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.

Management options of Retinoblastoma

Treatment options for Intraocular tumor
Unilateral retinoblastoma	Enucleation followed by chemotherapy Conservative ocular salvage approaches: Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
Bilateral retinoblastoma	Enucleation for large intraocular tumors, followed by risk-adapted chemotherapy when the eye and vision cannot be saved Conservative ocular salvage approaches when the eye and vision can be saved: Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy) EBRT
Cavitary retinoblastoma	Systemic and/or intra-arterial chemotherapy
Progressive or recurrent intraocular retinoblastoma	Enucleation Radiation therapy (EBRT or plaque radiation therapy) Local treatments (cryotherapy or thermotherapy) Salvage chemotherapy (systemic or intra-arterial) Intravitreal chemotherapy
Treatment options for Extraocular tumor
Orbital and locoregional retinoblastoma	Chemotherapy Radiation therapy
CNS disease	Systemic chemotherapy and CNS-directed therapy Systemic chemotherapy followed by myeloablative chemotherapy and stem cell rescue
Trilateral retinoblastoma	Systemic chemotherapy followed by surgery and myeloablative chemotherapy with stem cell rescue Systemic chemotherapy followed by surgery and radiation therapy
Extracranial metastatic retinoblastoma	Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue and radiation therapy
Progressive or recurrent extraocular retinoblastoma	Systemic chemotherapy and radiation therapy for orbital disease Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue, and radiation therapy for extraorbital disease

Surgery

Enucleation is indicated for:

• Group E tumor
• Advanced group D tumors may require enucleation, particularly in the setting of unilateral disease
• Poor visual potential and have failed previous "globe-conserving" approaches
• Secondary glaucoma
• Poor view to the fundus with presumed active tumor
• Anterior chamber invasion

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. When there is a positive optic nerve margin or extrascleral/trans-scleral extension, external beam radiotherapy should be considered. Although somewhat controversial, adjuvant chemotherapy may reduce the development of contralateral or intracranial tumors (PNET) in the child with a germline mutation. An orbital implant (typically hydroxyapatite or porous polyethylene) is placed at the time of enucleation. Six weeks after the conjunctiva has healed, a prosthesis can be fitted by an ocularist.