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Paediatrics RACP Exam Study > Paediatric Oncology 1 > Flashcards

Paediatric Oncology 1 Flashcards

1
Q

What CNS tumours are associated with NF1?

A

Optic pathway gliomas, astrocytoma, malignant peripheral nerve sheath tumors, neurofibromas

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2
Q

How common are medulloblastomas and how do they present?

A
  • 35-40% of CNS tumours
  • MOST COMMON malignant brain tumour of childhood, male>female
  • 2-3 mo of headaches, vomiting, truncal ataxia
  • Heterogeneously or homogeneously enhancing fourth ventricular mass; may be disseminated
  • overall 65-85% survival
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3
Q

How common are cerebellar astrocytomas and how do they present?

A
  • 35-40%
  • 3-6 mo of limb ataxia; secondary headaches, vomiting - Cerebellar hemisphere mass, usually with cystic and solid (mural nodule) components
  • 90-100% survival if totally resected, pilocytic
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4
Q

How common are atypical teratoid/rhabdoid tumours and how do they present?

A
  • Incredibly malignant and pretty rare
  • > 5 (10-15% of infantile malignant tumors)
  • lethargy/vomiting; often facial weakness/strabismus
  • Similar to medulloblastoma, fourth ventricular mass; may be disseminated but often more laterally extended
  • 2/3 cerebellar/cerebellopontine angle, invasion of surrounding structures; 1/4 supratentorial, 8% multifocal
  • ≤20% survival in infants, overall <10%
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5
Q

What tumours are associated with the “classic triad” of headache, nausea, and vomiting as well as papilledema?

A

Midline or infratentorial tumors

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6
Q

Which childhood cancers are associated with the highest risk of secondary malignancy?

A

Hereditary retinoblastoma (RB1) > Hodgkin > soft tissue sarcomas

Hereditary retinoblastoma by far has the highest rate of secondary malignancy ( ~50% at 50yrs) if RB1
Hodgkins - often radiation, therefore tumours in the radiation field (e.g. breast and thyroid).
ALL due to alkylating agents (secondary AML) or if craniospinal irradiation for relapse (brain tumours)

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7
Q

What are the key side effects of vincristine?

A
  • neuropathy
  • constipation
  • myalgias
  • arthralgias
  • cholestasis
  • extravasation causes tissue necrosis
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8
Q

What brain tumour typically presents with raised ICP (headaches) and pituitary dysfunction (short stature/growth failure)?

A

Craniopharyngioma

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9
Q

What are the sub-types of medulloblastoma and what is the prognosis?

A
  • Sonic hedgehog (SHH), overactive signalling, if TP53 mutation survival 40%, if “wild” TP53 survival 80%, associated with Gorlin syndrome SUFU/PTCH1 mutation
  • Wingless-related integration site (Wnt), 6-8% have APC/familial adenomatous polyposis mutation, survival 95%
  • Group 3 - worst prognosis, survival 50%
  • Group 4 - often metastatic, survival 75%
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10
Q

Where are most juvenile pilocytic astrocytomas found?

A

Posterior fossa (2/3)
Classically a cystic mass with contrast enhancing nodule in wall
Also in the optic pathway

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11
Q

Child with esotropia or strabismus in the first year of life:

A

Think retinoblastoma

Can be associated with RB1

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12
Q

Which brain tumours cause a rise in b-HCG or AFP?

A

Pineal tumours or germinomas.

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13
Q

What is the most common extracranial solid tumour in childhood?

A

Neuroblastoma is the most common extracranial solid

tumour in childhood

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14
Q

Rapid, dancing eye movements and

myoclonus could be which tumour?

A

‘Opsoclonus-myoclonus syndrome’ (1-3% presentations of neuroblastoma)
50% of children who present with this have neuroblastoma

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15
Q

What proportion of neuroblastoma arises in the adrenal cortex?

A

40%

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16
Q

Which chemotherapy agents cause bone marrow toxicity and risk of secondary malignancy?

A

Alkylating agents (e.g. Cyclophosphamide)

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17
Q

Which chemotherapy agent causes renal tubular damage?

A

Ifosfamide

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18
Q

Which chemotherapy agents cause haemorrhagic cystitis?

A
  • Ifosfamide and cyclophosphamide, from the toxic metabolite acrolein
  • Mesna helps prevent this
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19
Q

Which are the typical side effects of vincristine?

