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
Common conditions associated w/ Wilm's
- WAGR syndrome (Wilms' tumour, Aniridia, Genitourinary anomalies, and mental Retardation) - Beckwith-Wiedemann syndrome - Denys-Drash syndrome
26
Wilm's Px
- 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
Wilm's DDx
- NEUROBLASTOMA - abdo mass which CROSSES MIDLINE +/- systemic Sx - Mesoblastic Nephroma (haematuria rare) - Renal cell carcinoma (typically in ADULTS)
28
Wilm's Ix
- CT chest, abdo, pelvis (identify spread) - RENAL BIOPSY (definitive Dx + staging) - may see small, round, blue cells on histology (non-specific)
29
Wilm's Mx
- SUrgical resection (typically unilateral nephrectomy) - Adjuvent chemo / radio Depends on stage + hisology Prognosis >90% 5-year survival
30
Neuroblastoma
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
Neuroblastoma pathophys
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
RFx for neuroblastomas
No direct identidfiable cause but more likely if child has other neurocristopathies e.g.: - Hirschsprung's disease - Congenital Central Hypoventilation Syndrome
33
Neuroblastoma Px
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
Findings on examination suggestive of neuroblastoms
Non-specific: - Abdo swelling - Hypertension - Tachycardia - Periorbital bruising from mets - 'Bluberry muffin' purpuric rash from dermis mets - Evidence of bone pain / recurrent infections
35
Neuroblastoma DDx
Basically ddx for abdo mass: - Cysts - Hyperplasia - Neoplasia If only blueberry muffin rash -> consider TORCH infection / AML
36
Neuroblastoma Ix
- 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
2 most commonly used to stage neuroblastomas in UK
- International Neuroblastoma Risk Group - Neuroblastoma Staging System (older - based on surgical observations)
38
Neuroblastoma Mx
- 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
What improves prognosis for neuroblastoma
- Younger age at diagnosis - FEMALE - Lesser tumour stage at diagnosis - **MYCN mutation absent**
40
Neuroblastoma complications
- 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
Causes of retinoblastoma
- 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
Retinoblastoma Px
- **LEUKOCORIA - white pupil** - Deteriorating vision - Strabismus (squint) - FAILURE TO THRIVE Eye enlargement more common in developing nations
43
DDx for retinoblastoma
- 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
Retinoblastoma Ix
OPHTHALMIC examination - **indirect opthalmoscopy under anaesthaesia** - Systemic evaluation to rule out mets - Genetic testing for RB1 mutations - Imaging for Dx + staging
45
Retinoblastoma Mx
NEEDS IMMEDIATE intervention - Radiotherapy / enucleation +/- Chemo Good prognosis with Tx
46