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Human Development 2 > Cancer in Children > Flashcards

Cancer in Children Flashcards

1
Q

Describe the epidemiology of cancers in children

A
  • Cancer affects 1/500 children under the age of 15
  • Leading course of death in this age group
  • Distinct spectrum of malignancies at different ages
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2
Q

what do certain childhood magnifies reflect

A
  • Certain childhood malignancies reflect abnormal processes of embryonic development
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3
Q

some genetic disorders can..

A
  • Some genetic disorders predispose to childhood cancer
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4
Q 
describe the percentage changes in 
- leukaemia 
- lymphomas 
- carcianoma
 - renal tumours 
- neuroblastoma 
in those aged under 14 to those aged 15-19
A
  • Leukaemia is 31.1% in 0-14 years but goes down to 13.8% in 15-19 years
  • Lymphomas are 10% in under 14s but 20% in 15-19 year olds
  • Carcinomas are 4% in less than 14 years but increase in 19.6% for 15-19 year olds
  • Renal tumours decrease as you get older
  • Neuroblastoma decrease as you get older as well
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5
Q

What problems can cancers affect later on

A 
Growth and development 
Psychosical 
Organ function 
Cacner 
Fertility and reproduction
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6
Q

describe the different problems that cancer can cause later on

A 
Growth and development 
- skeletal maturation 
- linear growth 
•  Emotional & social maturation
•  Intellectual function
•  Sexual development
Psychosical 
•  Mental health
•  Education
•  Employment
•  Health insurance
•  Chronic symptoms
•  Physical/body imag
Organ function 
•  Cardiac
•  Endocrine
•  GI & hepatic
•  Genitourinary
•  Musculoskeletal
 •  Neurological
•  Pulmonary

Cancer
• Recurrent primary cancer
• Subsequent neoplasms

Fertility and reproduction
• Fertility
• Health of offspring
• Sexual functioning

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7
Q

Childhood cancers have fewer..

A

mutations than adults tumours

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8
Q

what is the most common cancers in 0-14 year olds

A
  • Leukaemia (31.1%),
  • CNS (25.4%),
  • Lymphomas (10%).
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9
Q

what is the most common cancer in 15-19 year olds

A
  • Lymphomas (20.7%),
  • Carcinomas and Melanoma (19.6%)
  • CNS (18.7%)
  • Leukaemia (13.8%).
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10
Q

why is chemotherapy especially toxic in children

A

Chemotherapy: especially toxic in children because they have many dividing cells.
Radiation: can damage growing cells and lead to the development of other tumours.

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11
Q

what do tumours in children arise from

A
  • they arise in cells that are naturally undergoing rapid developmental growth that have fewer breaks on their proliferation than cells in adults
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12
Q

what leads to altered gene expression

A
  • cancer can arise from accumulation of genetic aberrations in somatic cells
  • these aberrations consist of mutations and chromosome defects
  • epigenetic aberrations are also present
  • these all lead to altered gene expression
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13
Q

How are oncogenes activated

A

by gain of function

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14
Q

in order for an oncogene to work how many Alleles need to be activated

A
  • Dominant (activation of one allele sufficient to have an effect)
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15
Q

what is an oncogene

A

• Gene that encodes protein capable of inducing cancer

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16
Q

what causes an gain of function in an oncogene

A
  • Mutation
  • Chromosome translocation
  • Gene amplification
  • Retroviral insertion
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17
Q

list examples of oncogenes

A
  • KRAS
  • NRAS
  • BRAF
  • ALK
  • ABL1
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18
Q

what causes activation of a tumour suppressor gene

A

loss of function

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19
Q

how many alleles need to be present for tumour suppressor genes to be activated

A
  • Act when there is inactivation of both alleles necessary (recession)
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20
Q

what causes loss of function in tumour suppressor genes

A
  • Mutations
  • Deletions
    • DNA methylation (epigentic)
      These can cause predisposition to cancer
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21
Q

list examples of tumour suppressor genes

A
  • RB
  • WT1
  • BRCA1
  • BRCA2
  • APC
  • NF1
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22
Q

describe the two hit model

A
  • two copies of a gene need to be mutated in order for cancer to develop
  • you can already have an fault mutation but the other gene works properly
  • then there needs another insult to take place for cancer to be developed
  • therefore there is no functional gene lift
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23
Q

what is wilms tumour also called

A

nephoblastoma

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24
Q

describe how a wilms tumour presents

A
  • it is often asymptomatic but the abdominal mass can be felt without metastasis
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25
Q

