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    Retinoblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Options for Unilateral and Bilateral Retinoblastoma

    Standard Treatment Options for Unilateral Retinoblastoma

    Standard treatment options for unilateral retinoblastoma include the following:

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    1. Enucleation for large intraocular tumors, followed by risk-adapted chemotherapy when the eye cannot be saved.
    2. Conservative ocular salvage approaches when the eye and vision can be saved.
      • Chemoreduction with either of the following:
      • Local treatments including cryotherapy, thermotherapy, and plaque radiation therapy.
      • External-beam radiation therapy (EBRT).

    Enucleation followed by chemotherapy

    Because unilateral disease is usually massive and often there is no expectation that useful vision can be preserved, up-front surgery (enucleation) is commonly performed. Careful examination of the enucleated specimen by an experienced pathologist is necessary to determine whether high-risk features for metastatic disease are present. These features include the following:[1,2,3,4,5]

    • Anterior chamber seeding.
    • Massive choroidal involvement.
    • Tumor beyond the lamina cribrosa.
    • Scleral and extrascleral extension.

    Pre-enucleation magnetic resonance imaging has low sensitivity and specificity for the detection of high-risk pathology.[6]

    Systemic adjuvant therapy with vincristine, doxorubicin, and cyclophosphamide or with vincristine, carboplatin, and etoposide has been used to prevent the development of metastatic disease in patients with certain high-risk features assessed by pathologic review after enucleation.[3,7,8]; [9][Level of evidence: 2A]

    Conservative ocular salvage approaches

    Conservative ocular salvage approaches, such as chemotherapy and local-control treatments, may be offered in an attempt to save the eye and preserve vision.[10] Ocular salvage rates correlate with intraocular stage. In selected children with unilateral disease, the Reese-Ellsworth (R-E) Group was correlated with ocular outcomes. While the possibility of saving the eye without the use of EBRT was greater than 80% for children with R-E Group II or III disease, the ocular outcomes for children with R-E Group V eyes were poor, with less than 40% ocular salvage rates, even after the use of EBRT.[11]

    Caution must be exerted with extended systemic chemotherapy administration and delayed enucleation when tumor control does not appear to be possible, particularly for Group E eyes. Pre-enucleation chemotherapy for eyes with advanced intraocular disease may result in downstaging and underestimate the pathological evidence of extraretinal and extraocular disease, thus increasing the risk of dissemination.[12]

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