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HD 2 > Cancer in Children > Flashcards

Cancer in Children Flashcards

1
Q

How many children under 15 in the UK are affected by cancer? Why is this important?

A

1/500 - it is the leading cause of death in this age group

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2
Q

What is the most common cancer in under 14 year olds?

A

Leukaemia

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3
Q

What is the most common cancer in 15-19 year olds?

A

Lymphomas

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4
Q

This lecture focuses on acute lymphoblastic leukaemia, Wilms’ tumour, retinoblastoma and neuroblastoma. Where do these arise? When are they usually diagnosed?

A

Developing tissues and organ systems

Before 5 years of age

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5
Q

Why is there a low mutation frequency in children’s cancer?

A

Childhood tumours arise in cells that are naturally undergoing rapid developmental growth, with fewer brakes on their proliferation than cells in adults.
These tumour precursor cells are negotiating crucial developmental checkpoints that are susceptible to corruption, leading to incomplete or abnormal cellular maturation.

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6
Q

What is the most common malignancy in children and the most frequent cause of death from cancer in <20 year age group?

A

Acute lymphoblastic leukaemia

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7
Q

What do patients with ALL present with?

A

Bruising or bleeding (thrombocytopaenia)
Pallor and fatigue (anaemia)
Infection (neutropenia)
Infiltratration to the liver, spleen, lymph nodes and mediastinum

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8
Q

How many % of ALL are CD19+, CD10+ pre-B cell ALL?

A

80%

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9
Q

What are the two main categories of blood cells?

A

Myeloid and lymphoid

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10
Q

CD19+ and CD10+ means pre-B cell. What indicates pro-B cell ALL?

A

CD19+ (ALL with MLL translocation)

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11
Q

Name some genetic alterations associated with phenotypic subtypes of ALL (4).

A

Hyperdiploidy – common in 1-15 years
TEL-AML1 – common 1-15 years, causes pre-B cell ALL
MLL – common in <1 year, causes pro-B cell ALL
BCR-ABL1 – common in 16+ years

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12
Q

What are the standard treatment phases for ALL? (4)

A
  • Induction
  • Consolidation (CNS-directed treatment)
  • Maintenance
  • Bone marrow transplantation
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13
Q

Pre-B ALL or pro-B ALL – which is more favourable?

A

Pre-B ALL

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14
Q

What is Wilms’ tumour? How many children out of 10,000 does it affect? At what age is this most common?

A

Tumour of the kidney, also called nephroblastoma
Affects 1/10,000
Under 5

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15
Q

How does Wilms’ tumour normally present?

A

Asymptomatic abdominal mass without metastasis, often bilateral

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16
Q

What syndromes can Wilms’ tumour be associated with?

A

Beckwith-Wiedeman Syndrome
Mental retardation
Genito-urinary abnormalities
Aniridia

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17
Q

What cells does Wilms’ tumour arise from?

What three cell types does it classically contain?

A

Pluripotent embryonic renal precursors

The three cell types present in the embryonic kidney: blastema, epithelia, stroma.

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18
Q

What does Wilms’ tumour closely resemble? What does it express?

A

Closely resembles developing nephrogenic mesenchyme

Expresses markers of early kidney development

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19
Q

What somatic genetic alterations are associated with Wilms’ tumour?

A
  • inactivated WT1, WTX, TP53 genes
    • activated CTNNB1 (b-catenin) gene
    • epigenetic alterations at IGF2/H19 locus
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20
Q

What germline alterations are associated with Wilms’ tumour?

A
  • WT1 gene (can be part of WAGR)

- IGF2/H19 locus (can be part of BWS)

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21
Q

What is the role of WT1?

A

WT1 has a key role in ureteric branching. WT1 and the WNT pathway (activated by b-catenin) have key roles in epithelial induction of the metanephric mesenchyme.

22
Q

How is Wilms’ tumour treated?