How does a wilms tumour spread

A
  • Spreads by growth, or via lymphatics or blood stream
26
Q

is wilms tumour heritable

A

• Heritable in 1/20 of patients.

o Therefore, often bilateral.

27
Q

if you have wilms tumour what syndromes are you predisposed to

A

WAGR

  • Wilms tumour
  • Aniridia
  • Genito-urinary abnormalities
  • Mental retardation

Can also have
- Beckwith-wiederman syndrome (BWS)

28
Q

what are the cellular origins of wilms tumour

A

• Arises from the pluripotent embryonic renal precursors.
• Classically contains three cell types present in the embryonic kidney:
o Blasterma.
o Epithelia.
o Stroma.

29
Q

what does wilms tumour closely resembl e

A
  • Closely resembles developing nephrogenic mesenchyme
30
Q

what are the somatic and germ like alternations you can have with wilms tumour

A

Somatic genetic alternations:

  • Inactivated WT1, WTX, TP53 genes
  • Activated CTNNB1 gene
  • Epigenetic alterations at IGF2/H19 locus

Germline alterations

  • WT1 gene (CAN BE PART OF wagr)
  • IGF2/H19 (can be part of BWS)
31
Q

why is the WT1 gene linked to kidney tumours (wilms tumour)

A
  • WT1 plays a crucial role in ureteric branching
  • WT1 and the WNT pathway (activated by beta catenin) have key roles in epithelial induction of the metanephric mesenchyme
32
Q

what is the treatment of wilms tumour

A

• Surgery then chemotherapy (or other way around).
o Combination chemotherapy shows promising results.
• Use of radiotherapy is decreasing.
- genetic counselling is used if genetic predisposition is suspected

33
Q

what age group does retinoblastoma occur in

A

• Tumour of the retina.

o Usually occurs in children <5

34
Q

what gene is important in a mutation in retinoblastoma

A
  • Germline mutation of RB1 gene
35
Q

is retinoblastoma heritable

A

• Heritability in ~30% of cases.

36
Q

what are the symptoms to retinoblastoma

A
  • Leukocoria – white pupil when light is shone onto it
  • Eye pain
  • Redness
  • Vision problems
37
Q

describe the cellular origins of the retinoblastoma

A
  • Originates from cone precursor cells.

* Signalling pathways promotes cell survival after loss of RB1.

38
Q

where does the retinoblastoma grow

A
  • Tumour groups in the vitreous humour of the eye
39
Q

describe what the RB1 usually does

A
  • During the G1 phase the RB1 is either hyperphosphorylated or unphosphorylated
  • In the unphosphorylated state RB1 binds to E2F and the cell cannot progress to the G1-S phase
  • When the RB1 becomes phosphorylated it releases E2F
  • E2F drives S phase
40
Q

how does RB1 cause cancer

A

Cancer Cells without RB1: if you don’t have RB1: E2F is free to induce G1-S transition.

Activation of MYCN: proliferation.

41
Q

How do you treat retinoblastoma

A
  • small tumours = cryotherapy, laser therapy or thermotherapy is used
  • in more advanced tumours - chemotherapy, surgery and radiation is used
  • Systemic or intraocular chemotherapy can be used to shrink tumours before cryotherapy or laser therapy
42
Q

what is a neuroblastoma

A

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

43
Q

what age is a neuroblastoma commonly found in

A

• Most common cancer in the first year of life

44
Q

how does the neuroblastoma spread

A
  • via lymphatics and blood stream
45
Q

what are the prognostic factors of a neuroblastoma

A

stage, age, MYCN amplification, DNA ploidy, histopathology

46
Q

where does neuroblastoma metastasise to

A

metastatic disease to liver and skin

47
Q

what are the cellular origins of a neuroblastoma

A

• Derived from sympatho-adrenal linage of the neural crest during development.
o Originates from incompletely committed precursor cell.
• Key genes: MYCN, ALK.

