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Paeds: Acute Presentation > Childhood cancers > Flashcards

Childhood cancers Flashcards

1
Q

What are 9 types of cancers in children to be aware of?

A
  1. Leukaemias: ALL, AML, ANLL
  2. Brain tumours: astrocytoma, medulloblastoma, ependymoma, brainstem glioma, craniopharyngioma, atypical teratoid/rhabdoid tumour
  3. Retinoblastoma
  4. Neuroblastoma
  5. Lymphomas: Hodgkin, Non-Hodgkin, Burtkitt
  6. Wilms tumour (nephroblastoma)
  7. Soft tissue sarcomas
  8. Bone tumours
  9. Kaposi sarcoma
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2
Q

What are 3 ways that a child may present with cancer?

A
  1. Localised mass
  2. Consequences of disseminated disease e.g. bone marrow infiltratio, causing systemic ill-health
  3. Consequences of pressure from a mass on local structures or tissue, e.g. airway obstruction secondary to enlarged lymph nodes in the mediastinum
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3
Q

What is the commonest type of leukaemia in children?

A

Acute lymphoblastic leukaemia (ALL) - 80%

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4
Q

What 2 types of leukaemia make up most of the remainder of leukaemia after acute lymphoblastic leukaemia (ALL)?

A
  1. Acute myeloid leukaemia (AML)
  2. Acute nonlymphocytic leukaemia (ANLL)
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5
Q

What is the peak age of presentation of acute lymphoblastic leukaemia?

A

2-5 years

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6
Q

What causes clinical symptoms and signs in acute lymphoblastic leukaemia?

A

disseminated disease and systemic ill-health from infiltration of the bone marrow or other organs with luekaemic blast cells

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7
Q

How does acute lymphoblastic luekaemia present over time?

A

usually insidiously over several weeks, but in some children presents and progresses very rapidly

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8
Q

What are 3 effects of bone marrow infiltration in acute lymphoblastic leukaemia?

A
  1. Anaemia - pallor, lethargy
  2. Neutropenia - infection
  3. Thrombocytopenia - bruising, petechiae, nose bleeds
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9
Q

What are 3 clinical features of acute lymphoblastic leukaemia due to reticulo-endothelial infiltration?

A
  1. Hepatosplenomegaly
  2. Lymphadenopathy
  3. Superior mediastinal obstruction
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10
Q

What are 2 organs in addition to bone marrow the reticulo-endothelium that ALL can infiltrate and the symptoms it produces as a result?

A
  1. Central nervous system: headaches, vomiting, nerve palsies
  2. Testes: testicular enlargement
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11
Q

Overall what are 16 possible symptoms of acute lymphoblastic leukaemia?

A
  1. Malaise
  2. Anorexia
  3. Pallor
  4. Lethargy
  5. Infection
  6. Bruising
  7. Petechiae
  8. Nose bleeds
  9. Bone pain
  10. Hepatosplenomegaly
  11. Lymphadenopathy
  12. Superior mediastinal obstruction
  13. Headaches
  14. Vomiting
  15. Nerve palsies
  16. Testicular enlargement
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12
Q

What are 5 investigations that should be performed in acute lymphoblastic leukaemia?

A
  1. Full blood count
  2. Bone marrow examination - essential
  3. Clotting screen
  4. Lumbar puncture
  5. CXR
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13
Q

What are 3 things that FBC will show in leukaemia?

A
  1. Low haemoglobin
  2. Thrombocytopenia
  3. Evidence of circulating leukaemic blast cells
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14
Q

Why is it important to perform bone marrow examination e.g. bone marrow smear in leukaemia?

A

it is needed to confirm the diagnosis

needed to identify immunological and cytogenetic characteristics which give useful prognostic information

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15
Q

Why should a clotting screen be performed for leukaemia?

A

10% of patients have disseminated intravascular coagulation (DIC) at time of diagnosis - can have haemorrhagic or thrombotic complications

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16
Q

Why is a lumbar puncture performed in suspected leukaemia?

