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  1. Most common ocular tumor in children.
  2. 90% ocular before the age of 5 yr, average age at diagnosis is 18 months.
  3. Mostly unilateral with autosomal dominant inheritance.
  4. Usual chromosal anomaly is 139 14 (band 14 on longer of chromosome 13)
  5. M. C. presentation is leukokoria followed by strabismus.
  6. Specific histological findings are:
    1. Flexner Wintersteiner resettle
    2. Fleurettes. 

Enzyme markers of retinoblastoma are increase LDH, increased phosphoglucose isomerase.

  1. M.C. secondary malignancy in retinoblastoma is osteogenic sarcoma (Lung and breast cancer).
  2. High prevalence of retinoblastoma is reported with trisomy 21.
  3. CEA may be elevated in retinoblastoma.
  4. M.C. presenting pattern of growth is endophytic characterize by calcification, exophytic pattern causes retinal detachment.
  5. Most widely used staging system is reese – Ellsworth classification.
  6. Sensitive and diagnostic test is CT scan.
  7. M.C. cause glaucoma in patient with retinoblastoma is neovascularization.
  8. TOC for retinoblastoma filling most of globe is enucleation with a long stump of optic nerve.
  9. Other modalities of treatment are – cryotherapy, photocoagulation localized radioactive plagues and systemic chemotherapy.
    1. Cryotherapy – ant to equator, confined to surgery retina.
    2. Xenon ARC photocaugulation – posterior pole.
    3. Systemic chemotherapy – Cydophospharide

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