48
Q

what are two common genes to do with a genetic predisposition to neuroblastoma

A
  • Some are very rare, also common alleles which have some predispotion
  • Two which are commonly known are ALK and PHOX2B
49
Q

what can cause a high risk, low risk and hereditary cause of neuroblastoma

A

High Risk
• MYCN amplification (transcription factor), ATRX, ALK mutations.
o Near diploid/tetraploid karyotype.

Low Risk/Intermediate-Risk/Stage 4S
• Numerical chromosome gains.

Hereditary
• Germline ALK mutations.

50
Q

how do you calculate a prognosis for neuroblastoma

A
  • less than 10 copies of NMYC high prognosis, more than 10 copies of N-MYC poor prognosis
51
Q

what is the treatment for a neuroblastoma

A
  • Surgery, chemotherapy, radiation therapy
  • High risk disease – high dose chemotherapy and stem cell transplantation
  • Targeted therapy – crizotinib against ALK mutations
  • Immunotherapy
52
Q

what are chemotherapy related complications

A

hearing loss, infertility, cardiac toxicity and second malignancies

53
Q

what is the most common malignancy in children

A

Acute lymphoblastic leukaemia

54
Q

How does the patient present with acute lymphoblastic leukaemia

A
  • Bruising/bleeding.
  • Pallor or fatigue due to anaemia.
  • Infection due to neutropenia.
55
Q

where does acute lymphoblastic leukaemia spread

A

liver, spleen, lymph nodes and mediastinum

56
Q

what does acute lymphoblastic leukaemia cause

A

Clonal expansion of immature lymphocytes = lymphoblasts/blast cells

57
Q

What are the cellular origins in acute lymphoblastic leukaemia

A

• Traced back to haematopoiesis.

Pro-B: CD19+ on the cell surface.
Pre-B: CD19+ and CD10+.

These have very specific genetic changes that cause different types of leukaemia’s.
• ALL: MLL translocation (19)
• TEL-AML1 translocation (19 and 10).

58
Q

what is the molecular pathology of acute lymphoblastic luekamiea

A
  • Specific genetic alterations are associated with phenotypic subtypes of ALL
  • Incidence of the subtypes varies with age
  • Certain genetic alterations have been found in newborn babies who contract leukaemia a few years later
59
Q

What is the treatment for acute lymphoblastic leukaemia

A

• Patients stratified into risk groups according to clinical, biological and genetic features
• Therapies of varying intensities applied to different risk groups
• Standard treatment phases:
-Induction (e.g.Vincristine, Corticosteroid, L-asparaginase, Anthracycline)
- Consolidation; CNS directed treatment(e.g.Cyclophosphamide, Cytarabine, Mercaptopurine, Methotrexate; Dexamethasone)
- Maintenance(e.g. Mercaptopurine, Methotrexate) - Bone marrow transplantation

60
Q

describe the role of the two step model for the role in infection in acute lymphoblastic leukaemia

A
  • Inherited genetic background
  • Convert pre-leukaemia
  • Massive expansion of the immune system
  • Accumulation of the B cell
61
Q

what are high risks groups for cancer

A
  • Any tumour diagnosed in the perinatal period suggests a genetic predisposition syndrome.
  • Bilateral or multifocal disease, associated with congenital malformations.
  • Cancer in close relatives.

• Same rare tumour in more than one family member,
o e.g. familial Retinoblastoma

• Different types of tumours occurring in family members
o e.g. Li-Fraumeni syndrome

Human Development 2 (34 decks)

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