Cancer In Children

Question 1

Why can cancer have lifelong effects

Answer 1

Tumour effects
Chemo effects
Radio effects
Brain damage

Question 2

Malignancies originating in developing tissues and organ systems

Answer 2

Wilms tumour
Retinoblastoma
Neuroblastoma
Acute lymphoblastic leukaemia
Medulloblastoma
Hepatoblastoma
Rhabdomyosarcoma
Germ cell tumours

Question 3

What types of genetic alterations cause cancer

Answer 3

Mutations
Chromosome defects
Epigenetic changes

Question 4

Do oncogenes and tumour suppressor genes cause cancer by gain or loss of function

Answer 4

Oncogenes - gain
Tumour suppressor genes - loss

Question 5

How are oncogenes activated

Answer 5

Murptatiom
Chromosome translocation
Gene amplification
retro viral insertion

Question 6

What type of genes are RB, WT1, TP53, PTCH1, BRCA1, BRCA2, APC, NF1

Answer 6

Tumour suppressor genes

Question 7

What type of genes are MYCN, MDM2, ALK, ABL1, HRAS, KRAS, NRAS, BRAF

Answer 7

Oncogenes

Question 8

How are tipumour suppressor genes inactivated

Answer 8

Mutation
Deletions
DNA methylation - epigenetic

Question 9

Are oncogenes or tipumour suppressor genes dominant

Answer 9

Oncogenes - dominant - activation of 1 allele sufficient
Tumour suppressor genes - recessive - inactivation of both genes necessary

Question 10

Wilms tumour

Answer 10

Nephroblastoma - Kidney tumour most common under 5 yrs

Question 11

Wilms tumour presentation

Answer 11

Asymptomatic posterior abdominal mass without metastases

Question 12

Wilms tumour predisposition syndromes

Answer 12

• WAGR - wilms tumour, aniridia, genitourinary abnormalities, mental retardation
• BWS - beckwith wiedeman syndrome
  Denys d rash syndrome
  Perlman syndrome
  Franconi anaemia
  Mosaic variegated aneuploidy

Question 13

WT1 role and which cancer its alteration associated with

Answer 13

Ureteric branching, epithelial induction of metanephric mesenchyme
Wilms tumour

Question 14

What cells does Wilms tumours develop from

Answer 14

Pluripotent embryonic renal precursors

Question 15

Which cells are usually present in a Wilms tumour

Answer 15

Blastema
Epithelia
Stroma

Question 16

How does WT1 absence cause cancer

Answer 16

Prevents differentiation and keep cells in an immature state leading to Wilms tumour

Question 17

Wilms tumour treatment

Answer 17

Surgery
Chemo
Genetic counselling

Question 18

Retinoblastoma

Answer 18

Tumour of retina

Question 19

Retinoblastoma symptoms

Answer 19

Leukocoria
Eye pain
Eye redness
Vision problems

Question 20

What ages are Wilms tumours and retinoblastoma most common in

Answer 20

Under 5s

Question 21

Leukocoria

Answer 21

White reflection on pupil when light shone into eye

Question 22

What cells does retinoblastoma originate from

Answer 22

Cone precursor cells

Question 23

RB1 protein role in cell cycle

Answer 23

Keeps cells at G1 by Binding to E2F when Unphosphorylated, rb phosphorylation frees E2F inducing G1-S transition

Question 24

What is the main genetic change causing retinoblastoma

Answer 24

loss of both RB1 alleles

Question 25

Retinoblastoma treatment

Answer 25

Cryotherapy Laser therapy Thermotherapy Chemo Radio Surgery

Question 26

Neuroblastoma

Answer 26

Tumour of sympathetic nervous system usually arising in adrenal gland or sympathetic ganglia

Question 27

When is Neuroblastoma most common

Answer 27

1st yr

Question 28

How many cases of Neuroblastoma metastasise

Answer 28

>1/2 4s to liver and skin

Question 29

What cells does Neuroblastoma originate in

Answer 29

Sympatho adrenal lineage of the neural crest

Question 30

Key genes in Neuroblastoma development

Answer 30

MYCN ALK PHOX2B

Question 31

Neuroblastoma High risk molecular abnormalities

Answer 31

MYCN amplification ATRX mutation ALK mutation Near-diploid/near-haploid karyotype Chromosome aberrations1p and 11q deletions

Question 32

Neuroblastoma treatment

Answer 32

Surgery Chemo Radio Stem cell transplant 4s Can spontaneously regress Crizotinib against ALK mutations Immunotherapy

Question 33

Chemo related complications

Answer 33

Hearing loss Infertility Cardiac toxicity Second malignancies

Question 34

Most common malignancy in children

Answer 34

Acute lymphoblastic leukaemia

Question 35

Acute lymphoblastic leukaemia presentation

Answer 35

Bruising - thrombocytopenia Bleeding - thrombocytopenia Pallor - anaemia Fatigue - anaemia Infection - neutropenia

Question 36

Most common areas of metastases of acute lymphoblastic leukaemia

Answer 36

Liver Spleen Lymph nodes Mediastinum

Question 37

Types of acute lymphoblastic leukaemia

Answer 37

Pro B cell ALL Pre B cell ALL T cell ALL

Question 38

Acute lymphoblastic leukaemia origin

Answer 38

Lymphoblast Haematopoiesis

Question 39

Why are both children in a pair of identical twins likely to get cancer if 1 gets it

Answer 39

Shared blood supply in utero means both can get a first hit

Question 40

How are ALL patients separated into risk groups

Answer 40

Clinical features Biological features Genetic features

Question 41

ALL treatment

Answer 41

Induction - vincristine, corticosteroid, L asparaginase, anthracycline Consolidation - targets CNS - cyclophosphamide, cytarabine, mercaptopurine, methotrexate, dexamethasone Maintainance - mercaptopurine, methotrexate Bone marrow transplant

Question 42

What is consolidation treatment for ALL targeted at

Answer 42

CNS

Question 43

Predispositions to childhood cancer

Answer 43

Cancer diagnosed in perinatal period Bilateral of multi focal disease Cancer in close relatives Same rare tumour in >1 family member Different types of tumour in family members

Question 44

Genetic predisposition syndromes for childhood cancers

Answer 44

WAGR beckwith Weideman Familial retinoblastoma Familial Neuroblastoma Basal cell Nevis Li fraumeni