Retinoblastoma medical therapy: Difference between revisions

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===Enucleation of the eye===
===Enucleation of the eye===
Most patients with unilateral disease present with advanced intraocular disease and therefore usually undergo [[enucleation]], which results in a cure rate of 95%. In bilateral retinoblastoma, enucleation is usually reserved for eyes that have failed all known effective therapies or without useful vision.
Most patients with unilateral disease present with advanced intraocular disease and therefore usually undergo [[enucleation]], which results in a cure rate of 95%. In bilateral retinoblastoma, enucleation is usually reserved for eyes that have failed all known effective therapies or without useful vision.
===External beam radiotherapy (EBR)===
===External beam radiotherapy===
The most common indication for EBR is for the eye in a young child with bilateral retinoblastoma who has active or recurrent disease after completion of [[chemotherapy]] and local therapies. However, patients with hereditary disease who received EBR therapy are reported to have a 35% increased risk of recurrence.
*This type of therapy is not recommended as first-line treatment of primary intra-ocular retinoblastoma due to the high risk of secondary malignancies following radiation in patients carrying RB1 gene mutation.<ref name="pmid23674091">{{cite journal |vauthors=MacCarthy A, Bayne AM, Brownbill PA, Bunch KJ, Diggens NL, Draper GJ, Hawkins MM, Jenkinson HC, Kingston JE, Stiller CA, Vincent TJ, Murphy MF |title=Second and subsequent tumors among 1927 retinoblastoma patients diagnosed in Britain 1951-2004 |journal=Br. J. Cancer |volume=108 |issue=12 |pages=2455–63 |date=June 2013 |pmid=23674091 |pmc=3694232 |doi=10.1038/bjc.2013.228 |url=}}</ref>
 
*This therapy may be helpful in salvage of the remaining eye which is resistant to another kind of therapies.
===Brachytherapy===
===Brachytherapy===
[[Brachytherapy]] involves the placement of a radioactive implant (plaque), usually on the sclera adjacent to the base of a tumor. It is used as the primary treatment or, more frequently, in patients with small tumors or in those who had failed initial therapy including previous EBR therapy.
[[Brachytherapy]] involves the placement of a radioactive implant (plaque), usually on the sclera adjacent to the base of a tumor. It is used as the primary treatment or, more frequently, in patients with small tumors or in those who had failed initial therapy including previous EBR therapy.
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===Intra-arterial chemotherapy===
===Intra-arterial chemotherapy===
Chemotherapeutic drugs are administered locally via a thin catheter threaded through the groin, aorta, and the neck directly into the optic vessels.
Chemotherapeutic drugs are administered locally via a thin catheter threaded through the groin, aorta, and the neck directly into the optic vessels.
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| valign="top" |
|+
{| class="wikitable"
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Patient Group}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Treatment Line}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Treatment}}
|-
| colspan="3" style="font-weight: bold;" | With vitreous seeding
|-
| rowspan="2" style="font-weight: bold;" | With gross anterior chamber involvement or neovascular glaucoma or  orbital  inflammation
| First
| Enucleation
|-
| Adjunct
| Postoperative systemic chemotherapy
|-
| rowspan="5" style="font-weight: bold;" | Without gross anterior chamber involvement  or neovascular glaucoma or orbital inlammation
| First
| Systemic or intra-arterial chemotherapy
|-
| Plus
| Concurrent laser ablation or chemotherapy
|-
| Second
| External beam radiation therapy
|-
| Third
| Periocular carboplatin therapy
|-
| Fourth
| Enucleation
|-
| colspan="3" style="font-weight: bold;" | Without vitreous seeding
|-
| rowspan="5" style="font-weight: bold;" | Tumor >2 disc diameters in size
| First
| Systemic or intra-arterial chemotherapy
|-
| Plus
| Concurrent laser ablation or cryotherapy
|-
| Second
| External beam radiation therapy
|-
| Third
| Periocular carboplatin therapy
|-
| Fourth
| Enucleation
|-
| rowspan="6" style="font-weight: bold;" | Tumor 2 disc diameters or less in size
| First
| Focal laser ablation alone
|-
| Second
| Systemic or intra-arterial chemotherapy
|-
| Plus
| Concurrent laser ablation or cryotherapy
|-
| Third
| External beam radiation therapy
|-
| Fourth
| Periocular carboplatin therapy
|-
| Fifth
| Enucleation
|-
| style="font-weight: bold;" | Metastatic disease
| First
| Multimodal therapy
|-
| colspan="3" style="font-weight: bold;" | Recurrence
|-
| style="font-weight: bold;" | Post globe-salvaging therapy
| First
| Brachytherapy
|-
| rowspan="2" style="font-weight: bold;" | Post enucleation
| First
| External beam radiation therapy
|-
| Adjunct
| Systemic chemotherapy
|}
|}
===Management options of Retinoblastoma===
===Management options of Retinoblastoma===
:*  
:*  

