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Phase 3a > Paeds - Oncology > Flashcards

Paeds - Oncology Flashcards

1
Q

Which tumours are only usually seen in kids

A

EMBRYONAL TUMUORS (inborn changes - hence why it is so rare)

  • Wilms
  • Neuroblastoma
  • Rhabdomyosarcoma

In adults usually caused by DNA mutation caused by environmental exposures over lifetime

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2
Q

Peak incidence for bone tumour/lymphomas

A

Early adolescence / early adulthood

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3
Q

Leukaemia/Lymphoma kids vs adults

A

In kids it tends to be acute / high grade
Vice versa in adults

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4
Q

Childhood cancer aet

A
  • Idiopathic (most)
    - Double hit theory
  • Mutations in cellular genes
    - Oncogenes + Tumour suppressor genes
    - Inherited (Retinoblastoma) or sporadic
  • Increased risk due to other condition (screened earlier on)
    - Downs
    - Immunocompromise
    - Neurofibromatosis-1
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5
Q

Common presentations of cancer in kids

A
  • LOCALISED MASS
    - Lymphadenopathy
    - Organomegaly
    - Soft tissue/bony mass
  • Problems from disseminated disease
    - Bruising, tiredness, recurrent infections (from bone marrow infiltration)
  • Problems from localised mass
    - Lymphadenopathy, oedema, SOB
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6
Q

Claw sign

A

When only a little bit of kidney is visible under a big mass - on USS
- Wilm’s

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7
Q

DDx for recurrent fever + bone pain

A
  • Leukaemia (more systemic)
  • Arthritis
  • Ewing’s (bony)
  • Neuroblastoma
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8
Q

Abnormal red reflex can indicate

A

Retinoblastoma

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9
Q

Reasons for proptosis in kids

A
  • Neuroblastam
  • Infection
  • Rhabdomyosarcoma
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10
Q

Acute leukaemia Px

A
  • Fever + Fatigue
  • Bruising
  • Bone pain
  • Anaemia (chronic)
  • Lymphadenopathy
  • FREQUENT INFECTIONS
  • hepatospelenomegaly
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11
Q

ALL Ix

A
  • Blood film
  • Serum chemistry
  • CXR
  • Bone marrow aspirate (typically from hip; genetics screen)
  • Lumbar puncture (has it spread to brain)
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12
Q

ALL Tx

A

Chemotherapy (overall keep going for ~ 2yrs to ensure its all gone):

  • 2 wks high intensity ‘INDUCTION therapy’ (95% of cells killed)
  • CONSOLIDATION
  • Interim maintenance
  • Delayed intensification
  • Maintenance

If high risk / relapsed patients -> Haemopooitic stem cell transplant

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13
Q

CNS tumours Px

A
  • Headache (esp if overnight)
  • Vomiting (esp in early morning)
  • Papilloedema
  • Squint
  • Nystagmus
  • Ataxia
  • Personality / behaviour change
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14
Q

When to scan for a headache

A
  • associated papilloedema
  • associated neurological sign
  • Recurrent / in early morning
  • Associated vomiting
  • Decreasing growth
  • Sx of diabetes insipidus
  • Age < 3 yrs
  • Associated Neurofibromatosis 1
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15
Q

CNS tumour Tx

A
  • Surgery
    - Resection
    - VP shunt (to reduce risk of hydrocephalus)
  • Chemo
    - Single agent or combination
  • Radiotherapy
    - Not used in young kids
    - Typically for malignant tumours in older kids
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16
Q

DDx for lymphadenopathy

A
  • Infection (HIV esp is chronic)
  • Autoimmune
  • Storage disorders (metabolic?)
  • Malignancy
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17
Q

When to consider lymph node biopsy

A
  • Enlarged node without clear infective cause / Persistently enlarged
  • Unusual site (e.g. supraclavicular)
  • Associated Sx
    - Fever, wt loss, hepatosplenomegaly
  • Abnormal CXR
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18
Q

Lymphoma Tx

A
  • Chemo
  • Radiotherapy
    - Hodgkin’’s in particular - to reduce bulk
  • Surgery mainly just for biopsy, not curative
  • High dose therapy for relapse (hard to treat)
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19
Q

Abdo mass

A
  • Associated pain, haematuria, constipation, HTN, weight loss
  • Do USS first
  • then CT +/- biopsy
20
Q

Neuroblastoma Tx

A

Low risk mainly in infants:

  • Surgery (may have chemo after)

High risk metastatic in older kids

  • Chemo (High dose if high-risk; other wise based on stage + biology)
  • Radiotherapy (mainly for high risk / relapsed)
21
Q

Wilm’s tumour Tx

A
  • ~6 wks of chemo
  • Surgery
    - 4 more weeks of chemo
    - Partial nephrectomy or translplant if bilateral (careful follow-up)
  • Radiotherapy (if residual abdo / pulm disease)
22
Q

Retinoblastoma aet

A
  • RB1 gene mutation
  • 40% familial
23
Q

Late complications of cancer Tx (paeds)

A
  • Endocrine (hormones for growth + development)
  • Intellectual impairment
  • Cardiac toxicity
  • Renal toxicity
  • IMPAIRED FERTILITY (consider doing fertility tx before chemo/radio)
  • Psych problems (in teens + family members)
24
Q

Wilm’s tumour epid + type of tumour

A

Mainly in kids < 5 YEARS
- peaks at 3-4 y/o

  • malig embryonic tumour
25
Q

Common conditions associated w/ Wilm’s

A
  • WAGR syndrome (Wilms’ tumour, Aniridia, Genitourinary anomalies, and mental Retardation)
  • Beckwith-Wiedemann syndrome
  • Denys-Drash syndrome
26
Q

