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Year 4- Paediatrics > Oncology > Flashcards

Oncology Flashcards

1
Q

Describe the commonest haematological malignancy in childhood

  • Name and subtypes
  • Common genetic abboration
  • Typical patient characteristics
  • Proportion of childhood cancers
A

Acute lymphoblastic leukaemia (ALL)
- Particularly B cell lineage (B-ALL): 75% of cases

Causes:

  • most commonly t(12;21)
  • Ph+ t(9,22)(poor prognostic marker)

Age: Bimodal peak- 2.5years old and 50 year olds
Race: White
Gender: 58% males

Accounts for 30% of childhood cancer cases

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2
Q

Which factors increase risk of ALL?

A
  1. FHx
  2. Down syndrome +++ (20x)
  3. Previous exposure to chemotherapy, benzenes, radiation
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3
Q

State the three types of leukaemias seen in children

A
  1. ALL (80%) of cases

Remaining 20% is AML and ANLL (Acute non-lymphoid leukaemia)

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4
Q

How would a child present with ALL?

A

Cancer signs: pallor, anorexic, fatigued, lethargic, loss of appetite

Bone marrow invasion -->
Thrombocytopenia: easy bruising, prolonged bleeding, epistaxis, mucotanenous bleeding (petechiae)
Anaemia: pallor, tiredness, flow murmur
Neutropenia: reccurent infections
Bone pain

Reticulo-endothelial infilitation–>
Hepatosplenomegaly, parotid infltration, testicular enlargement, thymus enlargement–> DYSPNOEA

Metastasis–>
Meningism, headache, vomiting, cranial nerve palsies
Lymphadenopathy

Overall insidious onset

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5
Q

What investigations would you do on a query leukaemia patient?

What would you expect to find?

A

FBC: pancytopenia/ leukocytosis
Coagulation screen: 10% have DIC
Blood film: pancytopenia, few blasts, normocytic anaemia
Bone marrow aspiration: >20% lymphoblasts on smear

Flow cytometry:
B-ALL: Tdt, CD10+, CD19+
T-ALL: Tdt, CD2+, CD8+

Other tests:

  • LP if worried of CNS involvement
  • CXR: mediastinal mass (T-ALL)
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6
Q

Name 5 poor prognostic features for ALL

A 
Age <1 or >10
High tumour burden: 
MLL rearrangement
t(4,11) or t(9,22)
Hypodiploidy
Detectable minimal residual disease (MRD) after induction therapy
Extramedullary disease
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7
Q

What are the general management principles for ALL

A
  1. Remission induction
    - First correct anaemia, treat infections and transfuse platelets
    - Steroids + chemotherapy
    - Allopurinol to protect kidneys from damage from increased cell lysis due to chemo
    - Hydration
  2. Intensification
    - High dose chemotherapy
    - Increased risk of toxicity
  3. Continuous therapy
    - Low intensity therapy for 1-3 years
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8
Q

What alternative management strategies can be used in ALL for those with refractory/relapsing disease?

A
  • High dose chemotherapy + total body irradiation
  • Bone marrow transplantation
  • CAR-T therapy (B-ALL)
  • Imatinib or other TKIs (If Ph+)
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9
Q

Describe 3 complications of chemotherapy in ALL

A
  1. Tumour lysis syndrome: hypERkalaemia, hypERphosphataemia, hypERuraemia, hypOcalcaemia
  2. Myelosuppression: eradication of RBC, WBCs and platelets leading to their diminished functions systemically e.g. neutropenic sepsis
  3. Infertility (particularly male)
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10
Q

Describe 2 complications of ALL

A

Extramedullary involvement

CNS- seizures
Cardiac- arrhythmias
Renal failure
Infertility

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11
Q

What is a Wilms tumour?

A

A nephroblastoma that arises within the kidney and distorts its internal architecture

  • commonest childhood renal tumour
  • tends to be unilateral (5% bilateral)
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12
Q

Which syndromes are associated with Wilms tumour?

A
  • WAGR (Wilms tumour, Aniridia, Genitourinary anomalies and mental retardation)
  • Denys-Drash syndrome (congential nephropathy, Wilms and disorders of sexual development in males)
  • Beckwith-Weidemann (overgrowth and cancer predisposition)
  • Hemihypertrophy
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13
Q

What is the commonest genetic aberration in Wilms?

A 
WT1 deletion (in 30% of cases)
- Tumour suppressor gene

WT2

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14
Q

Describe a patient presenting with Wilms

A

2-5 years old

Abdominal mass (+/- distension, flank pain)
Painless, microscopic haematuria

HTN, anorexia, anaemia,

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15
Q

How does palpation of a Wilms tumour differ to that of a neuroblastoma?

