Oncology

Question 1

Neurofibromatosis Type 1 is associated with which type of CNS tumor

Answer 1

Optic gliomas

Question 2

Which tumor is associated with NF2

Answer 2

Bilateral vestibular schwannomas

Question 3

Tumours seen in TS

Answer 3

Subependymal giant cell tumor

Question 4

Tumour in von Hippel Lindau disease

Answer 4

Hemangioblastomas in cerebellum, medulla and spinal cord

Question 5

Tumours in Li Fraumeni

Answer 5

Gliomas, ependymomas, choroid plexus carcinomas

Question 6

Gorlin syndrome tumours

Answer 6

Medulloblastomas

Question 7

Cranial nerve finding in children with brain tumours

Answer 7

Diploplia, due to 6th nerve palsy

Question 8

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

Answer 8

Irritability, anorexia, vomiting

Question 9

Sun setting sign

Answer 9

Impairment of upwards gaze - early sign of increased ICP

Question 10

Infratentorial vs supratentorial tumours

Answer 10

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

Supratentorial (above cerebellum)
- headaches, weakness and seizures

Question 11

What is parinaud syndrome

Answer 11

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

Question 12

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

Answer 12

Intermittent mental status changes

Question 13

what is the mainstay of treatment for most CNS tumours

Answer 13

Surgery

Question 14

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

Answer 14

<3yo

Question 15

What are the most common presentations for medulloblastoma

Answer 15

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

Question 16

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

Answer 16

CSF flow blocked

Nausea, vomiting, morning headache, diplopia

Question 17

Most common primary CNs timor in children

Answer 17

Gliomas

Question 18

Clinical features of astrocytomas

Answer 18

Most common posterior fossa tumor, best prognosis

Climsiness, unsteadiness of arms and legs, headaches and vomiting

Question 19

Which type of astrocytoma has the most aggressive clinical behaviour

Answer 19

Glioblastoma multiforme

Question 20

How do pineal tumours present

Answer 20

Parinaud syndrome

Question 21

NAme some of the complication sfrom craniopharyingomas

Answer 21

Visual changes
Endocrine changes
Personality and sleep patterns

Calcifications on MRI

Question 22

Meningiomas aere more common in which patients

Answer 22

Neurofibromatosis type 2

Question 23

What is the most common malignancy diagnosed in infancy

Answer 23

Neuroblastoma

Question 24

From where do neuroblastomas arise

Answer 24

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

Question 25

What symptoms does VIP cause in neuroblastoma?

Answer 25

Vasoactive intestinal peptide secretion - intractable secretory diarrhoea

Question 26

How do you diagnose neuroblastoma?

Answer 26

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

Question 27

What finding warrents workup for neuroblastoma

Answer 27

Opsoclonus-myoclonus-ataxia syndrome

Question 28

Treatment for different groups

Answer 28

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

Question 29

Most common primary malignancy of the kidney

Answer 29

Wilms tumor (nephroblastoma)

Question 30

What congenital disorders / syndromes are associated with Wilms tumor

Answer 30

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

Question 31

Most common clinical presentation

Answer 31

Asymptomatic abdominal / flank mass | 3yo

Question 32

Mainstay of treatment for unilateral Wilms

Answer 32

Nephrectomy | Post surgical chemo

Question 33

Most common congenital renal disorder

Answer 33

Mesoblastic nephroma | - firm, solitary mass

Question 34

Most common soft tissue tumor

Answer 34

Rhabdomyosarcoma

Question 35

What cell type is affected in rhabdomyosarcoma

Answer 35

Striated skeletal muscle

Question 36

Most common presentation / site

Answer 36

Mass lesion, head and neck

Question 37

Which sites are common for botryoid subtype

Answer 37

Grape like | Vagina, bladder, nasopharynx and middle ear

Question 38

What is treatment for rhabdomyosarcoma

Answer 38

Surgical excision, chemo for all

Question 39

Presentation of familial retinoblastoma

Answer 39

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

Question 40

Treatment - retinoblastoma

Answer 40

enucleation if no change of useful vision Bilateral - chemo RB1 mutation, high risk for secondary malignancy - osteosarcoma or soft tissue sarcomas