Cancer In Children Flashcards

1
Q

Why can cancer have lifelong effects

A

Tumour effects
Chemo effects
Radio effects
Brain damage

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2
Q

Malignancies originating in developing tissues and organ systems

A

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

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3
Q

What types of genetic alterations cause cancer

A

Mutations
Chromosome defects
Epigenetic changes

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4
Q

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

A

Oncogenes - gain
Tumour suppressor genes - loss

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5
Q

How are oncogenes activated

A

Murptatiom
Chromosome translocation
Gene amplification
retro viral insertion

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6
Q

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

A

Tumour suppressor genes

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7
Q

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

A

Oncogenes

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8
Q

How are tipumour suppressor genes inactivated

A

Mutation
Deletions
DNA methylation - epigenetic

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9
Q

Are oncogenes or tipumour suppressor genes dominant

A

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

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10
Q

Wilms tumour

A

Nephroblastoma - Kidney tumour most common under 5 yrs

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11
Q

Wilms tumour presentation

A

Asymptomatic posterior abdominal mass without metastases

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12
Q

Wilms tumour predisposition syndromes

A
  • WAGR - wilms tumour, aniridia, genitourinary abnormalities, mental retardation
  • BWS - beckwith wiedeman syndrome
    Denys d rash syndrome
    Perlman syndrome
    Franconi anaemia
    Mosaic variegated aneuploidy
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13
Q

WT1 role and which cancer its alteration associated with

A

Ureteric branching, epithelial induction of metanephric mesenchyme
Wilms tumour

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14
Q

What cells does Wilms tumours develop from

A

Pluripotent embryonic renal precursors

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15
Q

Which cells are usually present in a Wilms tumour

A

Blastema
Epithelia
Stroma

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16
Q

How does WT1 absence cause cancer

A

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

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17
Q

Wilms tumour treatment

A

Surgery
Chemo
Genetic counselling

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18
Q

Retinoblastoma

A

Tumour of retina

19
Q

Retinoblastoma symptoms

A

Leukocoria
Eye pain
Eye redness
Vision problems

20
Q

What ages are Wilms tumours and retinoblastoma most common in

A

Under 5s

21
Q

Leukocoria

A

White reflection on pupil when light shone into eye

22
Q

What cells does retinoblastoma originate from

A

Cone precursor cells

23
Q

RB1 protein role in cell cycle

A

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

24
Q

What is the main genetic change causing retinoblastoma

A

loss of both RB1 alleles

25
Q

Retinoblastoma treatment

A

Cryotherapy
Laser therapy
Thermotherapy
Chemo
Radio
Surgery

26
Q

Neuroblastoma

A

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

27
Q

When is Neuroblastoma most common

A

1st yr

28
Q

How many cases of Neuroblastoma metastasise

A

> 1/2
4s to liver and skin

29
Q

What cells does Neuroblastoma originate in

A

Sympatho adrenal lineage of the neural crest

30
Q

Key genes in Neuroblastoma development

A

MYCN
ALK
PHOX2B

31
Q

Neuroblastoma High risk molecular abnormalities

A

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

32
Q

Neuroblastoma treatment

A

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

33
Q

Chemo related complications

A

Hearing loss
Infertility
Cardiac toxicity
Second malignancies

34
Q

Most common malignancy in children

A

Acute lymphoblastic leukaemia

35
Q

Acute lymphoblastic leukaemia presentation

A

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

36
Q

Most common areas of metastases of acute lymphoblastic leukaemia

A

Liver
Spleen
Lymph nodes
Mediastinum

37
Q

Types of acute lymphoblastic leukaemia

A

Pro B cell ALL
Pre B cell ALL
T cell ALL

38
Q

Acute lymphoblastic leukaemia origin

A

Lymphoblast Haematopoiesis

39
Q

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

A

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

40
Q

How are ALL patients separated into risk groups

A

Clinical features
Biological features
Genetic features

41
Q

ALL treatment

A

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

42
Q

What is consolidation treatment for ALL targeted at

A

CNS

43
Q

Predispositions to childhood cancer

A

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

44
Q

Genetic predisposition syndromes for childhood cancers

A

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