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a Paediatrics by themes > Malignant Disease > Flashcards

Malignant Disease Flashcards

1
Q

What is the most common solid organ tumour in childhood?

A

CNS → leading cause of childhood cancer deaths

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2
Q

What are the types of CNS tumours in childhood?

A
  • Astrocytoma (cerebellar) - 40%
  • Medulloblastoma (cerebellar) - 20%
  • Ependymoma - 8%
  • Brainstem glioma - 6%
  • Craniopharyngioma - 4%
  • Atypical teratoid/rhabdoid tumour
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3
Q

What are the features of astrocytoma?

A

Benign to highly malignant → most common = pilocytic astrocytoma

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4
Q

What are the features of medulloblastoma?

A
  • Arise from midline posterior fossa
  • Associated spinal metastases
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5
Q

What are the features of ependymoma?

A

Posterior fossa

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6
Q

What are the features of brainstem glioma?

A

Malignant tumours with poor prognosis

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7
Q

What are the features of craniopharyngioma?

A
  • Squamous remnant of Rathke pouch
  • Not truly malignant but locally invasive
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8
Q

What are the features of atypical teratoid/rhabdoid tumour?

A

Rare type of aggressive tumour occurring in young children

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9
Q

In which condition is pilocytic astrocytoma common?

A

Neurofibromatosis I (NF1)

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10
Q

What is the histopathology of pilocytic astrocytoma?

A
  • Piloid (hairy) cell
  • Rosenthal fibres and granular bodies
  • Slow growing with low mitotic activity
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11
Q

What is the most common mutation in pilocytic astrocytoma?

A

BRAF

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12
Q

What are the signs and symptoms of CNS tumours?

A
  • Headaches → worse in morning, coughing
  • Vomiting → on waking
  • Gait problems / Co-ordination problems / Clumsy
  • Irritability
  • Failure to thrive
  • Visual changes
  • Behaviour or personality change
  • Raised ICP
    • Papilledema = disc oedema, obscuration of margins, elevation, venous congestion, haemorrhages
  • Separation of sutures/tense fontanelle
  • Developmental delay
  • Increased head circumference
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13
Q

What is the management of benign intracranial hypertension?

A

LP with manometry → siphon off CSF to reduce intracranial pressure + monitor

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14
Q

What are the focal signs for intracranial hypertension?

A
  • Headache
  • Vomiting
  • Changed mental state
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15
Q

What are the focal signs for supratentorial?

A
  • Focal neurological deficits
  • Seizures
  • Personality change
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16
Q

What are the focal signs for subtentorial?

A
  • Cerebellar ataxia
  • Long tract signs
  • Cranial nerve palsies
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17
Q

What are the appropriate investigations for suspected CNS tumours?

A

MRI > CT / PET

  • Pilocytic astrocytoma = cerebellar; well circumscribed, cystic, enhancing
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18
Q

What is the management of CNS tumours in children?

A
  • MDT = paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent
  • 1st line = Surgery - maximal safe resection to obtain and extensive excision with minimal damage to the patient
    • Resectability is dependent on the location, site and number of lesions
    • Craniotomy = debulking (subtotal and complete resections)
    • Open biopsies = inoperable but approachable tumours
    • Stereotactic biopsy = open biopsy not indicated
  • Radiotherapy → for low and high-grade gliomas, metastases
  • Chemotherapy → for high-grade gliomas (temozolomide)
  • Biological agents (EGFR inhibitors, PD-1 inhibitors etc.)
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19
Q

When is the peak incidence of leukaemia in children?

A

2-5 years → M > F

  • 80% = ALL
  • 20% = AML
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20
Q

What are the signs and symptoms of childhood ALL?

A
  • Bone marrow failure - anaemia, thrombocytopenia, neutropoenia
  • Local infiltration
    • Lymphadenopathy (± thymic enlargement)
    • Splenomegaly
    • Petechial rash on face and trunk
    • Hepatomegaly
    • Bone (causing pain)
    • Testes, CNS → are ‘sanctuary sites’ as chemotherapy doesn’t readily reach them
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21
Q

What are the appropriate investigations for childhood ALL?