A

to identify any disease in the CSF

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17
Q

Why is a CXR performed in suspected leukaemia?

A

to identify a mediastinal mass characteristic of T-cell disease

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18
Q

What are the 2 most common subtypes of ALL and AML?

A

common (75%) and T-cell (15%)

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19
Q

What are 5 high-risk prognostic factors in acute lymphoblastic leukaemia?

A
  1. Age: <1 or >10 years
  2. Tumour load (WCC) >50 x 109 /L
  3. Cytogenetic/ molecular genetic abnormalities in tumour cells e.g. MLL rearrangement t(4;11), hypoploidy (<44 chromosomes)
  4. Speed of response to initial chemotherapy - persistence of leukaemic blasts in bone marrow
  5. Minimal residual disease (MRD) assessment (submicroscopic levels ofleukaemia detected by PCR - if detectable MRD after induction therapy, poor prognosticator
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20
Q

What are 5 aspects of the management of acute lymphoblastic leukaemia?

A
  1. Remission induction
  2. Intensification
  3. Central nervous system
  4. Continuing therapy
  5. Treatment of relapse
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21
Q

What does remission induction for ALL involve?

A

combination chemotherapy including steroids is given

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22
Q

How successful are current induction treatment schedules for ALL?

A

achieve remission rates of 95%

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23
Q

What are 4 treatments that may be performed before remission induction in ALL?

A
  1. Anaemia correction with blood transfusion
  2. Risk of bleeding minimised with transfusion of platelets
  3. Treatment of infection
  4. Renal protection against effects of rapid cell lysis: additional hydration and allopurinol (or urate oxidase if WCC high)
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24
Q

What does intensification of the treatment for ALL involve?

A

block of intensive chemotherapy given to consolidate remission

improves cure rates at expense of increased toxicity

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25
Q

What does treatment of the central nervous system in ALL involve?

A

cytotoxic drugs penetrate poorly into CNS so leukaemic cells here may survive systemic treatment

intrathecal chemotherapy used to prevent CNS relapse

if evidence of CNS disease at diagnosis, additional doses of intrathecal chemotherapy during induction

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26
Q

What does the continuing therapy for ALL involve?

A

chemotherapy of modest intensity continued over longer period, up to 3 years from diagnosis

cotrimoxazole prophylaxis given routinely to prevent Pneumocystis pneumonia

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27
Q

What prophylaxis is given during continuing therapy for ALL and why?

A

Cotrimoxazole to prevent Pneumocystis pneumonia

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28
Q

What does treatment of ALL relapse involve?

A

high dose chemotherapy with or without total body irradiation followed by bone marrow transplantation - used as alternative to conventional chemotherapy

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29
Q

Where do most brain tumours exist in children?

A

infratentorial: below tentorium cerebelli

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30
Q

What is the commonest type of solid tumour in children?

A

brain tumours

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31
Q

What are 6 types of brain tumours and which is the most common?

A
  1. Astrocytoma (40%)- commonest
  2. Medulloblastoma
  3. Epdenymoma
  4. Brainstem glioma
  5. Craniopharyngioma
  6. Atypical teratoid/rhabdoid tumour
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32
Q

What is the most malignant form of astrocytoma?

A

glioblastoma multiforme

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33
Q

Where do medulloblastomas typically occur and where can they metastasise?

A
  • arise in midline of posterior fossa
  • May seed through the CNS via the CSF and up to 20% have spinal metastases at diagnosis
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34
Q

Where do ependymomas typically occur?

A

posterior fossa (behaves like medulloblastoma)

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35
Q

What is the prognosis of brainstem gliomas?

A

they are malignant tumours with very poor prognosis

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36
Q

Where do craniopharyngiomas arise?

A

arise from the squamous remnant of the Rathke pouch

Not truly malignant but locally invasive and grow slowly in the suprasellar region

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37
Q

In which age group do typical teratoid/rhabdoid tumours most commonly ocur in?

