Retinoblastoma surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

The feasibility of surgery depends on the tumor size, tumor location, and presence or absence of vitreous or subretinal seeds at diagnosis. When the retinoblastoma is too large to be treated by other treatment modalities, surgery may be used. In these situations, enucleation may help to prevent metastasis.

Surgery

Enucleation is indicated for:

  • Group E tumor, based on International classification for Retinoblastoma. Group E tumors are large tumors (>50 percent of globe volume) with limited to no visual potential, painful eyes, and/or tumors that extend into the optic nerve.
  • Advanced group D tumors may require enucleation, particularly in the setting of unilateral disease.
  • Enucleation is also the preferred treatment in eyes that have poor visual potential and have failed previous "globe-conserving" approaches.
  • Enucleation is often performed if the eye has any one of the following:
    • Secondary glaucoma
    • Poor view to the fundus with presumed active tumor
    • Anterior chamber invasion

Potential complications of enucleation include scleral perforation with seeding of tumor cells into the orbit. Adjuvant systemic chemotherapy or brachytherapy may be considered in patients with high-risk features to prevent metastatic disease (eg, ciliary body, iris, massive scleral, or choroidal infiltration, and invasion of the optic nerve posterior to the lamina cribrosa). Adjuvant chemotherapy may reduce the development of contralateral or intracranial tumors (PNET) in children with a germline mutation [114]. When there is a positive optic nerve margin or extrascleral/trans-scleral extension, external beam radiotherapy should be considered.[1] Although somewhat controversial, adjuvant chemotherapy may reduce the development of contralateral or intracranial tumors (PNET) in the child with a germline mutation. [2] An orbital implant (typically hydroxyapatite or porous polyethylene) is placed at the time of enucleation. After Approximately after six weeks when the conjunctiva has healed, a prosthesis can be fitted by an ocularist.

References

  1. Honavar SG, Singh AD, Shields CL, Meadows AT, Demirci H, Cater J; et al. (2002). "Postenucleation adjuvant therapy in high-risk retinoblastoma". Arch Ophthalmol. 120 (7): 923–31. PMID 12096963.
  2. Kaliki S, Shields CL, Shah SU, Eagle RC, Shields JA, Leahey A (2011). "Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma". Arch Ophthalmol. 129 (11): 1422–7. doi:10.1001/archophthalmol.2011.289. PMID 22084213.


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