A
  • FBC and clotting studies – anaemia, neutropenia, thrombocytopaenia ± DIC, lumour lysis syndrome
  • Peripheral blood film – lymphoblasts
  • CXR – enlarged thymus
  • Bone marrow biopsy
    • >20% blasts in BM or peripheral blood
    • Immunological and cytogenic characteristics
22
Q

What is the management of Tumour Lysis Syndrome?

A
  • Allopurinol
  • Hyperhydration
23
Q

What is the management of childhood ALL?

A
  • Systemic chemotherapy
    • 2-3 years of therapy
    • Boys treated for longer because testes are a site of accumulation of lymphoblasts
  • CNS-directed therapy (e.g. intrathecal)
    • This is done in all patients even if initial LP is negative (6-8 treatments)
  • Molecular treatment
    • Imatinib (Tyrosine Kinase Inhibitor) for Ph +ve cases
    • Rituximab (monoclonal antibodies against CD20 for B-cell depletion)
  • Transplantation
  • Supportive care
    • Blood products
    • Broad-spectrum antibiotics
24
Q

What is the prognosis of ALL?

A
  • Children = 5-year disease-free survival of 80%
  • Adults = 5-year disease-free survival of 30-40%
25
Q

What are the poor prognostic markers for ALL?

A
  • Age <2 or >10yo
  • T/B-cell surface markers
  • Non-Caucasian
  • Male sex
26
Q

What forms of of lymphoma are most common in children?

A
  • NHL = more common in childhood
  • HL = more common in adolescence - more localised and contiguous spread
27
Q

What is an Ewing’s sarcoma?

A

Primitive Neuroendocrine Tumour (PNET)

  • Malignant, small round blue-cell tumour
28
Q

What are the signs and symptoms of Ewing’s sarcoma?

A
  • Mass or swelling and Bone pain
    • Long bones of arms, legs, chest, skull and trunk
  • Malaise
  • Fever
  • Paralysis → may precipitate osteomyelitis
29
Q

What mutations are associated with Ewing’s sarcoma?

A
  • t (11:22)
  • EWSR1/FLI1
  • q24; q12
30
Q

What are the signs and symptoms of Ewing’s sarcoma?

A
  • X-Ray → bone destruction with overlying onion-skin layers of periosteal bone formation
  • Biopsy → small round blue cells
  • CT/PET/MRI
31
Q

What is the management of Ewing’s sarcoma?

A
  • Specialised sarcoma team management
  • Surgery → limb-sparing surgery ± amputation + chemotherapy + radiotherapy
  • Post-treatment → OT, PT, dietician, orthotics/prosthetics, support
32
Q

What is the prognosis of Ewing’s sarcoma?

A

Survival 5-year at 75% - 20-40% for metastasis

33
Q

What is retinoblastoma?

A

Malignant tumour of retinal cells.

  • Rare but accounts for 5% of severe visual impairment in children
  • Unilateral = 80% spontaneous, 20% hereditary
  • Bilateral (100% hereditary)
    • Autosomal dominant, chromosome 13 → encodes pRB (protein retinoblastoma)
  • Average age of diagnosis = 18 months
34
Q

What are the signs and symptoms of retinoblastoma?

A
  • Red reflex -ve → white pupillary reflex instead of normal red one
  • Squint
35
Q

What are the appropriate investigations for suspected retinoblastoma?

A
  • Ophthalmological assessment
  • MRI
  • Examination Under Anaesthesia
36
Q

What is the management of retinoblastoma?

A
  • Unilateral = Enucleation = removal of eye - leaves eye muscles intact
  • Bilateral = Chemotherapy + Laser treatment to retina ± Chemotherapy (advanced disease)
  • Prognosis
    • Most cured
    • Some may be visually impaired
    • Risk of secondary malignancy (sarcoma) in survivors of hereditary retinoblastoma
37
Q

What is neuroblastoma?

A

Tumours arising from neural crest tissue in adrenal medulla and SNS. most common extra-cranial tumour in children

  • Spectrum of disease
    • Benign = ganglioneuroma
    • Malignant = neuroblastoma
  • Most common extra-cranial tumour in children
  • Most common <5yo
38
Q

What are the signs and symptoms of neuroblastoma?

A
  • Abdominal mass - can be anywhere on the sympathetic chain
  • Systemic symptoms → WL, pallor, hepatomegaly, bone pain, limp
  • Symptoms of spinal cord compression
  • Over 2yo → symptoms of metastatic disease = bone pain, BM suppression, WL, malaise
39
Q

What are the appropriate investigations for suspected neuroblastoma?