A

young children

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38
Q

Why is the developmental age of the child important when it comes to brain tumours?

A

presentation varies according to age and ability to report symptoms

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39
Q

What are 6 possible signs of brain tumour for all types and ages?

A
  1. Persistent or recurrent vomiting
  2. Problems with balance, coordination or walking
  3. Behavioural change
  4. Abnormal eye movements
  5. Seizurs (without fever)
  6. Abnormal head position - wry neck, head tilt or persistent stiff neck
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40
Q

What are 5 signs of brain tumours in a child/adolescent?

A
  1. Persistent or recurrent ehadache
  2. Blurred or double vision
  3. Lethargy
  4. Deteriorating school performance
  5. Delayed or arrested puberty, slow growth
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41
Q

What are 3 signs of brain tumour in infants?

A
  1. Developmental delay/ regression
  2. Progressive increase in head circumference, separation of sutures, bulding fontanelle
  3. Lethargy
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42
Q

What are 3 symptoms of brain tumour specific to supratentorial/ cortex tumours?

A
  1. Seizures
  2. Hemiplegia
  3. Focal neurological signs
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43
Q

What are 2 signs of brain tumours located in the midline?

A
  1. Visual field loss: bitemporal hemianopia
  2. Pituitary failure: growth failure, diabetes insipidus, weight gain
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44
Q

What are 3 symptoms specific to brain tumours in the cerebellum and IVth ventricle?

A
  1. Truncal ataxia
  2. Coordination difficulties
  3. Abnormal eye movements
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45
Q

What are 4 signs of brainstem brain tumours?

A
  1. Cranial nerve defects
  2. Pyramidal tract signs
  3. Cerebellar signs - ataxia
  4. Often no raised intracranial pressure
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46
Q

What investigation must be performed if there is persistent back pain in a child?

A

investigation with MRI

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47
Q

What are 3 investigations to perform for a suspected brain tumour?

A
  1. MRI
  2. MR spectroscopy - to examine biological activity
  3. Lumbar puncture - some metastasise within CSF
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48
Q

What should be done before performing a lumbar puncture in suspected CSF brain tumour infiltration?

A

neurosurgical advice needed if suspicion of raised ICP

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49
Q

What is the management of brain tumours?

A
  • surgery usually first treatment: aimed at treated hydrocephalus, providing tissue diagnosis and attempting maximum resection
  • use of radiotherapy and/or chemotherapy varies with tumour type and patient age
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50
Q

What are 2 examples of brain tumours for which biopsy is not safe?

A
  1. tumour in brainstem
  2. tumour in optic pathway
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51
Q

What are 5 complex late effects of brain tumours in survivors?

A
  1. Neurological disability
  2. Growth problems
  3. Endocrine problems
  4. Neuropsyhcological problems
  5. Educational problems
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52
Q

What are 3 types of lymphomas to know?

A
  1. Hodgkin lymphoma
  2. Non-Hodgkin lymphoma
  3. Burkitt lymphoma
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53
Q

What is the commonest lymphoma in childhood?

A

Non-Hodgkin lymphoma

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54
Q

What is the commonest lymphoma in adolescence?

A

Hodgkin lymphoma

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55
Q

What are 2 aspects of the clinical features of Hodgkin lymphoma?

A
  1. Painless, large, firm lymphadenopathy: most frequently in neck
    • can cause airways obstruction
  2. Systemic symptoms: sweating, pruritus, weight loss, fever (uncommon)
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56
Q

What are 3 investigations that should be performed in Hodgkin lymphoma?

A
  1. Lymph node biopsy
  2. Radiological assessment of all nodal sites
  3. Bone marrow biopsy
57
Q

What is the management of Hodgkin lymphoma?

A

combination chemotherapy with or without radiotherapy

Positron emission tomography (PET) scanning to monitor treatment response and guide further management

58
Q

What proportion of patients with Hodgkin lymphoma can be cured?

A

80%

59
Q

What are 2 ways that T-cell malignancies can present?

