Paediatric Oncology 1 Flashcards
What CNS tumours are associated with NF1?
Optic pathway gliomas, astrocytoma, malignant peripheral nerve sheath tumors, neurofibromas
How common are medulloblastomas and how do they present?
- 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
How common are cerebellar astrocytomas and how do they present?
- 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
How common are atypical teratoid/rhabdoid tumours and how do they present?
- 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%
What tumours are associated with the “classic triad” of headache, nausea, and vomiting as well as papilledema?
Midline or infratentorial tumors
Which childhood cancers are associated with the highest risk of secondary malignancy?
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)
What are the key side effects of vincristine?
- neuropathy
- constipation
- myalgias
- arthralgias
- cholestasis
- extravasation causes tissue necrosis
What brain tumour typically presents with raised ICP (headaches) and pituitary dysfunction (short stature/growth failure)?
Craniopharyngioma
What are the sub-types of medulloblastoma and what is the prognosis?
- 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%
Where are most juvenile pilocytic astrocytomas found?
Posterior fossa (2/3)
Classically a cystic mass with contrast enhancing nodule in wall
Also in the optic pathway
Child with esotropia or strabismus in the first year of life:
Think retinoblastoma
Can be associated with RB1
Which brain tumours cause a rise in b-HCG or AFP?
Pineal tumours or germinomas.
What is the most common extracranial solid tumour in childhood?
Neuroblastoma is the most common extracranial solid
tumour in childhood
Rapid, dancing eye movements and
myoclonus could be which tumour?
‘Opsoclonus-myoclonus syndrome’ (1-3% presentations of neuroblastoma)
50% of children who present with this have neuroblastoma
What proportion of neuroblastoma arises in the adrenal cortex?
40%
Which chemotherapy agents cause bone marrow toxicity and risk of secondary malignancy?
Alkylating agents (e.g. Cyclophosphamide)
Which chemotherapy agent causes renal tubular damage?
Ifosfamide
Which chemotherapy agents cause haemorrhagic cystitis?
- Ifosfamide and cyclophosphamide, from the toxic metabolite acrolein
- Mesna helps prevent this
Which are the typical side effects of vincristine?
- Peripheral neuropathy (i.e. avoid if you have Charcot Marie Tooth)
- Death if IT
- Jaw pain
Which chemotherapy agents cause cardiac issues?
- Anthracyclines
- Doxorubicin
- Dexrazoxane for cardioprotection
How do you assess tumour burden/severity of Non-Hodgkin Lymphoma?
Tumour burden at diagnosis is reflected in serum LDH level
What are the B symptoms for lymphoma?
Fever, night sweats, and weight loss >10%
Which type of tumour do you measure catecholamine
metabolites homovanillic acid (HVA) and vanillylmandelic acid (VMA) in?
- Neuroblastoma - 95% have them
Which type of tumour do you measure bHCG in?
- Germ cell
- Gestational trophoblastic disease
What do you measure in neural crest tumours?
Vasoactive intestinal peptide (VIP)
What tumour marker do you measure in hepatoblastoma?
AFP
What are the histology features of ependymoma?
- perivascular pseudorosettes
- ependymal rosettes
- monomorphic nuclear morphology
Which syndrome is associated with ependymoma?
NF2
Which syndrome is associated with pilocytic astrocytoma?
NF1 (20%)
What are some small round blue cell tumours?
- Hepatoblastoma
- Medulloblastoma
- Wilms
- Retinoblastoma
- Ewings
- Neuroblastoma
What syndromes are associated with medulloblastoma?
- Gorlin
- Li-Fraumeni
- Turcot
- Gardner
- Cowden
Where does craniopharyngioma arise?
- Rathke cleft
What hormone is most likely to be affected by cranial radiotherapy?
- Growth hormone (deficiency)
Which gene is associated with congenital central hypoventilation syndrome and neuroblastoma?
- PHOX2B
What are the main side effects of asparaginase?
- Pancreatitis
- Venous thrombosis
- Anaphylaxis
What chemotherapy agent(s) give(s) you an increased risk of secondary AML?
- alkylating agents
- epipodophyllotoxins
- anthracyclines
(3-5 y after chemo)
What agents give you a high risk of radiation recall?
There are many that are associated; uptodate suggests anthracyclines particularly Daunorubicin & Doxorubicin
Which chemotherapy agent is likely to give you chemical conjunctivitis?
- Cytarabine
What is the commonest cause of SVC syndrome?
- Lymphoma - mostly Non Hodgkin
What infection is associated with CVL sepsis/shock and high dose cytarabine/AML therapy?
- strep. mitis (alpha haemolytic)
Which chemo agent is SIADH related to?
vincristine
What is the major complication of allopurinol for tumour lysis syndrome?
Xanthine nephropathy
What are the risk factors for tumour lysis syndrome?
- 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
What chemotherapy is considered highest risk for anaphylaxis?
- Asparaginase and derivatives (PEG- and L- as well…)
- Carboplatin is also high risk but not used as frequently
What syndrome is associated with vestibular schwannoma?
NF2
What tumours are associated with tuberous sclerosis?
- benign tumours (renal angiomylipoma, cardiac rhabdomyoma, angiofibromas, retinal harmatoma)
- SEGA (subependymal giant cell astrocytoma)
What is a proto-oncogene?
A normal gene which, when altered by mutation, becomes an oncogene that can contribute to cancer
What does Klinefelter disease predispose you to?
Mediastinal germ cell tumour
Breast cancer
What translocation is Ewing Sarcoma associated with?
t(11:22)
Where is the tumour in diencephalic syndrome?
- 3rd ventricle, optic nerve or 4th ventricle
- Usually glioma or astrocytoma
