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Intraocular Retinoblastoma Treatment

    Treatment of retinoblastoma is individualized and considers the age of the patient, laterality, potential for vision, and intraocular tumor burden. Treatment options consider both cure of the disease and preservation of sight.[1,2,3] Different combinations of the following approaches may be applied to the individual patient, considering the two main scenarios, unilateral and bilateral disease.

    Treatment options for the involved eye include the following:

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    1. Enucleation: If the tumor is massive and there is little expectation for useful vision in the affected eye, up-front enucleation may be indicated, depending on laterality. Patients must be monitored closely for orbital recurrence of disease, particularly in the first 2 years after enucleation.[4][Level of evidence: 3iiA] Recurrence in the orbit is often associated with systemic disease (85%) and should be treated with aggressive therapy.
    2. Radiation therapy:
      • External-beam radiation therapy (EBRT): Retinoblastoma is a very radiosensitive malignancy; EBRT doses ranging from 35 Gy to 46 Gy usually result in long-term remissions. 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, stereotactic radiation therapy, and proton-beam radiation therapy (charged-particle radiation therapy).[5,6,7] EBRT in infants causes growth failure of the orbital bones and results in cosmetic deformity. It also increases the risk of subsequent neoplasms in children with hereditary retinoblastoma.
      • Brachytherapy: Brachytherapy with radioactive plaques is very effective in the treatment of localized retinal tumors that are not amenable to other means of local therapy.[8,9,10]
    3. Local treatments: For patients undergoing eye salvage treatment, aggressive local therapy is required.
      • Cryotherapy: Cryotherapy is based on the application of a cryoprobe to the sclera in the immediate vicinity of the retinal tumor. It is used as primary therapy or with chemotherapy for tumors smaller than 4 disc diameters (DD) in the anterior portion of the retina.
      • Laser therapy (thermotherapy): Laser therapy may be used as primary therapy for small tumors or in combination with chemotherapy for larger tumors. Traditional photocoagulation, in which the laser was applied around the tumor, has given way to thermotherapy. Thermotherapy is delivered directly to the tumor surface via infrared wavelengths of light.[11]
    4. Systemic chemotherapy: Systemic chemotherapy plays a role both in the adjuvant setting for patients with high-risk pathology, and in the eye-salvage regimens, where it is used in conjunction with aggressive focal treatments. During the past 15 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 laser therapy.[1,12,13]; [14][Level of evidence: 3iiDiii] Chemotherapy may also be continued or initiated with concurrent local control interventions.[15] Factors such as tumor location (macula), patient age (patient older than 2 months), and tumor size correlate with responsiveness to chemotherapy.[15,16]

      Multiagent chemotherapy is generally used, although carboplatin as a single agent causes shrinkage of retinoblastoma tumors.[17]; [18][Level of evidence: 3iiiDiii] Most standard regimens incorporate vincristine, carboplatin, and etoposide, although a two-drug regimen without etoposide may also be effective for early intraocular stages.[1,12,13,16,19,20,21,22] The success rate of these trials varies from center to center, but overall, the rate is highest for discrete tumors without vitreous seeding. Local tumor recurrence is not uncommon in the first few years after treatment [23] and can often be successfully treated with focal therapy.[10] Among patients with hereditary disease, younger patients and those with positive family histories 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.[24]

      There are data suggesting that the use of systemic chemotherapy may decrease the risk of development of trilateral retinoblastoma.[25]

    5. Subtenon (subconjunctival) chemotherapy: Periocular delivery of carboplatin results in high intraocular concentrations of the agent, and this approach is often used in ocular salvage approaches, particularly when there is a high intravitreous tumor burden. Carboplatin is administered by the treating ophthalmologist into the subtenon space, and it is generally used in conjunction with systemic chemotherapy and local ophthalmic therapies.[26,27,28] Responses have also been noted with subtenon topotecan.[29]
    6. Ophthalmic artery infusion of chemotherapy: Direct delivery of chemotherapy into the eye globe via cannulation of the ophthalmic artery is a feasible and effective method for ocular salvage. Melphalan was the chemotherapeutic agent used in the first studies, although it can be associated with significant local side effects, including third cranial nerve palsy, orbital edema, permanent retinal detachment, vitreous hemorrhage, and retinal pigment epithelium changes.[30] Other agents such as topotecan and carboplatin are also being tested. Ocular salvage rates are greater than 70% when ophthalmic artery infusion of chemotherapy is used as primary treatment, although success rates are inferior when this approach is used after failure of systemic chemotherapy or radiation.[31,32,33] Retinal and choroidal vasculopathy may occur in 10% to 20% of patients.[34] This modality continues to undergo study at very specialized retinoblastoma treatment centers, but preliminary data appear to indicate that this treatment modality results in satisfactory ocular salvage rates in patients with intraocular unilateral retinoblastoma.[31,33,34,35,36,37,38]

      This treatment is not without complications in some cases.[31,37,39]

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