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RACP Paediatrics Exam > Oncology > Flashcards

Oncology Flashcards

1
Q

If you have a constitutional mutation when did this error occur?

A

At conception, at the zygote stage. Can be inherited or denovo

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2
Q

What does an oncogene do? What does a proto-oncogene do?

A

Aids in growth and division of healthy cells.
Causes the oncogene to be constantly on leading to growth mutations.

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3
Q

What do germline mutations in the APC gene predispose to?

A

Hepatoblastoma

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4
Q

In children with transient myeloprolioferative disorder, what cancer are they predisposed to and what percentage of children get it?

A

25% of these children will get AMKL or MLD

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4
Q

What surveillance is required for children with Beckwith Wiedemanns syndrome?

A

Exam and 3 monthly abdominal ultrasound until the age of 7

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5
Q

Gene associated with Ewings sarcoma

A

t(11,22) - EWSR1

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6
Q

Gene mutation in neuroblastoma

A

MYCN Amplification - High risk with poor prognosis

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7
Q

What causes Li Fraumeni Syndrome?

A

Defective TP53 in germline = loss of TSG

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8
Q

What is the pathognomonic gene translocation in Alveolar rhabomyosarcoma?

A

t (1, 13) or t (2, 13)

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9
Q

What present with small round blue cells on biopsy?

A

Ewings, Neuroblastoma, Rhabdomyosarcoma, Medulloblastoma, wilms, Retinoblastoma, anapaestic hepatoblastic

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10
Q

Which chemotherapy agent can lead to heart failure due to cardiomyopathy?

A

Doxorubicin

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11
Q

Which chemotherapy agents have the highest risk of secondary malignancy?

A

Etoposide, cyclophosphamide

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12
Q

Where does a wilms tumour metastasise to most commonly?

A

Lung

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13
Q

Which renal tumour most commonly metastasises to the brain?

A

Rhabdoid

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14
Q

Which renal tumour most commonly metastasises to the skeleton?

A

Clear cell sarcoma

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15
Q

What is the survival for children with a wilms tumour?

A

90%

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16
Q

Which organ system could be at risk of tumour formation in a child with aniridia?

A

Renal tumour
WAGR - Wilms
Aniridia
Genitourinary abnormality
Retardation

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17
Q

Which chemotherapy agent can cause pulmonary fibrosis?

A

Bleomycin

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18
Q

What is the difference between Ewings and Osteosarcoma?

A

Osteosarcoma: Metaphysis - often knee and proximal humerus, sunburst sclerotic on X-ray, spread to lung and bones, present with pain after exercise, not treated with radiation
Ewings: Diaphysis, pelvic bone tumours, ludic onion skin change on Xray, spread to lungs/bone/marrow, classic translocation t (11, 22), treated with radiation

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19
Q

Which two sarcomas can spread to the bone marrow?

A

Ewings
Alveolar Rhabdomyosarcoma

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20
Q

Which tumour is associated with opsoclonus/myoclonus, racoon eyes, horners syndrome? What screening test can you do?

A

Neuroblastoma
Urine catecholamines and MIBG but if suspecting should go on to confirm with body MRI

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21
Q

What does the ch14.18 monoclonal antibody used to treat neuroblastoma target?

A

GD2 - its an antibody that binds to neuroblastoma cells and induces immune system to attack the cancer via antibody-dependent cell mediated cytotoxicity. Severe pain requiring morphine infusion is frequent. It can cause capillary leakage, fluid retention and allergy.

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22
Q

What can be given to protect against cisplatin induced sensorineural hearing impairment?

A

Sodium thiosulfate (STS), used in children with hepatoblastoma.

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23
Q

What are the side effects of radiation?

A

Growth restriction
Hypothyroidism
Early onset coronary artery disease
Pericardial disease
Pulmonary fibrosis
Increased risk of breast cancer

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24
Q

Child presents with nausea, vomiting, abdominal mass and pain in the ileocecal junction area with fever. CT scan shows a mass at the ileocecal junction. What is the most likely diagnosis?

A

Burkitts lymphoma -90% originate from mature B cells in peyers patches within the GI Tract.

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25
Q

What cell line do Non Hodgkins lymphomas presenting as mediastinal masses originate form?

A

Thymic T cells

26
Q

10 year old male, bony non painful mass on his distal femur. Xray - stalks or broad based projections from the surface of the bone with an associated cap that is 0.5cm thick. What is the most likely diagnosis?

A

Osteochondroma

27
Q

Metaphyseal lucency of the proximal femur on X-ray that causes pain somewhat relieved by ibuprofen?

A

Osteoid osteoma

28
Q

What disease is associated with haemangioblastomas in the cerebellum, medullar and spinal cord?

A

von Hippel-Lindau disease

29
Q

Most common malignant CNS tumour in childhood

A

Medulloblastoma - ~20% cases of CNS tumours. Arise from the posterior fossa (cerebellum). Peak age 3-9 years. Highly malignant primitive neuroectodermal tumour.
Small blue round cells on histopathology.