A
  • Peripheral neuropathy (i.e. avoid if you have Charcot Marie Tooth)
  • Death if IT
  • Jaw pain
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20
Q

Which chemotherapy agents cause cardiac issues?

A
  • Anthracyclines
  • Doxorubicin
  • Dexrazoxane for cardioprotection
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21
Q

How do you assess tumour burden/severity of Non-Hodgkin Lymphoma?

A

Tumour burden at diagnosis is reflected in serum LDH level

22
Q

What are the B symptoms for lymphoma?

A

Fever, night sweats, and weight loss >10%

23
Q

Which type of tumour do you measure catecholamine

metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) in?

A
  • Neuroblastoma - 95% have them
24
Q

Which type of tumour do you measure bHCG in?

A
  • Germ cell

- Gestational trophoblastic disease

25
Q

What do you measure in neural crest tumours?

A

Vasoactive intestinal peptide (VIP)

26
Q

What tumour marker do you measure in hepatoblastoma?

A

AFP

27
Q

What are the histology features of ependymoma?

A
  • perivascular pseudorosettes
  • ependymal rosettes
  • monomorphic nuclear morphology
28
Q

Which syndrome is associated with ependymoma?

A

NF2

29
Q

Which syndrome is associated with pilocytic astrocytoma?

A

NF1 (20%)

30
Q

What are some small round blue cell tumours?

A
  • Hepatoblastoma
  • Medulloblastoma
  • Wilms
  • Retinoblastoma
  • Ewings
  • Neuroblastoma
31
Q

What syndromes are associated with medulloblastoma?

A
  • Gorlin
  • Li-Fraumeni
  • Turcot
  • Gardner
  • Cowden
32
Q

Where does craniopharyngioma arise?

A
  • Rathke cleft
33
Q

What hormone is most likely to be affected by cranial radiotherapy?

A
  • Growth hormone (deficiency)
34
Q

Which gene is associated with congenital central hypoventilation syndrome and neuroblastoma?

A
  • PHOX2B
35
Q

What are the main side effects of asparaginase?

A
  • Pancreatitis
  • Venous thrombosis
  • Anaphylaxis
36
Q

What chemotherapy agent(s) give(s) you an increased risk of secondary AML?

A
  • alkylating agents
  • epipodophyllotoxins
  • anthracyclines

(3-5 y after chemo)

37
Q

What agents give you a high risk of radiation recall?

A

There are many that are associated; uptodate suggests anthracyclines particularly Daunorubicin & Doxorubicin

38
Q

Which chemotherapy agent is likely to give you chemical conjunctivitis?

A
  • Cytarabine
39
Q

What is the commonest cause of SVC syndrome?

A
  • Lymphoma - mostly Non Hodgkin
40
Q

What infection is associated with CVL sepsis/shock and high dose cytarabine/AML therapy?

A
  • strep. mitis (alpha haemolytic)
41
Q

Which chemo agent is SIADH related to?

A

vincristine

42
Q

What is the major complication of allopurinol for tumour lysis syndrome?

A

Xanthine nephropathy

43
Q

What are the risk factors for tumour lysis syndrome?

A
  • Related to high tumour burden and rapid cell turnover
  • Certain diagnoses e.g. T-ALL, burkitt
  • Labs at diagnosis e.g. high LDH or urate prior
44
Q

What chemotherapy is considered highest risk for anaphylaxis?

A
  • Asparaginase and derivatives (PEG- and L- as well…)

- Carboplatin is also high risk but not used as frequently

45
Q

What syndrome is associated with vestibular schwannoma?

A

NF2

46
Q

What tumours are associated with tuberous sclerosis?

A
  • benign tumours (renal angiomylipoma, cardiac rhabdomyoma, angiofibromas, retinal harmatoma)
  • SEGA (subependymal giant cell astrocytoma)
47
Q

What is a proto-oncogene?

A

A normal gene which, when altered by mutation, becomes an oncogene that can contribute to cancer

48
Q

What does Klinefelter disease predispose you to?

A

Mediastinal germ cell tumour

Breast cancer

49
Q

What translocation is Ewing Sarcoma associated with?

A

t(11:22)

50
Q

Where is the tumour in diencephalic syndrome?

A
  • 3rd ventricle, optic nerve or 4th ventricle

- Usually glioma or astrocytoma

Paediatrics RACP Exam Study (30 decks)

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