A
  1. Acute lymphoblastic leukaemia
  2. Non-Hodgkin lymphoma
60
Q

What is Non-Hodgkin lymphoma?

A

T-cell malignancy characterised by a mediastinal mass with varying degrees of bone marrow infiltration

61
Q

What may occur secondary to the mediastinal mass of Non-Hodgkin lymphoma? What are 5 signs of this?

A

Superior vena cava obstruction:

  1. dyspnoea
  2. facial swelling
  3. flushing
  4. venous distention in the neck
  5. distended veins in upper chest and arms
62
Q

In addition to T-cell malignancies, what else can present as non-Hodgkin lymphoma?

A

B-cell malignancies - usually in head and neck or abdomen

63
Q

How can abdominal Non-Hodgkin lymphoma present?

A

pain from intestinal obstruction, palpable mass or intussusception in cases with involvement of ileum

64
Q

What are 4 investigations to perform in Non-Hodgkin’s lymphoma?

A
  1. Biopsy
  2. Radiological assessment of all nodal sites: CT or MRI
  3. Examination of bone marrow
  4. Lumbar puncture to examine CSF
65
Q

What is the management of Non-Hodgkin lymphoma?

A

multiagent chemotherapy

66
Q

What are the survival rates of Non-Hodgkin lymphoma?

A

over 80% for both T and B-cell disease

67
Q

What type of lymphoma shows the Owl’s Eye appearance on histology? What does this represent?

A

Hodgkin lymphoma - Reed-Sternberg cells

68
Q

What is Burkitt lymphoma?

A

type of B-cell non-Hodgkin lymphoma

69
Q

What are 3 variants of Burkitt lymphoma?

A
  1. Endemic variant: malaria-endemic regions, Epstein-Barr virus (EBV) infection is thought to be cause
  2. Sporadic variant
  3. Immunodeficiency-associated variant
70
Q

Why does the endemic variant most commonly occur in children living in malaria endemic regions of the world?

A

Epstein-Barr virus (EBV) found in nearly all these patients

chronic malaria believed to reduce resistance to EBV

71
Q

What part of the body does the endemic variant of Burkitt lymphoma characteristically involve?

A

jaw or other facial bone

72
Q

What commonly causes Burkitt lymphoma in the Western world?

A

cases are sporadic, can be associated with EBV infection still

73
Q

What are 2 things that immunodeficiency-associated Burkitt lymphoma can occur due to?

A
  1. HIV infection
  2. Immunosuppression post-transplant
74
Q

What is the management of Burkitt lymphoma?

A

Multiagent chemotherapy

75
Q

What do neuroblastomas arise from?

A

neural crest tissue in the adrenal medulla and sympathetic nervous system

76
Q

What make neuroblastoma an unusual tumour?

A

spontaneous regression sometimes occurs in very young infants

77
Q

What represents the ends of the spectrum of neuroblastoma type tumours?

A

benign ganglioneuroma to highly malignant neuroblastoma

78
Q

In what age group does neuroblastoma most commonly occur?

A

before age of 5 years

79
Q

What is the usual presentation of neuroblastoma?

A

abdominal mass

80
Q

What anatomical structures can neuroblastomas involve?

A

classically the primary is of adrenal origin but at presentation of crosses midline and envelops major blood vessels and lymph nodes

paravertebral tumours can cause spinal cord compression - emergency

81
Q

what usually causes the symptoms of neuorblastoma in patients over the age of 2 years?

A

metastatic disease: bone pain, bone marrow suppression causing weight loss, malaise

82
Q

What are 6 common symptoms of neuroblastoma?

A
  1. Pallor
  2. Weight loss
  3. Abdominal amss
  4. Hepatosplenomegaly
  5. Bone pain
  6. Limp
83
Q

What are 5 less common presenting symptoms of neuroblastoma?

A
  1. Paraplegia
  2. Cervical lymphadenopathy
  3. Proptosis
  4. Periorbital bruising
  5. Skin nodules
84
Q

What are 4 investgiations to perform for neuroblastoma?

