Retinoblastoma Flashcards
1
Q
Retinoblastoma is the most common ocular malignancy found in children. What is the average age of diagnosis?
A
18 months old
2
Q
What % of cases are hereditary?
(The rest have no inheritance)
A
10%
(Usually inherited in an Autosomal Dominant Fashion)
3
Q
How is it caused?
A
It is caused by loss of function of the retinoblastoma tumour suppressor gene on Chromosome 13.
4
Q
How does Retinoblastoma usually present?
A
- Absence of red-reflex, repalced by a white pupil (leukocoria) - the most common presenting symptom
- Strabismus
- Visual problems
5
Q
How is it managed?
A
- Enucleation is not the only option
- Depending on how advanced the tumour is other options include external beam radiation therapy, chemotherapy and photocoagulation
6
Q
What is the prognosis of Retinoblastoma?
A
Excellent
>90% surviving in adulthood
