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| __NOTOC__ | | __NOTOC__ |
| {{Retinoblastoma}} | | {{Retinoblastoma}} |
| {{CMG}}{{AE}}{{Simrat}} | | {{CMG}}; {{AE}} {{Sahar}} {{Simrat}} |
| ==Overview== | | ==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]]. | | There are different modalities of treatment available for retinoblastoma. The feasibility of each strategy depends on the [[Cancer staging|stage]] of retinoblastoma at the time of [[diagnosis]]. |
| ===Management options of Retinoblastoma===
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| :*
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| {| class="wikitable"
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| ! colspan="2" |Treatment options for Intraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
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| |Unilateral retinoblastoma
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| * Enucleation followed by chemotherapy
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| *Conservative ocular salvage approaches:
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| **Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
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| **Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
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| |Bilateral retinoblastoma
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| * Enucleation for large intraocular tumors, followed by risk-adapted chemotherapy when the eye and vision cannot be saved
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| * Conservative ocular salvage approaches when the eye and vision can be saved:
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| **Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
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| **Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
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| **EBRT
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| |Cavitary retinoblastoma
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| * Systemic and/or intra-arterial chemotherapy
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| |Progressive or recurrent intraocular retinoblastoma
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| * Enucleation
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| * Radiation therapy (EBRT or plaque radiation therapy)
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| * Local treatments (cryotherapy or thermotherapy)
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| * Salvage chemotherapy (systemic or intra-arterial)
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| * Intravitreal chemotherapy
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| ! colspan="2" |Treatment options for Extraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
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| |Orbital and locoregional retinoblastoma
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| * Chemotherapy
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| * Radiation therapy
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| |CNS disease
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| * Systemic chemotherapy and CNS-directed therapy
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| * Systemic chemotherapy followed by myeloablative chemotherapy and stem cell rescue
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| |Trilateral retinoblastoma
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| * Systemic chemotherapy followed by surgery and myeloablative chemotherapy with stem cell rescue
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| * Systemic chemotherapy followed by surgery and radiation therapy
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| |Extracranial metastatic retinoblastoma
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| * Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue and radiation therapy
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| |Progressive or recurrent extraocular retinoblastoma
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| * Systemic chemotherapy and radiation therapy for orbital disease
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| * Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue, and radiation therapy for extraorbital disease
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| |}
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| ==Surgery== | | ==Surgery== |
| Enucleation is indicated for:
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| *Group E tumor | | * [[Enucleation]] is the most commonly applied method for the treatment of retinoblastoma.<ref name="KimMurphree2015">{{cite journal|last1=Kim|first1=Jonathan W.|last2=Murphree|first2=A. Linn|last3=Singh|first3=Arun D.|title=Retinoblastoma: Treatment Options|year=2015|pages=89–107|doi=10.1007/978-3-662-43451-2_9}}</ref> |
| *Advanced group D tumors may require enucleation, particularly in the setting of unilateral disease
| | * This method is [[Indication (medicine)|indicated]] for: |
| *Poor visual potential and have failed previous "globe-conserving" approaches
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| *Secondary [[glaucoma]]
| | **Unilateral or [[bilateral]] group E [[disease]] |
| *Poor view to the [[fundus]] with presumed active [[tumor]]
| | **Unilateral group D [[disease]] |
| *[[Anterior chamber]] invasion
| | **Maintenance of [[tumor]] activity in a [[Blindness|blind]] [[eye]] after treatment for [[primary tumor]] |
| 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.<ref name="pmid12096963">{{cite journal| author=Honavar SG, Singh AD, Shields CL, Meadows AT, Demirci H, Cater J et al.| title=Postenucleation adjuvant therapy in high-risk retinoblastoma. | journal=Arch Ophthalmol | year= 2002 | volume= 120 | issue= 7 | pages= 923-31 | pmid=12096963 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12096963 }} </ref> Although somewhat controversial, adjuvant chemotherapy may reduce the development of contralateral or intracranial tumors (PNET) in the child with a germline mutation.<ref name="pmid22084213">{{cite journal| author=Kaliki S, Shields CL, Shah SU, Eagle RC, Shields JA, Leahey A| title=Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma. | journal=Arch Ophthalmol | year= 2011 | volume= 129 | issue= 11 | pages= 1422-7 | pmid=22084213 | doi=10.1001/archophthalmol.2011.289 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22084213 }} </ref> 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.
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| ==References== | | ==References== |
| {{reflist|2}} | | {{reflist|2}} |
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