A
  • Radiological findings
  • Raised urinary catecholamine metabolites (VMA/HVA)
  • Confirmatory biopsy from bone marrow and MIBG sampling
40
Q

What is the management of neuroblastoma?

A
  • Spontaneous regression can occur in very young infants
  • Localised primaries without metastatic disease = surgery alone
  • Metastatic disease = chemotherapy + radiotherapy (with autologous stem cell rescue) + surgery
    • High risk of relapse
  • Poor Prognosis
    • Metastatic disease cure rate = 40%
    • MYCN gene
41
Q

What are the types of Hodgkin’s lymphoma?

A
  • Classical (subtypes exist) - 95%
  • Nodular Lymphocyte Predominant HL (NLPHL) - 5%
42
Q

What are the sign and symptoms of Hodgkin’s lymphoma?

A
  • Painless lymphadenopathy (in neck) → painful on drinking alcohol (in 10%)
  • B symptoms (fever, night sweats, weight loss) → uncommon even in advanced disease
43
Q

What are the appropriate investigations for suspected Hodgkin’s lymphoma?

A
  • LN biopsy – Reed-Sternberg cells “Owl’s eyes”
  • PDG-PET or CT staging – Ann Arbor Staging
  • Bloods – prognostic markers
    • FBC
    • ESR
    • LFTs
    • LDH
    • Alb
  • Immunophenotyping – CD30, CD15 – diagnostic markers
44
Q

How is lymphoma staged?

A

Ann Arbor Staging

45
Q

What is the management of Hodgkin’s lymphoma?

A
  • Combination Chemotherapy (ABVD) ± Radiotherapy
    • Adriamycin
    • Bleomycin
    • Vincristine
    • DTIC (Dacarbazine)
  • PET scanning monitors response and guides therapy
  • Prognosis = 80% cured
    • In disseminated disease = 60%
46
Q

What are the types of Non-Hodgkin’s lymphoma?

A
  • Common
    • Diffuse Large B-cell (30-40%)
    • Follicular (35%)
  • Interesting/uncommon
    • H. pylori MALToma
    • EATL
    • HIV-associated
47
Q

What are the signs and symptoms of Non-Hodgkin’s lymphoma?

A
  • Painless lymphadenopathy ± compression symptoms
  • B symptoms (fever, night sweats, weight loss)
48
Q

What are the appropriate investigations for suspected Non-Hodgkin’s lymphoma?

A
  • LN biopsy – cytology, histology and immunophenotyping
  • PDG-PET or CT staging – Ann Arbor Staging
  • Bloods – prognostic markers
    • FBC
    • ESR
    • LFTs
    • LDH
    • Alb
49
Q

What is the management of Non-Hodgkin’s lymphoma?

A
  • Depends on type of NHL
    • Urgent chemotherapy
    • Monitor only
    • HSCT
    • Antibiotic eradication (H. pylori gastric MALToma)
  • Diffuse large B-cell lymphoma (30-40% of all NHLs):
    • Treated with 6-8 cycles of R-CHOP
      • Rituximab
      • Cyclophosphamide
      • Adriamycin
      • Vincristine
      • Prednisolone
    • Some eligible for HSCT
50
Q

What is Burkitt’s lymphoma?

A

A type of B-cell Non-Hodgkin’s lymphoma

  • Prognosis = bad → fastest growing human tumour known
51
Q

What are the types of Burkitt’s lymphoma?

A
  • Endemic – EBV infection
    • Most commonly in children living in malaria endemic regions - chronic malaria may reduce EBV resistance
      • Most common childhood cancer in Africa
    • Involves JAW or facial bones
  • Sporadic – EBV infection
    • Western world, associated with EBV infection
  • Immunodeficiency – HIV infection
    • Usually associated with HIV infection or immunocompromised post-transplant
52
Q

What is the histopathology and molecular results of Burkitt’s lymphoma?

A
  • Hispathology
    • Arises from germinal centre cells
    • Starry-sky appearance
  • Molecular
    • C-myc translocation
      • (8;14, 2;8 or 8;22)

a Paediatrics by themes (26 decks)

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