Criteria

International Classification of Retinoblastoma[6]

The goal of this classification is to reflect the likelihood of ocular survival based on modern treatment techniques (typically chemotherapy plus focal therapy).

Group A

  • Retinoblastoma 3 mm or less in basal dimension or thickness, located at least 3 mm from the foveola and 1.5 mm from the optic nerve.

Group B

  • Retinoblastoma not in Group A with one or more of the following:

    • Macular location (≤3 mm to foveola)

    • Juxtapapillary location (≤1.5 mm to optic nerve)

    • Additional subretinal fluid (≤5 mm from margin).

Group C

  • Retinoblastoma tumor with one of the following:

    • Focal subretinal seeds

    • Focal vitreous seeds

    • Both focal subretinal and vitreous seeds.

Group D

  • Retinoblastoma tumor with one of the following:

    • Diffuse subretinal seeds

    • Diffuse vitreous seeds

    • Both diffuse subretinal and vitreous seeds.

Group E

  • Very high-risk eyes with one or more of the following:

    • Neovascular glaucoma

    • Massive intraocular hemorrhage

    • Aseptic orbital cellulitis

    • Tumor anterior to the vitreous face

    • Tumor touching the lens

    • Diffuse infiltrating retinoblastoma

    • Phthisis bulbi (also known as end-stage eye, this is a nonfunctioning, atrophic, scarred, and disorganized globe, frequently with dystrophic calcification).

Reese-Ellsworth classification[7]

Reflects the likelihood of ocular survival after lateral port external beam radiation. The Reese-Ellsworth classification has largely been superseded by the International Intraocular Retinoblastoma Classification (following the introduction of intravenous chemotherapy for intraocular retinoblastoma).

Group I

  • a. Solitary tumor, <4 disc diameters in size, at or posterior to the equator of the eye (an imaginary line in the coronal plane that marks the division between the anterior and posterior halves of the eye).

  • b. Multiple tumors, none >4 disc diameters in size, all at or behind the equator.

Group II

  • a. Solitary tumor, 4 to 10 disc diameters in size, at or behind the equator.

  • b. Multiple tumors, 4 to 10 disc diameters in size, behind the equator.

Group III

  • a. Any lesion anterior to the equator.

  • b. Solitary tumors >10 disc diameters behind the equator.

Group IV

  • a. Multiple tumors, some >10 disc diameters in size.

  • b. Any lesion extending anteriorly to the ora serrata (the serrated junction between the retina and the ciliary body).

Group V

  • a. Massive tumors involving over half the retina.

  • b. Vitreous seeding.

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