele need to be activated in oncogenes
Just one
Name 4 ways in which oncogenes are activated
Mutation
Chromosome translocation
Gene amplification
Retroviral insertion
How do tumour suppresor genes acts
By loss of function
Do you need to inactivate both alleles for tumour suppressor genes to cause cancer
Yes
Describe the two hit model for tumour suppressor genes
Deletion/ mutation in somatic or inherited cells
Then a second hit
What forms can the ‘second hit’ in the 2 hit model take
Loss Loss and duplication Chromosome deletion Mutation Recombination
Another name for Wilm’s tumour
Nephroblastoma
How many children are affected by Wilm’s tumour
1/10,000
How does Wilm’s tumour usually present
Asymptomatic abdominal mass without metastasis
What is WAGR?
Wilm’s tumour
Aniridia
Genito-urinary abnormalities
Mental Retardation
Name a predisposition syndrome associated with Wilms tumour
Beckwith-Wiedeman Syndrome
What cells does Wilm’s tumour arise from?
Pluripotent embryonic renal precursors
What 3 cell types present in embryonic kidney and are found in Wilm’s tumour>
Blastema
Epithelia
Stroma
What does Wilm’s tumour resemble?
Nephrogenic mesenchyme
What somatic genes are inactivated in Wilms tumour (3)
WT1
WTX
TP53
What gene is activated in wilms tumour
CTNNB1
What germline altercations occur in Wilm’s tumour
WT1 gene
1GF2/H19 locus