A
  1. Urinary catecholamines: increased e.g. vanillylmandelic acid (VMA) and homovanillic acid (HVA)
  2. Confirmatory biopsy
  3. Bone marrow sample
  4. MIBG scan - to look for neural crest tumour metastasis
85
Q

What is the prognosis of neuroblastoma?

A

poor - usually present with advanced disease

86
Q

What predicts aggressive behaviour of neuroblastomas? 2 things

A
  1. amplification of the MYCN oncogene predicts aggressive behaviour
  2. evidence of deletion or gain or genetic material on part or one or more chromosomes (as compared with a change in the number of copies of whole chromosomes) associated with poorer prognosis
87
Q

What is the management of localised neuroblastoma primaries without metastatic disease?

A

often cured with surgery alone

in some infants may resolve spontanesouly (including when metastatic)

88
Q

What is the management of metastatic neuroblastoma in older children?

A

chemotherapy including high-dose thearpy with autologous stem cell rescue, surgery, and radiotherapy

89
Q

What is the risk of relapse with metastatic neuroblastoma?

A

high

90
Q

What are 2 treatment options for metastatic neuroblastoma that are being developed?

A
  1. Immunotherapy
  2. Maintenance treatment with differentiating agents (retinoic acid)
91
Q

What do Wilms tumours originate from?

A

embryonal renal tissue

92
Q

When do most children present with Wilms tumour?

A

>80% present before 5 years

rarel seen after 10 years of age

93
Q

What are 2 common features of Wilms tumours?

A
  1. Abdominal mass
  2. Haematuria
94
Q

What are 4 less common features of Wilms tumours?

A
  1. Abdominal pain
  2. Anorexia
  3. Anaemia (haemorrhage into mass)
  4. Hypertension
95
Q

What is the key investigation for Wilms tumour?

A

ultrasound and/or CT/MRI usually characteristic, showing intrinsic renal mass distorting the normal structure

96
Q

Where do distant metastastases from Wilms tumours usually occur?

A

in lung

97
Q

What are 3 aspects to the management of Wilms tumours?

A
  1. Initial chemotherapy
  2. Delayed nephrcetomy - histological staging, further treatmnet planned
  3. Radiotherapy - for more advnaced disease
98
Q

What proportion of patients with Wilms tumours have bilateral disease and how does this change the management?

A
  • 5%
  • require careful management to preserve as much renal function as possible
99
Q

What is the prognosis for Wilms tumours?

A
  • good prognosis, >80% cured
  • relapse carries poor prognosis
100
Q

Wha are sarcomas?

A

cancers of connective tissue such as muscle or bone

101
Q

What is the most common form of soft tissue sarcoma in childhood?

A

rhabdomyosarcoma

102
Q

What are rhabdomyosarcomas thought to arise from?

A

primitive mesenchymal tissue

103
Q

What are the most common sites of soft tissue sarcomas in children?

A

head and neck

104
Q

What are 3 examples of the clinical features that could be caused by soft tissue sarcomas of the head and neck?

A
  1. Proptosis
  2. Nasal obstruction
  3. Bloodstained nasal discharge
105
Q

What are 3 places in the genitourinary tract where soft tissue sarcomas may occur?

A
  1. Bladder
  2. Paratesticular structures
  3. Female genitourinary tract
106
Q

What are 4 clinical features of genitourinary soft tissue sarcomas?

A
  1. Dysuria
  2. Urinary obstruction
  3. Scrotal mass
  4. Bloodstained vaginal discharge
107
Q

What is associated with particularly poor prognosis in soft tissue sarcoma?

A

metastatic disease - 15% of patients at diagnosis

108
Q

What are 4 sites that soft tissue sarcoma may metastasise to?

A
  1. Lung
  2. Liver
  3. Bone
  4. Bone marrow
109
Q

What does investigation of soft tissue sarcoma involve?

A

biopsy and full radiological assessment of primary disease and any evidence of metastasis

110
Q

What is the management of soft tissue sarcoma?

