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RACP Paediatric Exam - Medstudy edition > Oncology > Flashcards

Oncology Flashcards

1
Q

Neurofibromatosis Type 1 is associated with which type of CNS tumor

A

Optic gliomas

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2
Q

Which tumor is associated with NF2

A

Bilateral vestibular schwannomas

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3
Q

Tumours seen in TS

A

Subependymal giant cell tumor

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4
Q

Tumour in von Hippel Lindau disease

A

Hemangioblastomas in cerebellum, medulla and spinal cord

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5
Q

Tumours in Li Fraumeni

A

Gliomas, ependymomas, choroid plexus carcinomas

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6
Q

Gorlin syndrome tumours

A

Medulloblastomas

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7
Q

Cranial nerve finding in children with brain tumours

A

Diploplia, due to 6th nerve palsy

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8
Q

What clinical finding is important to look for in an infant you suspect has a brain tumour

A

Irritability, anorexia, vomiting

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9
Q

Sun setting sign

A

Impairment of upwards gaze - early sign of increased ICP

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10
Q

Infratentorial vs supratentorial tumours

A

Infratentorial (cerebellum and below)
- coordination and cranial nerve dysfunction

Supratentorial (above cerebellum)
- headaches, weakness and seizures

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11
Q

What is parinaud syndrome

A

Impaired upwards gaze
Dilated pupils with better reactivity to accomodation than to light
Retraction or conversion nystagmus with lid retraction

Compression or infiltration of the midbrain tectum - pineal tumours

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12
Q

what symptoms can occur if a brain tumor has spread to the leptomeninges

A

Intermittent mental status changes

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13
Q

what is the mainstay of treatment for most CNS tumours

A

Surgery

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14
Q

Which age group is not treated with CNS radiation therapy due to increased risk of toxicity

A

<3yo

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15
Q

What are the most common presentations for medulloblastoma

A

Morning headache, vomiting and lerthargy
Ataxia
Head tilt (4th CN palsy)

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16
Q

If ependymoma tumors involve the 4th ventricle, what complication can occur

A

CSF flow blocked

Nausea, vomiting, morning headache, diplopia

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17
Q

Most common primary CNs timor in children

A

Gliomas

18
Q

Clinical features of astrocytomas

A

Most common posterior fossa tumor, best prognosis

Climsiness, unsteadiness of arms and legs, headaches and vomiting

19
Q

Which type of astrocytoma has the most aggressive clinical behaviour

A

Glioblastoma multiforme

20
Q

How do pineal tumours present

A

Parinaud syndrome

21
Q

NAme some of the complication sfrom craniopharyingomas

A

Visual changes
Endocrine changes
Personality and sleep patterns

Calcifications on MRI

22
Q

Meningiomas aere more common in which patients

A

Neurofibromatosis type 2

23
Q

What is the most common malignancy diagnosed in infancy

A

Neuroblastoma

24
Q

From where do neuroblastomas arise

A

Abdomen - adrenal medulla
30% in nonadrenal abdomen
20% paravertebral column of neck / chest

25
Q

What symptoms does VIP cause in neuroblastoma?

A

Vasoactive intestinal peptide secretion - intractable secretory diarrhoea

26
Q

How do you diagnose neuroblastoma?

A

Biopsy with histologic evidence of neural origin of the tumor
Urinary catecholamine excretion (VMA, HVA) - diagnosis and for tumor screening

27
Q

What finding warrents workup for neuroblastoma

A

Opsoclonus-myoclonus-ataxia syndrome

28
Q

Treatment for different groups

A

High risk - surgery, chemo and stemcell rescue, cis-retinoc acid and immune modulating therapy

29
Q

Most common primary malignancy of the kidney

A

Wilms tumor (nephroblastoma)

30
Q

What congenital disorders / syndromes are associated with Wilms tumor

A

GU anomalies
Hemihyperplasia
Sporadic aniridia

Syndrome
WAGR - wilms, aniridia, GU abnormalities, retardation
Backwith-Weidermann
Denys-Drash

31
Q

Most common clinical presentation

A

Asymptomatic abdominal / flank mass

3yo

32
Q

Mainstay of treatment for unilateral Wilms

A

Nephrectomy

Post surgical chemo

33
Q

Most common congenital renal disorder

A

Mesoblastic nephroma

- firm, solitary mass

34
Q

Most common soft tissue tumor

A

Rhabdomyosarcoma

35
Q

What cell type is affected in rhabdomyosarcoma

A

Striated skeletal muscle

36
Q

Most common presentation / site

A

Mass lesion, head and neck

37
Q

Which sites are common for botryoid subtype

A

Grape like

Vagina, bladder, nasopharynx and middle ear

38
Q

What is treatment for rhabdomyosarcoma

A

Surgical excision, chemo for all

39
Q

Presentation of familial retinoblastoma

A

Multifocal and bilateral
Requires 2 mutational hits for tumor development to occur
RB1 gene

40
Q

Treatment - retinoblastoma

A

enucleation if no change of useful vision
Bilateral - chemo

RB1 mutation, high risk for secondary malignancy - osteosarcoma or soft tissue sarcomas

RACP Paediatric Exam - Medstudy edition (9 decks)

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