A

Wilms tumour: smooth palpable mass that doesnt extend past midline

Neuroblastoma: firm, irregular shaped mass that crosses the midline

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16
Q

Which investigations would be important when considering a diagnosis of Wilms?

A

Bedside: Urinalysis (haematuria), CXR
Bloods: FBC, U&E, LFTs, LDH
Imaging: Abdo USS (would see intrarenal mass with varying densities - cystic and solid)

17
Q

Outline the general principles of management for WIlms tumour

A
  • Chemotherapy
  • Delayed radical nephrectomy + biopsy for staging at this stage (risk of seeding if done earlier)
  • Staging informs subsequent management

Radiotherapy in advanced disease

18
Q

Where does Wilms commonly metastise to?

A

Lungs (do CXR)

19
Q

Does Wilms have a good prognosis?

What is a poor prognostic marker?

A

Yes- >80% survival at 5 years, still >60% with presentation of metastatic disease

Relapsing is a poor prognostic marker

20
Q

On examination of a Wilms tumour biopsy which signs would be good and bad prognostically?

A

Good- primitive glomeruli + blastema

Bad- Large mitotic figures and hyperchromatic nuclei

21
Q

State three complications associated with Wilms/treatment?

A
  • Renal failure
  • Myelosuppresion
  • Neutropenic sepsis
22
Q

NEUROBLASTOMA

  • What is it?
  • Who gets it?
  • Where does it appear?
  • What is the genetic aberration?
A
  • A sympathetic nervous system solid tumour- the commonest solid tumour in infants- that arises from neural crest cells
  • Median age 18 months
  • Most commonly arises from adrenal gland- other sights include sympathetic chain (neck-back), chest (paravertebral ganglia)
  • MYCN: gain of function oncogene
23
Q

How would you expect a neuroblastoma arising primarily from the adrenal gland to present?

A
  • Firm irregular abdo mass that crosses midline
  • abdominal distention
  • abdominal pain
  • constipation
  • hepatomegaly
24
Q

How would you expect a neuroblastoma arising primarily from the paravertebral ganglia to present?

A

Chest

  • dyspnoea
  • stridor

Spinal cord compression

  • back pain
  • reduced bowel and bladder control
  • numbness and weakness
  • ataxia
  • scoliosis
25
Q

How would you expect a neuroblastoma arising primarily from the neck to present?

A

Horners syndrome: ptosis, miosis, enopthalmos, anhydrosis

26
Q

What are the symptoms that form part of the paraneoplastic syndrome associated with neuroblastoma?

A

Chronic diarrhoea –> electrolyte disturbances

Catecholamines (–> palpitations, tachycardia, sweating, flushing)

Opsonclonus-myclonus ataxia

27
Q

What are your differentials for neuroblastoma?

A
  • Wilms tumour
  • Phaechromocytoma
  • Lymphoma
  • Osteomyelitis
28
Q

Which investigations would you do if you were concerned about a possible neuroblastoma?

A

Bedside: Urinalysis
Blood: FBC, U&E, LFTs, LDH (increased cell turnover)
Imaging: Abdo USS
Scintography: MIBG

Special tests:

  • Scintography: MIBG
  • Biopsy
  • 24hr Urine: Raised catecholamine breakdown products (homovanilic acid-HVA; vanillymandelic acid-VMA)
29
Q

Define the three risk categories of neuroblastoma

How does prognosis differ between these groups?

A

Low risk
Children with early stage (1-2) and no MYCN amplification; excellent prognosis >90%

Intermediate risk
Children with intermediate-late stage (3-4) and no MYC; >80% with surgery and chemo

High risk
Children with late stage disease/ MYC amplification; <50%

30
Q

How does management of neuroblastoma differ between the risk groups?

A

Low risks: can be observed for some time. Then surgery +/- chemo

Intermediate risk: preop and post op chemo + radiation if cancer persists

High risk: intense multimodal therapy (chemo, surgery, radiation, targeted immunotherapy, autologous BM transplantation

31
Q

Name three predisposition syndromes for neuroblastoma

A
  • Turners syndrome
  • Hirschsprung disease
  • Neurofibromatosis type 1
32
Q

Name 5 sequelae of neuroblastoma/treatment?

A
  • Renal impairment

Chemo–>
Ototoxicity, infertility, secondary malignancies, cardiotoxicity, endocrine complications

Year 4- Paediatrics (1 decks)

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