Retinoblastoma
The most common primary intraocular malignancy of childhood with high cure rates when detected early.
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What is Retinoblastoma?
Retinoblastoma is the most common primary intraocular malignancy of childhood, occurring in approximately 1 in 15,000-20,000 live births. It arises from the retina, typically in children under 5 years of age, with most cases diagnosed before age 3. Retinoblastoma is a model genetic cancer arising from biallelic inactivation of the RB1 tumor suppressor gene (Knudson's two-hit hypothesis).
Retinoblastoma is classified as heritable (germline RB1 mutation, ~40% of cases, often bilateral, increased risk of secondary cancers) or non-heritable (somatic RB1 mutations, ~60%, typically unilateral). Approximately 25% of unilateral cases are heritable. Children with germline RB1 mutations have lifelong increased risk of secondary cancers (osteosarcoma, soft tissue sarcoma, melanoma, lung cancer) and require lifelong surveillance.
Survival rates exceed 95% in developed countries with prompt diagnosis and treatment, but vision and globe preservation depend on stage at presentation. Treatment has evolved from primary enucleation to globe-sparing therapies including chemotherapy (intravenous, intra-arterial, intravitreal), focal therapies (cryotherapy, laser), and radiation. International Intraocular Retinoblastoma Classification (IIRC) guides treatment intensity. In low-resource settings, late presentation with extraocular disease remains common and lethal.
Symptoms
Risk Factors
When to See a Doctor?
If you experience any of the following symptoms, seek medical attention promptly:
- White reflex in pupil (leukocoria) — urgent evaluation
- Eye crossing (strabismus) in young child
- Vision problems noticed by family
- Red, painful eye
- Eye that looks different in photographs (white instead of red reflex)
- Family history of retinoblastoma — pediatric ophthalmology screening
- Eye enlargement or proptosis
- Routine pediatric eye check (red reflex testing in well-child visits)
- Any persistent eye abnormality in child under 5
Treatment Methods
Which Department to Visit?
You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.
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You can make an appointment with our specialists or contact us for your concerns.
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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.