childhood cancer.txt

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kavinashah
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childhood cancer.txt
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2011-06-19 18:09:16
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cancer
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cancer
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  1. what fraction of childhood cases does leukaemia account for?
    one third
  2. what are the 3 important causative factors in childhood cancer?
    • genetic
    • infections
    • environmental
  3. what is the gene defect in retinoblastoma?
    chromosome 13 deletion of tumour suppressor gene
  4. what is the gene defect in Li-Fraumeni?
    p53 mutation
  5. what is the inheritance of Li-Fraumeni?
    autosomal dominant
  6. which cancers are people with Li-Fraumeni at risk of getting?
    • breast
    • brain
    • leukaemia
    • sarcoma - soft tissue & bone
    • adrenal cortical
  7. what is the genetic defect with ataxia telangiectasia?
    DNA repair defect
  8. what is the gene defect assoc with downs?
    trisomy 21
  9. which are the 2 main infections that are associated with malignancy?
    • EBV: disruption of c-myc oncogene on chromosome 8 leading to malignant change eg Burkitt's lymphoma
    • HIV: lymphoma
  10. name an environmental risk factor for childhood cancers?
    previous treatment of malignancy in a child
  11. how do lymphomas usually present?
    • constitutional symptoms
    • weight loss
    • fevers
    • night sweats
  12. if there is a localised mass in the abdomen, which 2 cancers could that be?
    • wilms tumour (nephroblastoma)
    • neuroblastoma (most common extracranial solid cancer in childhood)
  13. if there is a localised mass in the thorax, what cancer could that be?
    non hodgkins lymphoma
  14. if there is a localised mass in a soft tissue, what cancer could that be?
    rhabdomyosarcoma
  15. what cancer causes LN enlargement?
    lymphoma
  16. what cancer causes symptoms of bone marrow failure?
    acute leukaemia
  17. which 2 cancers can cause bone pain?
    • leukaemia
    • bone tumours
  18. how does a primary CNS tumour present?
    signs of raised ICP
  19. what are the 5 main long term problems in survivors of childhood cancer?
    • secondary tumours
    • reduced fertility: from alkylating chemotherapy
    • cognitive/psychosocial difficulties: methotrexate, school absence
    • reduced growth and endocrine problems: irradiated glands
    • auditory: platinum drugs
    • cardiac: doxorubicin
  20. what is the most common malignancy of childhood?
    leukaemia
  21. how do you characterise leukaemia?
    proliferation of immature white cells
  22. what is the onset of leukaemia?
    insiduous onset
  23. what are the signs/symptoms of acute leukaemia? what causes them?
    • infiltration of bone marrow/other organs with leukaemia blast cells
    • anaemia: pallor & malaise
    • thrombocytopenia: purpura, easy bruising, epistaxis
    • neutropenia: infection
    • reticuloendothelial cell infiltration: hepatosplenomegaly, lymphadenopathy
    • expansion of marrow cavity: bone pain
    • general: malaise, anorexia
  24. how do you confirm the diagnosis of leukaemia?
    bone marrow aspiration
  25. what % of leukaemias are ALL?
    80%
  26. what is the peak age for incidence of ALL?
    between 3-6 years old
  27. is ALL more in boys or girls?
    boys
  28. how is ALL classified?
    according to cell surface antigens = immunophenotype
  29. what are the 3 immunophenotypes of ALL?
    • non-T, non-B cell ALL: 80% are mostly early B cell clone
    • T cell ALL: 15%
    • B cell aLL: 1%
  30. what are the 3 main prognostic factors taken into consideration for ALL and what is poor?
    • age, WBC, lineage
    • age: < 1yr
    • WBC > 50
    • lineage: T cell or B cell
  31. which children get T cell ALL?
    older children and teenagers
  32. what are 2 features of T cell ALL?
    • high peripheral WCC
    • mediastinal mass
  33. what % of ALL can be cured?
    65%
  34. which cell type of ALL have the best prognosis?
    • null cell (common)
    • ie non-T, non-B cell
  35. what % of children with ALL go into remission? and what % survive beyond 5 years?
    75% both
  36. which 3 ares of the body can relapses occur?
