Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the formal ranking system used by the PDQ Editorial Boards to assess evidence supporting the use of specific interventions or approaches. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Intraocular retinoblastoma is localized to the eye and may be confined to the retina or may extend to involve other structures such as the choroid, ciliary body, anterior chamber, and optic nerve head. Intraocular retinoblastoma, however, does not extend beyond the eye into the tissues around the eye or to other parts of the body.
Extraocular (metastatic) retinoblastoma has extended beyond the eye. It may be confined to the tissues around the eye (orbital retinoblastoma), or it may have spread to the central nervous system, bone marrow, or lymph nodes (metastatic retinoblastoma).
Reese-Ellsworth Classification for Intraocular Tumors
Reese and Ellsworth developed a classification system for intraocular retinoblastoma that has been shown to have prognostic significance for maintenance of sight and control of local disease at a time when surgery and external-beam radiation therapy (EBRT) were the primary treatment options.
Group I: very favorable for maintenance of sight
Solitary tumor, smaller than 4 disc diameters (DD), at or behind the equator.
Multiple tumors, none larger than 4 DD, all at or behind the equator.
Group II: favorable for maintenance of sight
Solitary tumor, 4 to 10 DD at or behind the equator.
Multiple tumors, 4 to 10 DD behind the equator.
Group III: possible for maintenance of sight
Any lesion anterior to the equator.
Solitary tumor, larger than 10 DD behind the equator.
Group IV: unfavorable for maintenance of sight
Multiple tumors, some larger than 10 DD.
Any lesion extending anteriorly to the ora serrata.
Group V: very unfavorable for maintenance of sight
Massive tumors involving more than one half of the retina.
International Classification System for Intraocular Retinoblastoma
There is a new classification system for retinoblastoma, which may offer greater precision in stratifying risk for newer therapies. The International Classification for Intraocular Retinoblastoma that is used in the current Children's Oncology Group treatment studies, as well in some institutional studies, has been shown to assist in predicting those who are likely to be cured without the need for enucleation or EBRT.[1,2,3,4]
Group A: Small intraretinal tumors away from foveola and disc.
All tumors are 3 mm or smaller in greatest dimension, confined to the retina and
All tumors are located further than 3 mm from the foveola and 1.5 mm from the optic disc.
Group B: All remaining discrete tumors confined to the retina.
All other tumors confined to the retina not in Group A.
Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
Group C: Discrete local disease with minimal subretinal or vitreous seeding.
Tumor(s) are discrete.
Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina.
Local fine vitreous seeding may be present close to discrete tumor.
Local subretinal seeding less than 3 mm (2 DD) from the tumor.
Group D: Diffuse disease with significant vitreous or subretinal seeding.
Tumor(s) may be massive or diffuse.
Subretinal fluid present or past without seeding, involving up to total retinal detachment.
Diffuse or massive vitreous disease may include "greasy" seeds or avascular tumor masses.
Diffuse subretinal seeding may include subretinal plaques or tumor nodules.
Group E: Presence of any one or more of the following poor prognosis features.
Tumor touching the lens.
Tumor anterior to anterior vitreous face involving ciliary body or anterior segment.
Murphree L: Staging and grouping of retinoblastoma. In: Singh A, Damato B: Clinical Ophthalmic Oncology. Philadelphia, Pa: Saunders Elsevier, 2007, pp 422-7.
Zage PE, Reitman AJ, Seshadri R, et al.: Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma. Pediatr BloodCancer 50 (3): 567-72, 2008.
Shields CL, Mashayekhi A, Au AK, et al.: The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology 113 (12): 2276-80, 2006.
Novetsky DE, Abramson DH, Kim JW, et al.: Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies--an analysis of impact. Ophthalmic Genet 30 (1): 40-4, 2009.
In this article
This information is produced and provided by the National
Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National
Institute via the Internet web site at http://
.gov or call 1-800-4-CANCER.
WebMD Public Information from the National Cancer Institute
September 04, 2014
This information is not intended to replace the advice of a doctor.
Healthwise disclaims any liability for the decisions you make based on this