RETINOBLASTOMA

Question 1

Retinoblastoma

Answer 1

Retinoblastoma is a congenital malignant tumour originating from the retina of the eye.

Question 2

Retinoblastoma is the …………..most commonest cancer amongst children in Ghana

Answer 2

Third

Question 3

Most cases of retinoblastoma occur before…………. of age

Answer 3

5 years

Question 4

Causes of retinoblastoma

Answer 4

Genetic
Sporadic (spontaneous mutation)

Question 5

Symptoms of retinoblastoma

Answer 5

Cat’s eye reflex
Squint (strabismus)
Visual loss
Protruding eyeball
Redness of the eye

Question 6

Non-pharmacological interventions in retinoblastoma

Answer 6

Enucleation of the affected eye is curative in the early stages
Prosthetic eye insertion
Laser therapy for focal control
Radiotherapy for local spread of the tumour
Counselling to help cope with psychological impact of the disease

Question 7

First symptom of retinoblastoma in most patients

Answer 7

Cat’s eye reflex
White, shiny spot (leukocoria) in the pupil

Question 7

Investigations in retinoblasmo

Answer 7

Ultrasound scan of orbit
Head CT or MRI scan
Lumbar puncture for cerebrospinal fluid cytology
Bone marrow aspirate

Question 7

Treatment of chemotherapy associated febrile neutropenia in retinoblastoma

Answer 7

Ceftriaxone, IV, 100 mg/kg daily
And
Gentamicin, IV, 5 mg/kg daily

And (if still febrile after 48 hours, add)
Cloxacillin, IV, 25-50 mg/kg 6 hourly

And (if still febrile after 5 days, add)
Fluconazole, oral, 10 mg/kg daily

Question 7

Pharmacotherapy for Advanced disease with intracranial metastases in retinoblastoma

Answer 7

Palliative care with adequate pain and other symptom control

Question 7

Signs of retinoblastoma

Answer 7

Absent red reflex
Tumour in the retina on fundoscopy
Vitreous haemorrhage and retinal detachment on fundoscopy
Increased intraocular pressure (glaucoma)
Orbital cellulitis

Question 8

Treatment objectives in retinoblastoma

Answer 8

To arrest the progression of the tumour
To prevent distant spread of tumour
To achieve long term cure
To provide adequate supportive and palliative care in advanced
disease

Question 9

Pharmacotherapy for Intraocular and extraocular retinoblastoma

Answer 9

Vincristine, IV,
Etoposide, IV,
Carboplatin, IV,
Or
Vincristine, IV,
Etoposide, IV,
Cyclophosphamide, IV,

Question 10

Treatment of vomiting in retinoblastoma associated chemotherapy

Answer 10

Metoclopramide
or
Granisetron
or
Ondansetron

Question 11

Treatment of chemotherapy induced anaemia and thrombocytopenia in retinoblastoma

Answer 11

Blood and blood product transfusions

Question 11

Dose of granisetron for chemotherapy associated vomiting

Answer 11

Granisetron, IV, 40 microgram/kg (max. 3 mg) stat.
May repeat 12 hourly if necessary

Or

Granisetron, oral,
20 microgram/kg (max. 1 mg) within 1 hour before start of treatment
Then
20 microgram/kg 12 hourly for up to 5 days

Question 11

Dose of metoclopramide for chemotherapy associated vomiting

Answer 11

Metoclopramide, IV or oral, 100-400 microgram/kg 8 hourly

Question 11

Dose of ondansetron for chemotherapy associated vomiting

Answer 11

Ondansetron, IV,
Adult
5 mg/m2 stat.
Repeat 8 hourly if necessary
Children
12-18 years; 8 mg stat. (immediatley before chemotherapy)

or

Ondansetron, oral,
Adult
8 mg 8 hourly, administared 30 minutes before the start of chemotherapy
Children
12-18 years; 8 mg 8-12 hourly up to 5 days
1-12 years; 4 mg 8-12 hourly up to 5 days