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Paediatrics > Malignancy > Flashcards

Malignancy Flashcards

1
Q

What is the most common malignancy in children

A

Acute Lymphoblastic Leukaemia (ALL)

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2
Q

What is ALL

A

Malignancy of the Lymphoid Cells (peak age 4-7):

  • Affects B and T lymphocyte cell lines - arresting maturation and prompting uncontrolled proliferation of immature blast cells of the lymphoid line (multiplying out of control)
  • This leads to marrow failure and tissue infiltration
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3
Q

What is AML

A

Malignancy of the Granulocytes (neutro, baso, esinophils)

  • Arrests maturation and prompting uncontrolled proliferation of immature blast cells of the myeloid line (multiplying out of control)
  • This leads to marrow failure and tissue infiltration
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4
Q

What do the acute luekaemias lead to and what does this result in

A

Bone Marrow Failure and Tissue Infiltration:

  • Thrombocytopenia (bleeding)
  • Anaemia
  • Neutropenia (infection)
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5
Q

What are the signs and symptoms of Luekaemia

A

Sudden onset of symptoms and rapid progression (days to weeks)

  • Fever
  • Fatigue
  • Frequent infections
  • Lymphadenopathy
  • Hepatomegaly and/or splenomegaly
  • Anaemia
  • Bruising and petechiae
  • Bone or joint pain
  • Weight Loss and Dyspnoea

CNS involvement rare: headache, N&V, irritability, papillodoema, cranial nerve involvement (3rd,4th,6th,7th)
Intracranial/spinal mass may present as nerve compression

Testicular involvement rare - but often presents as painless testicular cancer

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6
Q

What are the investigations of Leukaemia

A
  • CXR: mediastinal mass
  • Testicular Examination - US if enlarged
  • FBC: Anaemia, Neutropenia, Thrombocytopenia, blast cells
  • Blood Film - presence of blast cells!!!
  • Bone Marrow aspiration and biopsy diagnostic:
    ALL: >20% lymphoblasts
    AML: >20% myoblasts and Auer Rods
  • Lumber Puncture: to assess CNS involvement
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7
Q

What chromosomal abnormality is seen in ALL and what does it predict

A

Philadelphia Chromosome - poor prognosis

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8
Q

What is the treatment for ALL

A

Support

Immediate IV Abx if infection due to neutropenia

Chemotherapy - 4 phases

  • Induction
  • Consolidation +/- CNS treatment
  • Intensification
  • Maintenance

Stem Cell Transplant - poor prognostic factors, high risk patients, relapsed patients

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9
Q

What is the treatment for AML

A

Support

Immediate IV Abx if infection

Intensive Chemotherapy

Stem Cell Transplant - poor prognostic factors, high risk patients, relapsed patients

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10
Q

What is the prognosis for ALL

A

80% cure

Good prognostic factors: Age 2-10, female, WCC<50 and no CNS involvement

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11
Q

How do CNS tumours present

A
  • Headache: often worse early morning/lying down
  • Vomiting: especially early morning
  • Papilloedema
  • Squint
  • Nystagmus
  • Ataxia
  • Personality or behaviour change
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12
Q

What are 3 types of CNS tumours found in children

A

Pilocytic Astrocytoma
Pontine Glioma
Medulloblastoma

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13
Q

What is the treatment for CNS tumours

A

Surgery

  • Resection
  • VP shunt

Chemotherapy (most are ineffective as don’t cross blood/brain barrier)

  • Single agent
  • Combination treatment

Radiotherapy

  • For malignant tumours in older children
  • Whole brain not used in very young can damage developing nervous system
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14
Q

What is the most common cause of Lymphodenopathy

A

Very common in childhood (up to 50%)
- Mostly due to self limiting benign cause due to bacterial/viral infection

Other causes

  • HIV infection
  • Auto immune conditions
  • Storage disorders
  • Malignancy
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15
Q

When should you biopsy lymphodenopathy

A
  • Enlarging node without clear infective cause
  • Persistently enlarged node
  • Unusual site e.g. supraclavicular
  • If have associated symptoms and signs
  • Fever, weight loss, enlarged liver/spleen
  • If CXR abnormal
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16
Q

How do you treat paediatric Lymphoma

A

Chemotherapy

  • Combination Chemo to treat NHL and HL
  • Determined by histology and stage

Radiotherapy

  • Hodgkin’s – to residual bulk disease
  • rarely used in NHL

Surgery – mainly limited to biopsy

High dose chemotherapy with stem cell transplant mainly for relapse

17
Q

How may a child with a malignant abdominal mass present

A 
Abdominal mass
Pain
Haematuria 
Constipation 
Hypertension 
Weight Loss
18
Q

What are the differentials for an abdominal mass in a child

A 
Hepatoblastoma
Wilms tumour
Neuroblastoma
Lymphoma/leukaemia
Sarcoma
Constipation
Enlarged kidneys – polycystic disease
19
Q

What are useful lx for a abdominal mass

A

US
CT
Biopsy

20
Q

What may be present on Ex and Ix of a Neuroblastoma

A
  • Child with proptosis, as result of orbital involvement from metastatic neuroblastoma
  • CT scans of head show orbital mass
  • CT scan of abdomen shows large mass crossing midline and encasing the aorta and IVC
21
Q

What may present on Ex and Ix of a Wilms Tumour

A
  • Child with distended abdomen, otherwise well. Known to have hemi-hypertrophy (risk for developing Wilms)
  • Painless, palpable abdo mass
  • CT scan of abdomen shows large tumour within kidney
  • CT scan of chest shows pulmonary metastases
22
Q

What is the treatment for Neuroblastoma

A

Surgery

  • Primary - if resectable (mild disease no further treatment)
  • Chemotherapy may be given before or after to control disease if intermediate risk

Chemotherapy
Type determined by stage and biology
High dose with HPSC (haemopoietic progenitor stem cell transplant) - high risk groups
May be given if resection not possible

Radiotherapy
Mainly for high risk group or at relapse

23
Q

What is the most common type of renal tumour in children

A

Wilms Nephroblastoma

24
Q

What Increases the risk of Wilms Tumour

A

Both two do with abnormalites on chromosome 11:

  • Beckwith - Weidmann Syndome
  • WAGR - Wilms tumor, anirida, genitourinary anomalies and retardation
25
Q

What is the treatment for Wilms Tumour

A 
Chemotherapy
Prior to surgery
Following surgery
Surgery
Nephrectomy
Partial nephrectomy if bilateral
Radiotherapy
If residual abdominal or pulmonary disease

Paediatrics (13 decks)

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