Wilm’s Px

A
  • Palpable MASS - usually doesn’t cross midline - but sometimes bilateral
  • Abdo DISTENSION / sometimes pain
  • HAEMATURIA
  • HTN

Oft asymp till it grows too large / disruptive

27
Q

Wilm’s DDx

A
  • NEUROBLASTOMA - abdo mass which CROSSES MIDLINE +/- systemic Sx
  • Mesoblastic Nephroma (haematuria rare)
  • Renal cell carcinoma (typically in ADULTS)
28
Q

Wilm’s Ix

A
  • CT chest, abdo, pelvis (identify spread)
  • RENAL BIOPSY (definitive Dx + staging)
    • may see small, round, blue cells on histology (non-specific)
29
Q

Wilm’s Mx

A
  • SUrgical resection (typically unilateral nephrectomy)
  • Adjuvent chemo / radio

Depends on stage + hisology

Prognosis >90% 5-year survival

30
Q

Neuroblastoma

A

Paediatric cancer of NEURAL CREST cells
- typically in ADRENAL MEDULLA or ABDO SYMPATHETIC chain

  • most common cancer in kids < 1
  • uncommon > 5 y/o
31
Q

Neuroblastoma pathophys

A

Stalled migration of NEURAL CREST cells (derived from ECTODERM) to adrenal medulla / sympathetic chain
- Poorly differentiates + acquires mutations

Linked to MYCN + ALK oncogene mutations + Loss of function of tumour suppressor PHOX2B

32
Q

RFx for neuroblastomas

A

No direct identidfiable cause but more likely if child has other neurocristopathies e.g.:

  • Hirschsprung’s disease
  • Congenital Central Hypoventilation Syndrome
33
Q

Neuroblastoma Px

A

Oft non-specific:

  • Abdo DISTENSION
  • FATIGUE
  • Loss of APPETITE
    - WEIGHT LOSS
  • Sx of raised catecholamines (SWEATING, AGITATION)
  • Sx of mets ( BONE PAIN, recurrent infections)
  • Urinary incontinence if symp chain compressed
    - sometimes SOB / chest pain if in thoracic section
34
Q

Findings on examination suggestive of neuroblastoms

A

Non-specific:

  • Abdo swelling
  • Hypertension
  • Tachycardia
  • Periorbital bruising from mets
  • ‘Bluberry muffin’ purpuric rash from dermis mets
  • Evidence of bone pain / recurrent infections
35
Q

Neuroblastoma DDx

A

Basically ddx for abdo mass:

  • Cysts
  • Hyperplasia
  • Neoplasia

If only blueberry muffin rash -> consider TORCH infection / AML

36
Q

Neuroblastoma Ix

A
  • HOMOVANILLIC ACID (HVA) + VANILLYLMANDELIC ACID (VMA) in urine
    - indicates catecholine breakdown
    - raised in 90%
  • Bone marrow / skin BIOPSY if sign of mets

Imaging:

  • USS for point of care identification of lumps
  • then MRI
  • CXR if THORACIC

Definitive = MIBG scan
- iodine isotope injected
- 2 scans 24hrs apart
- tumour looks v dark on scan as iodine collects there

37
Q

2 most commonly used to stage neuroblastomas in UK

A
  • International Neuroblastoma Risk Group
  • Neuroblastoma Staging System (older - based on surgical observations)
38
Q

Neuroblastoma Mx

A
  • oft can regress to nothing / benign ganglioma in INFANTS (<18 months / no mets) so just CLOSELY monitor
  • Older kids / more aggressive = SURGERY
    • curative if L1
    • adjuvent chemo/radio if L2 (involves vital structures)
  • Consider novel immuniotherapy
    (- e.g. against neuroblast cell-surface protein GD2)
  • SUPPORTIVE CARE
    - Analgesia, antiemetics, play therapists etc
39
Q

What improves prognosis for neuroblastoma

A
  • Younger age at diagnosis
  • FEMALE
  • Lesser tumour stage at diagnosis
  • MYCN mutation absent
40
Q

Neuroblastoma complications

A
  • 40-50% with high-risk neuroblastoma -> RELAPSE
  • Opsoclonus-myoclonus ataxic syndrome (in 2-4% of cases)
    - Auto-immune disorder
    - Autoantibodies to proteins from dying neuroblasts -> CNS immune reaction esp in CEREBELLUM
    - opsiclonus = uncontrolled / irregular eye movement
    - myoclonus + ataxia
    - confusion + irritability
41
Q

Causes of retinoblastoma

A
  • HEREDITARY -> germline mutations in RB1 tumour suppressor gene
  • NON-HEREDITARY -> somatic mutations in RB1 gene

Mutations to this gene is also associated with risk of other cancers

42
Q

Retinoblastoma Px

A
  • LEUKOCORIA - white pupil
  • Deteriorating vision
  • Strabismus (squint)
  • FAILURE TO THRIVE

Eye enlargement more common in developing nations

43
Q

DDx for retinoblastoma

A
  • Congenital cataracts
  • Congenital toxoplasmosis (TORCH - chorioretinitis + visual disturbance)
  • Congenital Rubella syndrome (salt-and-pepper retinopathy)
  • Persistent hyperplastic primary vitreous (leukocoria + MICROphthalmia)
  • Retinopathy of prematurity
44
Q

Retinoblastoma Ix

A

OPHTHALMIC examination
- indirect opthalmoscopy under anaesthaesia

  • Systemic evaluation to rule out mets
  • Genetic testing for RB1 mutations
  • Imaging for Dx + staging
45
Q

Retinoblastoma Mx

A

NEEDS IMMEDIATE intervention

  • Radiotherapy / enucleation +/- Chemo

Good prognosis with Tx

46
Q
A

Phase 3a (26 decks)

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