A

multimodality treatment: chemothearpy, surgery, radiotherapy

depends on patient, site, size, extent

111
Q

Why are attempts at primary surgical excision of soft tissue sarcoma often unsuccessful?

A

primary surgical excision

112
Q

What are the overall cure rates of soft tissue sarcoma?

A

65%

113
Q

When do malignant bone tumours typically occur?

A

usually after puberty

114
Q

What are 2 types of bone tumours seen in childhood and which age group do they affect?

A
  1. Osteosarcoma - older children
  2. Ewing sarcoma - younger children
115
Q

Which gender do bone tumours have a predominance before?

A

males

116
Q

What is the commonest site for bone tumours?

A

limbs

117
Q

What is the clinical presentation of bone sarcomas?

A

persistent, localised bone pain - usually precedes detection of a mass

usually otherwise well at diagnosis

118
Q

What are 5 aspects of the investigations for bone tumours?

A
  1. X-ray
  2. MRI
  3. Bone scan
  4. CXR - to assess for lung metastases
  5. Bone marrow sampling - exclude marrow involvement
119
Q

What is the management of bone tumours? 3 aspects

A

for both osteosarcoma and Ewing’s sarcoma

  1. combination chemotherapy
  2. surgery - amputation avoided using en bloc resection with endoprosthetic resection
  3. radiotherapy for Ewing sarcoma to manage local disease if surgery impossible/incomplete e.g. pelvis/axial skeleton
120
Q

What is retinoblastoma?

A

malignant tumour of the retinal cells

121
Q

What proportion of bilateral retinoblastoma is hereditary?

A

100%

122
Q

What proportion of unilateral retinoblastoma is hereditary?

A

20%

123
Q

On which chromosome does the retinoblastoma susceptibility gene exist?

A

chromosome 13

124
Q

What is the pattern of inheritance of hereditary retinoblastoma?

A

dominant but incomplete penetrance

125
Q

When does retinoblastoma most commonly present?

A

within first 3 years of life

126
Q

When should you screen for retinoblastoma?

A

children from families with the hereditary form of the disease - regular screening from birth (alongside routine)

127
Q

What are the 2 most common presentations of retinoblastoma?

A
  1. White pupillary reflex replaces normal red one
  2. Squint
128
Q

What are the 2 investigations of retinoblastoma?

A
  1. MRI
  2. Examination under anaesthetic
129
Q

What is frequently the distribution of retinoblastomas?

A

frequently multifocal

130
Q

What is the aim of treatment for retinoblastoma?

A

cure but preserve vision

131
Q

What is the treatment of retinoblastoma based on?

A

biopsy not undertaken, treatment is based on ophthalmological findings

132
Q

What are 3 options for the treatment of retinoblastoma?

A
  1. Enucleation may be necessary if advanced
  2. Chemotherapy, particularly bilateral disease, to shrink tumour(s) followed by local laser treatment to retina
  3. Radiotherapy - advanced or recurrence
133
Q

What is the prognosis of retinoblastoma?

A

most patients cured, but many visually impaired

significant risk of second malignancy (especially sarcoma) among survivors of hereditary retinoblastoma

134
Q

What is Kaposi sarcoma?

A

low-grade cancer, arises from cells of blood or lymph vessels, triggered by human herpes virus-8

135
Q

What pathogen triggers Kaposi sarcoma?

A

human herpes virus 8

136
Q

In what region is Kaposi sarcoma common and why?

A

sub-Saharan Afica due to prevalence of HIV infection in children

137
Q

What is the presentation of Kaposi sarcoma in children and how does this differ from adults?

A

generalised lymphadenopathy suggestive of lymphoma

unlike adults - typically with purple/brown skin rash

138
Q

What investigation is required for diagnosis of Kaposi sarcoma?

A

biopsy confirmation

139
Q

What is the treatment of Kaposi sarcoma?

A

combination of chemotherapy and antiretroviral therapy

Paeds: Acute Presentation (14 decks)

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