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Staging of retinoblastoma

A number of systems have been devised to stage retinoblastoma, with various end-points, and multiple systems are often used concurrently. 

The Reese Ellsworth classification is assessed with fundoscopy and aims at predicting the chance of preserving the eye with external beam radiotherapy 3.

More relevant to radiology practice is the International retinoblastoma classification which predicts overall survival 1-4.

  • Stage 0: patients treated conservatively
  • Stage I: eye enucleated, completely resected histologically
  • Stage II: eye enucleated, microscopic residual tumour
  • Stage III: regional extension
    • A: overt orbital disease
    • B: preauricular or cervical lymph node extension
  • Stage IV: metastatic disease
    • A: haematogeneous metastasis without CNS involvement
      • single lesion
      • multiple lesions
    • B: CNS metastatic involvement
      1. prechiasmatic lesion
      2. CNS mass
      3. leptomeningeal disease

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