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3A > paediatric oncology > Flashcards

paediatric oncology Flashcards

1
Q

What is a retinoblastoma?

A

the most common ocular malignancy in children

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2
Q

What age does retinoblastomas usually present?

A

18 months

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3
Q

Aetiology of retinoblastoma

A

autosomal dominant condition caused by a loss of the tumour suppressor gene found on chromosome 13

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4
Q

Presentation of retinoblastoma

A

absent red reflex - most common presenting symptom
strabismus
visual problems
bulging in one eye
red painful eye

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5
Q

How are retinoblastomas diagnosed?

A

fundus photography
Fluorescein angiography
Ophthalmic USS- gold standard
MRI

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6
Q

How are retinoblastomas treated?

A

initial management may involve- external beam radiation, chemotherapy and photocoagulation.

In unsuccessful may need enucleation (removal of the eye)

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7
Q

Where do neuroblastomas arise from

A

the neural crest cells of the adrenal medulla or the sympathetic nervous system

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8
Q

What is the median age of onset of neuroblastomas

A

20 months

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9
Q

how do neuroblastomas present?

A

blueberry muffin rash - from seeding metastasis in the dermis.
abdominal mass
pallor
weight loss
bone pain and limp
hepatomegaly
paraplegia
proptosis
signs of increased catecholamine secretion -sweating, agitation

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10
Q

What is the peak age of acute lymphoblastic leukaemia

A

2-3 years

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10
Q

How are neuroblastomas diagnosed?

A

raised urinary vanillymandelic acid (VMA) and homovanillic acid (HVA) levels

USS or MRI

calcifications seen on X ray

Definitive test= MIBG scan (radioactive isotope injected and 2 scans taken 24 hours apart- iodine will stay in tumour)

Gold= biopsy

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10
Q

What is the most common leukaemia in children

A

acute lymphoblastic leukaemia

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11
Q

What leukaemias are common in children

A

acute lymphoblastic
acute myeloid
chronic myeloid leukaemia

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12
Q

risk factors for leukaemia

A

radiation exposure during pregnancy
Down’s syndrome
Kleinfelter’s syndrome
Noonan syndrome
Fanconi anaemia

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13
Q

pathophysiology of leukaemia

A

A cancer of the bone marrow- a genetic mutation leads to excessive production of a single blood cell
Leads to supression of all the other types of blood cells- pancytopenia

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14
Q

How does leukaemia present?

A

general symptoms- anorexia, malaise, fever
bone marrow infiltration symptoms- pancytopenia (anaemia- lethargy, pallor; neutropenia- infection; thrombocytopenia - bruising, petechiae, epistaxis)
bone pain
reticulo-endothelial infiltration symptoms- hepatosplenomegaly, lymphadenopathy, superior mediastinal obstruction
symptoms of other organ infiltration - CNS, testes, mediastinal mass

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15
Q

differentials for anaemia

A

viral infection
EBV infection
parvovirus
ITP,
aplastic anaemia

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16
Q

how is leukaemia diagnosed

A

FBC- pancocytopenia
blood film- blast cells
bone marrow biopsy
lymph node biopsy

CT, chest x ray

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17
Q

what is the most common malignant bone tumour in children

A

osteosarcoma

18
Q

two types of paediatric bone tumours

A

osteosarcoma and ewings sarcoma

19
Q

What is wilms tumour

A

a type of kidney tumour that typically affects children under 5

20
Q

what age group is most commonly affected by wilms tumours

A

children under 5- mean age is 3

21
Q

what genetic conditions are associated with wilms tumours (particularly if bilateral)

A

WAGR syndrome
Beckwith- Wiedemann syndrome
hemihypertrophy

22
Q

How does wilms tumour present?

A

abdominal mass
painless haematuria
flank pain = rare
anorexia
fever
weight loss

23
Q

Where does wilms tumour most commonly metastasise?

A

lung

24
Q

what genetic mutation is associated with wilms tumour

A

loss of function of the WT1 gene on chromosome 11

25
Q

Diagnosis of wilms tumour

A

1st line- USS abdo
gold- biopsy

26
Q

How quickly should children presenting with an unexplained abdominal mass be reviewed in paediatrics

A

within 48 hours

27
Q

how is wilms tumour treated?

A

nephrectomy
chemotherapy
radiotherapy if advanced

28
Q

what is the prognosis of wilms tumour

A

80% cure

29
Q

what two bone tumours are most common in children?

A

osteosarcoma (most common)
Ewings sarcoma

30
Q

what age group is typically affected by osteosarcomas?

A

aged 10-20

31
Q

what mutation is associated with osteosarcomas

A

a mutation in the Rb gene

32
Q

what conditions increase the risk of osteosarcomas?

A

Paget’s disease
radiotherapy
Li-Fraumeni syndrome

33
Q

what mutation is associated with Ewing’s sarcoma

A

t(11:22) translocation which results in a EWS-FLI1 gene product

34
Q

where in the bone do most osteosarcomas occur

A

in the metaphyseal region of long bones before closure of the epiphyseal plate

35
Q

what bones are most commonly affected in osteosarcomas?

A

femur - 40%
Tibia- 20%
Humerus - 10%

36
Q

what cells are osteosarcomas derived from

A

the primitive transformed cells of mesenchymal origin

37
Q

What bones do Ewing’s sarcomas commonly effect?

A

pelvis and long bones

38
Q

What type of tumour is Ewing’s sarcoma

A

neuroectodermal tumour

39
Q

How doe bone tumours in children present?

A

persistent bone pain- often worse at night and can wake them up from sleep

May have bone swelling, a palpable mass and restricted joint movement

40
Q

How are bone tumours diagnosed

A

X ray- NICE recommends within 48 hours of child presenting with unexplained bone pain or swelling
Bloods- raised ALP
CT/MRI for staging

Bone biopsy is definitive

41
Q

How does a osteosarcoma present on X ray

A

Codman triangle and a ‘sunburst’ pattern
May show a poorly defined lesion in the bone with fluffy appearance

42
Q

How does Ewing’s sarcoma present on X ray

A

onion skin appearance

43
Q
A

3A (33 decks)

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