staging of retinoblastoma

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Three stage of retinoblastoma have been described:

  • intraocular
    • is the earliest stage of the disease and the tumour is limited to the retina or may extend to involve other structures such as the choroid, ciliary body, anterior chamber, and optic nerve head (does not extend beyond the eye) (1,2)
  • extraocular
    • is advanced stage of retinoblastoma with the tumour spreading beyond the sclera into the orbital content and to the CNS (via the optic nerve) or distant metastasis 
  • recurrent
    • the tumour has recurred or progressed after initial treatment

Various staging systems have been brought forward to describe the severity of the disease at presentation (1).

  • the Reese-Ellsworth classification
  • the International Classification for Intraocular Retinoblastoma (ICIRB)


Last reviewed 01/2018