Revision as of 15:52, 6 May 2019

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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] Sahar Memar Montazerin, M.D.[3]

Overview

Medical therapy for intraocular tumors

The priority of retinoblastoma treatment is:[1]

  • Save the life of the child
  • Preserve vision
  • Minimize complications or side effects of treatment.

The exact course of treatment will depend on the individual case and will be decided by the ophthalmologist in discussion with the pediatric oncologist. Children with the involvement of both eyes at diagnosis usually require multimodality therapy (chemotherapy and local therapies).
The choice of therapy depends upon several factors such as:[2]

  • Tumor size
  • Tumor location
  • Presence or absence of vitreous or subretinal seeds
  • Age of the patient

Treatment modalities which are currently available for retinoblastoma include:

Enucleation of the eye

Most patients with unilateral disease present with advanced intraocular disease and therefore usually undergo enucleation, which results in a cure rate of 95%. In bilateral retinoblastoma, enucleation is usually reserved for eyes that have failed all known effective therapies or without useful vision.

External beam radiotherapy

  • This type of therapy is not recommended as first-line treatment of primary intra-ocular retinoblastoma due to the high risk of secondary malignancies following radiation in patients carrying RB1 gene mutation.[3]
  • This therapy may be helpful in salvage of the remaining eye which is resistant to another kind of therapies.

Brachytherapy

Brachytherapy involves the placement of a radioactive implant (plaque), usually on the sclera adjacent to the base of a tumor. It is used as the primary treatment or, more frequently, in patients with small tumors or in those who had failed initial therapy including previous EBR therapy.

Thermotherapy

Thermotherapy involves the application of heat directly to the tumor, usually in the form of infrared radiation. It is also used for small tumors.

Laser photocoagulation

Laser photocoagulation is recommended only for small posterior tumors. An argon or diode laser or a xenon arc is used to coagulate all the blood supply to the tumor.

Cryotherapy

Cryotherapy induces damage to the vascular endothelium with secondary thrombosis and infarction of the tumor tissue by rapidly freezing it. Cryotherapy may be used as primary therapy for small peripheral tumors or small recurrent tumors previously treated with other methods.

Systemic chemotherapy

Systemic chemotherapy has become forefront of treatment in the past decade, in search of globe preserving measures and to avoid the adverse effects of EBR therapy. The common indications for chemotherapy for intraocular retinoblastoma include:

Intra-arterial chemotherapy

Chemotherapeutic drugs are administered locally via a thin catheter threaded through the groin, aorta, and the neck directly into the optic vessels.

Management options of Retinoblastoma

Treatment options for Intraocular tumor[4]
Unilateral retinoblastoma
Bilateral retinoblastoma
Cavitary retinoblastoma
Progressive or recurrent intraocular retinoblastoma
Treatment options for Extraocular tumor[4]
Orbital and locoregional retinoblastoma
CNS disease
Trilateral retinoblastoma
Extracranial metastatic retinoblastoma
Progressive or recurrent extraocular retinoblastoma

References

  1. Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13, 2015
  2. Shields CL, Shields JA (1999). "Recent developments in the management of retinoblastoma". J Pediatr Ophthalmol Strabismus. 36 (1): 8–18, quiz 35-6. PMID 9972509.
  3. MacCarthy A, Bayne AM, Brownbill PA, Bunch KJ, Diggens NL, Draper GJ, Hawkins MM, Jenkinson HC, Kingston JE, Stiller CA, Vincent TJ, Murphy MF (June 2013). "Second and subsequent tumors among 1927 retinoblastoma patients diagnosed in Britain 1951-2004". Br. J. Cancer. 108 (12): 2455–63. doi:10.1038/bjc.2013.228. PMC 3694232. PMID 23674091.
  4. 4.0 4.1 "Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute".

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