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Intraocular Retinoblastoma

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)

Treatment of retinoblastoma should be planned after the extent of the tumor within and outside the eye is known. Treatment options consider both cure and preservation of sight.[1,2,3,4]

Treatment options for the involved eye include the following:

Enucleation, if the tumor is massive or if there is little expectation for useful vision.
External-beam radiation therapy (EBRT) with doses ranging from 35 Gy to 46 Gy. Because of the need to sedate young children and the intricacies of field planning, special expertise in pediatric radiation therapy is important. Newer methods of delivering EBRT are being used at many centers in an attempt to reduce adverse long-term effects. This includes intensity-modulated radiation therapy (IMRT), stereotactic radiation therapy, and proton-beam radiation therapy.[5,6,7] The Children's Oncology Group (COG) is conducting a clinical trial in which reduced-dose (26 Gy) IMRT is being used in combination with chemoreduction.
Cryotherapy, used in addition to radiation or in place of photocoagulation for lesions smaller than 4 disc diameters (DD) in the anterior portion of the retina.
Light coagulation (photocoagulation), occasionally used alone with small tumors. In patients with early-stage disease, light coagulation is usually used in addition to radiation therapy or when there is limited recurrence following radiation therapy. Photocoagulation is used for posteriorly located tumors that are smaller than 4 DD, distinct from the optic nerve head and macula, and without involvement of large nutrient vessels or choroid involvement. Thermotherapy delivered via infrared radiation is an alternative to laser photocoagulation.[8]
Brachytherapy with radioactive plaques for either focal unilateral presentations or recurrent disease following previous EBRT.[9,10,11]
Systemic chemotherapy: During the past 10 years, systemic chemotherapy to reduce tumor volume (chemoreduction) and to avoid the long-term effects of radiation therapy for patients with intraocular tumors has succeeded in rendering many eyes amenable to treatment with cryotherapy or photocoagulation.[1,2,12] Chemotherapy may also be continued or initiated with concurrent local control interventions.[13] Factors such as tumor location (macula), patient age (patient older than 2 months), and tumor size correlate with responsiveness to chemotherapy.[13,14] Multiagent chemotherapy is generally used although carboplatin as a single agent causes shrinkage of retinoblastoma tumors.[15,16][Level of evidence: 3iiiDiii] Most tumors treated with vincristine and carboplatin require additional local therapy;[1,2,12,17,18] the addition of etoposide to the chemotherapy regimen may improve outcome.[14,19] One study utilized carboplatin and etoposide with focal therapy, without vincristine and found acceptable vision salvage rates for Reese-Ellsworth (R-E) Groups I through IV and International Classification Groups A and B retinoblastoma.[20] The success rate of these trials varies from center to center, but overall, the rate is highest for tumors that are unilateral or unifocal and without vitreous seeding (see below). There are emerging data suggesting that the use of systemic chemotherapy may decrease the risk of development of trilateral retinoblastoma.[21] Local tumor recurrence is not uncommon in the first few years after treatment,[22] and can often be successfully treated with focal therapy.[11] Among patients with heritable disease, younger patients and those with a positive family history are more likely to form new tumors. Chemotherapy may treat small previously undetected lesions by slowing their growth and this may improve overall salvage with focal therapy.[23]
Subtenon (subconjunctival) chemotherapy: Carboplatin is administered by the treating ophthalmologist into the subconjunctival space. This modality is undergoing testing in phase I and II trials and is generally used in conjunction with systemic chemotherapy and local ophthalmic therapies for retinoblastoma with vitreous seeding. This approach offers some promise in this group of patients.[24,25]

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WebMD Public Information from the National Cancer Institute

Last Updated: August 02, 2010

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