30
Q

Most common posterior fossa CNS tumour of childhood?

A

Cerebellar astrocytoma - present with unsteadiness, clumsiness of the arms and legs, headaches and vomiting

31
Q

Most common benign CNS tumour in childhood?

A

Gliomas

32
Q

Which molecular subgroup of medulloblastoma has the best prognosis and what is the gene mutation?

A

Group One
WNT gene
>92% survival 5 years

33
Q

After resection of medulloblastoma that was causing obstruction at the 4th ventricle what is required to complete staging?

A

MRI spine and CSF from lumbar spine at least 10 days post resection

34
Q

What is standard treatment for medulloblastoma?

A

Resection, craniospinal irradiation with a boost to the resection site bed followed by adjuvant chemotherapy.

35
Q

Describe posterior fossa syndrome

A

Difficulty verbalising, excessive irritability, nystagmus, mutism, emotional lability.
Seen in about 20% after resection of medulloblastoma.

36
Q

What is the management of ependymoma?

A

Surgical resection and radiation

37
Q

What is the classic triad seen at presentation of a child with DIPG?

A

Cerebellar signs (ataxia, dysmetria, dysarthria), long track signs (increased tone, hyperreflexia, clonus, babinski sign, motor deficit), isolated or multiple cranial nerve palsies.

38
Q

High alpha fetoprotein in germinoma or non-germionomatous germ cell tumours?

A

Non-germinomatous GCT

39
Q

Usual management of low grade glioma?

A

Observation, Complete resection +/- chemotherapy

40
Q

Rosenthal fibres are pathognomonic for which CNS tumour?

A

Low grade glioma

41
Q

Toddler with emaciation, with weight loss despite good oral intake. Also concerns that the child may not be able to see properly. Where is the tumour?

A

Diencephalus - diencephalic syndrome

42
Q

What tumours are tuberous sclerosis children predisposed to?

A

Subependymal giant cell astrocytomas - TSC1 and TSC2

43
Q

What syndrome predisposes to medulloblastoma?

A

Gorlin syndrome - PTCH gene

44
Q

Plexiform fibroma treatment in NF1

A

Trametinib - MEK Inhibitor

45
Q

Treatment for subependymal giant cell astrocytoma not resectable?

A

Everolimus

46
Q

Chemotherapy that causes sensorineural high frequency hearing loss?

A

Cisplatin - damages cochlear hair cells

47
Q

Which region does a mediastinal neuroblastoma arise from?

A

Posterior

48
Q

What is the management of tumour lysis syndrome (hyperkalaemia, hyperuraemia, hyperphosphataemia)?

A

Hyperhydration
Hypouricaemic agents:
- Allopurinol –> Xanthine oxidase inhibitor to halt uric acid production
- Rasburicase (converts urate oxidase to allantoin = more soluble) –> contraindicated in G6PD deficiency
Calcium glutinate –> myocardium protection
Regular electrolyte monitoring
Only treat hypocalcaemia if symptomatic as calcium replacement binding with phosphate can cause worsening renal failure.

49
Q

Which of the following is the most commonly associated with anaphylaxis?
Asparaginase, vincristine, carboplatin, doxorubicin

A

Asparaginase

50
Q

Which of the following is the most likely to cause delayed nausea, 5-FU, Cisplatin, methotrexate, vincristine?

A

Cisplatin

51
Q

What is the best prophylactic antiemetic combination for someone receiving highly emetogenic chemotherapy?

A

Ondansetron, aprepitant (NK 1 receptor antagonist), dexamethasone

52
Q

Which chemotherapy acts on the M (Mitotic) phase of the cell cycle?

A

Vincristine

53
Q

Which chemotherapy agent is related to risk of pancreatitis?

A

Asparaginase

54
Q

Which chemotherapy agent can cause constipation?

A

Vincristine

55
Q

Which chemotherapy agent does not cause bone marrow toxicity?

A

Vincristine

56
Q

Steroid side effects?

A

HTN
Hyperglycaemia
Gastritis
Weight gain
Proximal myopathy
Behavioural mood disturbance
Reduced bone density
Avascular necrosis

57
Q

What can be used to prevent cardiac toxicity after doxorubicin?

A

Dexrazoxane

58
Q

Which chemotherapy is most likely to cause infertility secondary to gonadal dysfunction?

A

Alkylating agents such as cyclophosphamide

59
Q

Which chemotherapy agent can cause encephalopathy hours to days after treatment is initiated?

A

Ifosfamide - main symptom is generally drowsiness, rarely progressing to coma/seizures

60
Q

What is the target of rituximab?

A

CD20 on memory B cells

61
Q

What stage of the cell cycle does bleomycin act on?

A

G2

62
Q

What stage of the cell cycle does asparaginase act on?

A

G1

63
Q

What are the two common markers seen on peripheral blood film in those with AML?

A

MPO and CD7

RACP Paediatrics Exam (25 decks)

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