A

Chemotherapy (combination chemotherapy)
Surgery
Counselling if genetic predisposition is suspected

23
Q

What are the prognostic factors for Wilms’ tumour?

A

Stage, histology and age at diagnosis are prognostic factors

24
Q

When does retinoblastoma usually occur?

How many % of tumours in this age group does this account for?

A

Children under 5 years

~5%

25
Q

Where in the world does retinoblastoma appear to be more prevalent?

A

Sub-Saharan Africa

26
Q

In how many % of cases is retinoblastoma heritable?

A

30%

27
Q

What mutation is retinoblastoma associated with?

A

Germline mutation of RB1 gene - loss of RB1.

28
Q

What is leukocoria? What is it a symptom of?

A

White pupil when light shone onto it

Retinoblastoma

29
Q

What are some other symptoms of retinoblastoma?

A

Eye pain or redness

Vision problems

30
Q

How many % of patients with retinoblastoma have metastatic disease?

A

10-15%

31
Q

What cells does retinoblastoma arise from?

A

Cone precursor cells

32
Q

Why is phosphorylation of RB1 important?

A

The RB1 protein plays a critical role in the cell cycle.
Phosphorylation of RB1 releases E2F, inducing G1-S transition. Cancer cells without RB1 - E2F is free to induce G1-S transition.

33
Q

What is the molecular pathology of retinoblastoma?

A

Whole genome sequencing shows very few genetic changes.
Loss of RB1
MYCN activation
MDM2 or MDM4 amplification - leads to inactivation of p53 pathway.

34
Q

How are small retinoblastomas treated?

A

Cryotherapy, laser therapy or thermotherapy

35
Q

How are more advanced retinoblastomas treated?

A

Chemotherapy, surgery and/or radiation

36
Q

What can be used to shrink tumours before cryotherapy or laser therapy?

A

Systemic or intraocular chemotherapy

37
Q

What is neuroblastoma? Where does it usually arise?

A

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

38
Q

What is the most common cancer in the first year of life?

A

Neuroblastoma

39
Q

In how many cases of neuroblastoma is there a family history?

A

1-2%

40
Q

How many % of cases of neuroblastoma have metastatic disease at diagnosis? How does it spread?

A

> 50%

Via lymphatics and blood stream

41
Q

What are prognostic factors for neuroblastoma?

A

Stage, age, MYCN amplification, DNA ploidy, histopathology

42
Q

What is neuroblastoma 4S?

A

It presents in infants - metastatic disease to liver and skin, spontaneous maturation and regression without cytotoxic therapy.

43
Q

What are the cellular origins of neuroblastoma?

A

Derived from the sympatho-adrenal lineage of the neural crest during development. Believed to originate from an incompletely committed precursor cell.

44
Q

What are the key genes in development and neuroblastoma? (3)

A

MYCN
ALK
PHOX2B

45
Q

What is the molecular pathology of neuroblastoma - high risk? (3)

A
  • MYCN amplification; ATRX, ALK mutations
  • Near-diploid/near-tetraploid karyotype, complex chromosome aberrations
  • Deletions in 1p and 11q
46
Q

What is the molecular pathology of neuroblastoma - low risk? (3)

A
  • Numerical chromosome gains
  • Hereditary
  • Germline ALK mutations
47
Q

How is neuroblastoma treated? (3)

A

Surgery, chemotherapy, radiation therapy

48
Q

How is high risk disease neuroblastoma treated? (2)

A

High-dose chemotherapy

Stem cell transplantation

49
Q

What are the chemotherapy-related complications? (4)

A

Hearing loss
Infertility
Cardiac toxicity
Second malignancies

50
Q

What targeted therapies are there for neuroblastoma? (2)

A

Crizotinib against ALK mutations

Immunotherapy

51
Q

What does a tumour diagnosed in the perinatal period suggest?

A

A genetic predisposition syndrome

HD 2 (25 decks)

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