Paeds Oncology Flashcards

1
Q

ALL

Patho

A
  • Malignant disorder of lymphoid progenitor cells
  • Majority = B-cell origin
  • Some are T-cell origin
  • Lymphoid precursors proliferate –> replace normal bone marrow cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

ALL

Epidemiology

A
  • Affects boys slightly more than girls
  • 2 - 5 years
  • Most common cancer in children
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

ALL

Presentation

A
  • PANCYTOPAENIA = PALLOR, INFECTION, BLEEDING
  • Fever (w/o obvious cause)
  • Fatigue, dizziness, palps
  • Severe/unusual joint pain
  • Recurrent/severe infections
  • Early satiety (hepato/splenomegaly)
  • MEDIASTINAL LYMPHADENOPATHY (painless lumps in neck/axilla/groin
  • Dyspnoea
  • Headache/irritability/altered mental state
  • Bleeding
  • Pallor
  • Tachycardia/flow murmur
  • Petechiae
  • Abdo distension
  • Testicular enlargement
  • Gum hypertrophy
  • Cranial nerve palsys
  • Leukaemia cutis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

ALL

Associations

A
  • Ionising radiation during pregnancy
  • Downs syndrome
  • Kleinfelters
  • Noonans
  • Fanconi’s anaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

ALL

Ix

A
  • FBC - anaemia, thrombocytopaenia, neutropenia
  • Blood film = BLAST cells
  • Clotting: DIC
  • Raised lactic dehydrogenase levels
  • CXR: pneumonia/mediastinal mass
  • Liver/renal function
  • Testicular US - large
  • Bone marrow aspirate/biopsy - CONFIRMS DIAGNOSIS –> BLAST CELLS
  • LP to assess CNS involvement
  • genetic analysis and immunophenotyping (tDT)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

ALL

Tx

A
  • Radiotherapy
  • CHEMO
    o 5 stages: induction, consolidation, interim maintenance, delayed intensification, maintenance
  • Blood replacement therapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

ALL

Complications

A
  • neutropenic sepsis
  • hyperuricaemia
  • poor growth
  • cancer elsewhere
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Neuroblastoma

Patho

A
  • Arise from neural crest tissue in adrenal medulla + sympathetic nervous system
  • Cells surrounding tumour produce chemokines (CXCL12)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Neuroblastoma

Epidemiology

A

< 5 yrs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Neuroblastoma

Features

A
  • Chemokine release: fatigue, fever, weight loss, sweating
  • Location-dependent:
    o Thoracic - breathing problems
    o Neck - Horner’s syndrome
    o Spine - Neuro symptoms
    o Abdominal mass
    o Bones - base of skull –> periorbital ecchymosis (RACOON EYES)
    o Bone marrow - fatigue, easy bruising, freq infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Neuroblastoma

Ix

A
  • Urinalysis - RAISED vanillylmandelic acid (VMA) + homovanillic acid (HVA)
  • Bloods
  • CT - location + size
  • BIOPSY
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Neuroblastoma

Tx

A
  • Early + local = surgery
  • Late + mets = CHEMO, radio, surgery
  • May spontaneously resolve in YOUNG infants
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Wilm’s Tumour/Nephroblastoma

Patho

A
  • originates from embryonal renal tissue

- Triphasic nephroblastoma = blastemal, stromal and epithelial cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Wilm’s Tumour/Nephroblastoma

Genetics and chromosome
+ Epidemiology

A
  • Autosomal dominant
  • Mostly idiopathic but sometimes genes WT1 / WT2
  • Chromosome 11
  • MOST COMMON renal tumour in childhood
  • Most = < 5yrs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Wilm’s Tumour/Nephroblastoma

Associated syndromes

A

WAGR: WT1 and PAX6
- Wilms, Aniridia (absence of iris), Gonadoblastoma (GU malformation), mental Retardation

Dennys Drash syndrome: WT1
+ nephrotic syndrome
+ male pseudohermaphrotism

  • Edward’s
  • Blooms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Wilm’s Tumour/Nephroblastoma

Presentation

A
  • First 5 years of life
  • 95% = unilateral
  • Asymptomatic abdominal mass - incidental finding!
  • Haematuria
  • Hypertension
17
Q

Wilm’s Tumour/Nephroblastoma

Ix

A
  • USS
  • CT/MRI
  • FBC, U&Es
  • Urinalysis
  • AVOID RENAL BIOPSY - MAY MAKE WORSE
18
Q

Wilm’s Tumour/Nephroblastoma

Tx

A
  • Nephrectomy preceded by chemo
  • Radiotherapy for advanced
  • 90% = long term survival
19
Q

CNS tumours

Presentation

A
  • Raised ICP
  • Headache - worse lying down
  • Vomiting - early morn
  • Papilloedema
  • Squint
  • Nystagmus
  • Ataxia
  • Personality/behavioural change
20
Q

CNS tumours

Examples

A
  • Medulloblastoma
  • Pilocytic astrocytoma
  • Pontine glioma
21
Q

CNS tumours

Tx

A
  • Surgery: resection, VP shunt
  • Chemo: single/combination, many don’t pass BBB so not as effective
  • Radio: malignant tumours in older children, post-surgery, in combo with chemo
22
Q

CNS tumours

ix

A
  • MRI
23
Q

Retinoblastoma

Genetics + chromosome

A
  • Autosomal dominant
  • Chromosome 13
  • Average age of diagnosis = 18 months
24
Q

Retinoblastoma

Features

A
  • Absence of red reflex
  • Replaced by a white pupil
  • Visual problems
  • Strabismus
25
Q

Retinoblastoma

Tx

A
  • Enucleation is not the only option
  • External beam radiation therapy
  • Chemotherapy
  • Photocoagulation
26
Q

Retinoblastoma

Prognosis

A
  • Excellent, 90% survive into adulthood