    • bone marrow
    • CNS
    • testes
  37. if there is a relapse of leukaemia, what needs to be done?
    • high dose chemo
    • total body irradiation
    • BM transplant
  38. why do boys need longer treatment than girls?
    as relapses can occur in their testes
  39. what has a worse prognosis ALL or AML?
    AML and has more intensive treatment
  40. what are 80% of AML cases associated with?
    chromosomal abnormalities
  41. what is the tumour marker for neuroblastoma?
    VMA
  42. what is the tumour marker for germ cell tumour/liver tumour?
    AFP
  43. which oncogene is associated with poor prognosis in neuroblastoma?
    N-myc
  44. which 2 chromosomes are translocated in Ewings sarcoma?
    11 and 12
  45. before treatment of ALL is started, what needs to be ensured?
    • anaemia is corrected with blood transfusion
    • risk of bleeding minimised with platelet infusion
    • hydration/allopurinol to protect renal function against effects of rapid cell lysis
  46. what does remission mean?
    eradication of the leukaemic blasts and restoration of normal marrow function
  47. why are blocks of intensification chemotherapy given? give one adv and one disadv
    • to consolidate remission
    • they improve cure rates but at expense of increased toxicity
  48. what is given for CNS protection? why?
    • intrathecal methotrexate
    • because systemic treatment of cytotoxic drugs poorly penetrate into CNS
  49. how many years is maintenance chemotherapy continued for in boys and girls?
    • boys 3 years (as testes involvement)
    • girls 2 years
  50. what prophylaxis treatment is given during chemotherapy and why?
    • co-trimoxazole
    • to prevent PCP
  51. what are the 6 main short term SE of chemotherapy?
    • BM suppression: anaemia, low plt, low neutrophils
    • immunosuppression: infection
    • GI: anorexia, N+V, gut mucosal damage - leading to undernutrition
    • alopecia
  52. what is the specific SE with doxorubicin?
    cardiotoxicity
  53. what are the 2 specific SE with cisplatin?
    • renal failure
    • deafness
  54. what are the 2 SE of cyclophosphamide?
    haemorrhagic cystitis
  55. what is the SE of vincristine?
    neuropathy
  56. which type of lymphoma is more common in childhood?
    NHL
  57. which type of lymphoma is more common in adolescence?
    Hodgkins lymphoma
  58. how do T cell malignancies usually present?
    • as ALL or NHL
    • both - mediastinal mass & BM infiltration
  59. how do B cell malignancies more commonly present?
    • as NHL
    • localised lymph node disease usually head, neck, abdomen
  60. how does abdominal lymphoma usually present?
    • pain
    • palpable mass
    • intussusception if the ileum is involved
  61. how does hodgkins lymphoma usually present?
    painless lymphadenopathy - neck usually
  62. how can you tell between benign lymphadenopathy and lymphoma?
    LN are larger and firmer
  63. can HL be cured even if disseminated?
    yes, 60% can be cured
  64. which age group is NHL more common in?
    young children
  65. which age group is HL more common in?
    • adolescents
    • young adults
  66. what increases your risk of NHL?
    immunocompromised eg HIV, SCID
  67. what is the growth of NHL like?
    aggressive, rapidly growing
  68. what are the different ways NHL may present?
    • peripheral LN enlargement: B cell
    • intrathoracic mass: T cell
    • mediastinal mass or pleural effusion
    • abdominal mass: advanced B cell disease
    • Gut of LN masses
  69. what is the mainstay of Rx of lymphoma?
    chemotherapy
  70. what extra Rx may be needed for abdominal tumours NHL?
    debulking surgery
  71. what are the 2 complications of NHL in the acute setting?
    • SVC syndrome: SOB, face or arm swelling, distended veins
    • tumour lysis syndrome: occurs after Rx of cancer due to breakdown products of cancer cells eg high K, high phosphate (causing low calcium - tetany seizures), high uric acid - renal failure
  72. which cell is HL characterised by?
    Reed Sternberg cell
  73. does HL affect f or m more?
    female adolescents
  74. how does HL usually present?
