Cancer In Children Flashcards
Why can cancer have lifelong effects
Tumour effects
Chemo effects
Radio effects
Brain damage
Malignancies originating in developing tissues and organ systems
Wilms tumour
Retinoblastoma
Neuroblastoma
Acute lymphoblastic leukaemia
Medulloblastoma
Hepatoblastoma
Rhabdomyosarcoma
Germ cell tumours
What types of genetic alterations cause cancer
Mutations
Chromosome defects
Epigenetic changes
Do oncogenes and tumour suppressor genes cause cancer by gain or loss of function
Oncogenes - gain
Tumour suppressor genes - loss
How are oncogenes activated
Murptatiom
Chromosome translocation
Gene amplification
retro viral insertion
What type of genes are RB, WT1, TP53, PTCH1, BRCA1, BRCA2, APC, NF1
Tumour suppressor genes
What type of genes are MYCN, MDM2, ALK, ABL1, HRAS, KRAS, NRAS, BRAF
Oncogenes
How are tipumour suppressor genes inactivated
Mutation
Deletions
DNA methylation - epigenetic
Are oncogenes or tipumour suppressor genes dominant
Oncogenes - dominant - activation of 1 allele sufficient
Tumour suppressor genes - recessive - inactivation of both genes necessary
Wilms tumour
Nephroblastoma - Kidney tumour most common under 5 yrs
Wilms tumour presentation
Asymptomatic posterior abdominal mass without metastases
Wilms tumour predisposition syndromes
- WAGR - wilms tumour, aniridia, genitourinary abnormalities, mental retardation
- BWS - beckwith wiedeman syndrome
Denys d rash syndrome
Perlman syndrome
Franconi anaemia
Mosaic variegated aneuploidy
WT1 role and which cancer its alteration associated with
Ureteric branching, epithelial induction of metanephric mesenchyme
Wilms tumour
What cells does Wilms tumours develop from
Pluripotent embryonic renal precursors
Which cells are usually present in a Wilms tumour
Blastema
Epithelia
Stroma
How does WT1 absence cause cancer
Prevents differentiation and keep cells in an immature state leading to Wilms tumour
Wilms tumour treatment
Surgery
Chemo
Genetic counselling