Other solid organ cancers

Question 1

Neuroblastoma is what type of tumour?

Answer 1

Small blue round cell tumour of the SNS

Question 2

What paraneoplastic syndromes are associated with neuroblastoma?

Answer 2

Opsoclonus myoclonus syndrome (OMAS)

Persistent watery diarrhoea (VIP secretion)

Question 3

What causes patients with neuroblastoma to be hypertensive?

Answer 3

Increased catecholamine secretion

or renal artery compression

Question 4

What type of urinary investigation would you like for neuroblastoma?

Answer 4

Spot urinary catecholamines for HVA/VMA

Question 5

What are the poor prognostic markers for neuroblastoma?

Answer 5

Age >1yr
Stage 3 & 4 disease (not 4S)
Raised tumour markers: ferritin, LDH, NSE (neurone specific enolase)
Cytogenetic abnormalities: N-MYC amplification, 17q gain, 1p loss

Question 6

What is a Wilms tumour also called?

Answer 6

nephroblastoma

Question 7

Peak age for wilms tumour?

Answer 7

2-3yrs

Question 8

Which syndromes/malformations are at increased risk for Wilms tumour? (5)

Answer 8

WAGR syndrome
Denys-drash syndrome
Beckwith-Wiedemann syndrome
Hemi-hypertrophy
Genitourinary malformations

Question 9

Which coagulopathy occurs secondary to Wilms tumour?

Answer 9

acquired von Willebrand deficiency

Question 10

What are the features of WAGR syndrome?

Answer 10

W: Wilms
A: Aniridia
G: Genital deformity
R: retardation

Question 11

What are the features of denys-drash syndrome? (3)

Answer 11

Pseudohermaphroditism
GN
Wilms

Question 12

What are the features of Beckwith-Wiedemann syndrome?

Answer 12

Hemihypertrophy
Omphalocele
Macrosomy
Macroglossia
Hypoglycaemia

Question 13

Where does Wilms tumour metastasise to?

Answer 13

lung

Question 14

What are the 3 most common sites for rhabdomyosarcoma?

Answer 14

Head & neck (40%)
Genitourinary (20%)
Extremities (20%)

Question 15

What are the two cellular types of rhabdomyosarcoma?

Answer 15

Embryonal (75%, better prognosis)

Alveolar (25%, worse prognosis)

Question 16

What are the negative prognostic factors for rhabdomyosarcoma?

Answer 16

Site: paramegningeal, extremities, bladder/prostate
Tumour size: >5cm
LN involvement
Incomplete initial surgery
Age: 10yrs

Question 17

Which genetic anomaly is associated with retinoblastoma?

Answer 17

deletion of tumour suppressor gene at chr 13q14

Question 18

Presenting complaint of retinoblastoma?

Answer 18

leukocoria (white eye reflex)
strabismus
decreased vision

Question 19

Breakdown of retinoblastoma (hereditary vs sporadic)?

Answer 19

Hereditary 40%

Sporadic 60%