    • painless cervical or supraclavicular lymphadenopathy
    • systemic symptoms are uncommon
  75. where does mets of HL occur?
    • lung
    • liver
    • BM
  76. how is HL diagnosed?
    LN biopsy and histological examination
  77. what aerate 4 subtypes of HL and how does this affect prognosis?
    • lymphocyte predominance: best prognosis
    • mixed cellularity
    • nodular sclerosing: most common in children and adolescents
    • lymphocyte deplete: worse prognosis
  78. which subtype of HL has best prognosis?
    lymphocyte predominance
  79. which subtype of HL has worse prognosis?
    lymphocyte deplete
  80. which is most common subtype of HL in children?
    nodular sclerosing
  81. what is Rx of HL?
    • stage first by imaging chest mediastinum abdomen
    • Rx with combo chemo except if localised disease - radiotherapy
  82. what is overall prognosis of HL?
    • good
    • 80% cured
  83. what is the 2nd most common form of childhood cancer?
    brain tumour
  84. what is the commonest solid tumour of childhood?
    brain tumour
  85. where are 60% of brain tumours in children located?
    infratentorially
  86. what are signs and symptoms of brain tumours DUE TO?
    • increased ICP
    • cerebellar dysfunction
  87. what are the 3 main types of brain tumours?
    • astrocytomas
    • PNET - primitive neuroectodermal tumours (medulloblastoma)
    • craniopharyngioma
  88. where are high grade astrocytomas located?
    supratentorial
  89. where are low grade astrocytomas located?
    cerebellar
  90. what age do children get astrocytoma?
    6-9 years
  91. how do cerebellar astrocytomas present?
    • headache and vomiting: due to obstructive hydrocephalus
    • papilloedema
    • cerebellar: ATAXIA, nystagmus, incoordination
    • eyes: diplopia, SQUINT due to CN6 palsy
  92. how do supratentorial astrocytomas present?
    • focal neurological signs
    • seizures
  93. how do brainstem gliomas present?
    • CN palsy
    • ataxia
    • pyramidal tract signs
  94. what are worrying features of headache?
    • especially worse when lying down
    • worse first thing in morning - as due to high ICP
  95. what are worrying features of vomiting?
    • especially on waking in mornings
    • no nausea assoc with it
  96. what is the most common MALIGNANT brain tumour in childhood?
    PNET - medulloblastoma
  97. which gender and age does PNET occur in?
    • boys
    • age 2-6
  98. where do PNET arise and where do they invade?
    • arise in MIDLINE
    • invade 4th ventricle and cerebellar hemispheres
  99. what is different about PNET tumours from the other brain tumours?
    • they seed through the CNS
    • 20% have spinal mets at diagnosis
  100. what are the presenting features of PNET?
    • headache
    • vomiting
    • ataxia
  101. what is Rx of PNET?
    • surgical removal
    • whole CNS irradiation
  102. what are 5yr survival rates of PNET?
    50%
  103. where do craniopharyngiomas arise from?
    remnant of Rathke's pouch
  104. how do craniopharyngiomas present?
    • visual field loss: compression of optic chasm
    • pituitary dysfunction: growth failure, diabetes insipidus
  105. which Ix do craniopharyngiomas show up on and why?
    • XR
    • they are calcified
  106. what is Rx of craniopharyngioma?
    • surgical excision
    • may need radiotherapy
  107. what is prognosis and sequelae of craniopharyngioma?
    • good prognosis
    • sequelae: visual impairment, endocrine deficiency
  108. what is neuroblastoma?
    malignancy of NEURAL CREST CELLS
  109. what do neural crest cells normally give rise to?
    • paraspinal SYMPATHETIC ganglia
    • adrenal medulla
  110. what age children does neuroblastoma occur in?
    • preschool
    • 2yo
  111. what do clinical features of neuroblastoma depend on?
    location
  112. what is the most common mode of presentation of neuroblastoma?
    • firm
    • non tender abdominal mass
  113. what are clinical features of neuroblastoma? top to toe
    • eyes: opsoclonus myoclonus from immune response, unilateral proptosis due to mets to eye
    • mediastinum: mass on CXR
    • abdominal mass: firm, non tender
    • GI: diarrhoea due to secretion of VIP
    • liver: hepatomegaly
    • Lymphadenopathy
    • systemic: pallor, weight loss
    • bone pain: disseminated disease
  114. which Ix can be used to help diagnose neuroblastoma?
    raised urinary catecholamines - VMA
  115. how do you do a confirmatory diagnosis?
    MIBG radiolabelled tumour specific agent
  116. what is Rx of neuroblastoma?
    • surgical resection
    • chemotherapy
    • irradiation
  117. what makes prognosis worse in neuroblastoma?
    • older children
    • metastatic disease
    • overexpression of N-myc oncogene
  118. what is the proper name for Wilms tumour?
    nephroblastoma
  119. what age does wilms present?
    first 5 years
  120. what is the most common clinical presentation of wilms?
    • asymptomatic abdominal mass
    • that does not cross the midline
  121. why would you get abdominal pain in wilms?
    haemorrhage into the tumour
  122. what are the 2 causes of hypertension in wilms?
    • compression of renal artery
    • renin production by tumour cells
  123. what are other features of a wilms apart from abdominal mass?
    • abdominal pain if bleeding into tumour
    • haematuria
    • hypertension - compression of renal artery or renin production by tumour cells
  124. what other abnormalities is Wilms associated with?
    • hemihypertrophy - one side of body is larger than other
    • GU tract abnormalities
    • mental retardation
    • aniridia - absence of iris
  125. which cells does wilms tumour arise from?
    embryonic renal cells
  126. how do you make diagnosis of wilms?
    • CT characteristic appearance: INTRINSIC renal mass with MIXED solid and cystic densities
    • biopsy
  127. where are the most common mets of Wilms?
    • lungs
    • liver
  128. what is Rx of wilms?
    • surgical resection
    • chemo
    • radio if advanced disease
  129. what is prognosis of wilms?
    good 80% cure rate if no mets
  130. what age group and gender does bone tumours affect?
    • adolescents
    • mainly boys
  131. what are the 2 main types of bone tumours in children? which is most common?
    • osteogenic sarcoma - most common
    • ewing's sarcoma
  132. what age group does osteosarcoma affect?
    older children
  133. what age group does ewings sarcoma affect?
    younger children
  134. what part of the bone is osteosarcoma a tumour of?
    • bone producing mesenchyme
    • m:f 2:1
  135. what is the usual presenting feature of osteosarcoma?
    • local pain - persistent
    • swelling
  136. where do most cases of osteosarcoma occur?
    around knee joint - metaphysis of distal femur or proximal tibia
  137. where do osteosarcoma metastasise to?
    lungs
  138. what does bone x-ray of osteosarcoma show?
    • destruction
    • sunburst appearance as tumour breaks through the cortex and spicules of new bone are formed
  139. what is Rx of osteosarcoma?
    • surgery of primary AND metastatic deposits
    • aggressive chemo to treat micromets
  140. what is survival of osteosarcoma?
    >50%
  141. what is the origin of ewings - what type of tissue?
    • undifferentiated sarcoma
    • uncertain tissue of origin - bone and sometimes soft tissues
  142. which 2 types of bones does ewing's most commonly affect?
    • long bones - mid to proximal femur
    • also flat bones - pelvis
  143. what are the presenting features of ewing's?
    • pain
    • localised swelling
  144. what does XR of Ewing's look like?
    • destructive lesion
    • periosteal elevation
    • OR soft tissue mass - onion skin appearance
  145. where do Ewing's mets occur to?
    lung and other bones
  146. what is Rx of Ewing's?
    • radiotherapy to primary tumour
    • combo with chemotherapy for prevention or Rx of mets
  147. what is the genetic abnormality in retinoblastoma and which chromosome?
    • deletion of tumour suppressor gene
    • chromosome 13
  148. what % of retinoblastoma is bilateral?
    bilateral in 40%
  149. how does retinoblastoma present?
    absent red reflex
  150. what is the pattern of inheritance in Rb?
    dominant but with incomplete penetrance
  151. when do most cases of Rb present?
    first 3 yrs

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