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==Medical therapy for intraocular tumors==
==Medical therapy for intraocular tumors==
The priority of retinoblastoma treatment is to preserve the life of the child, then to preserve vision, and then to 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 paediatric oncologist. Children with involvement of both eyes at diagnosis usually require multimodality therapy ([[chemotherapy]] and local therapies).<ref name="wiki">  Retinoblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Retinoblastoma Accessed on October 10 2015</ref>  
The priority of retinoblastoma treatment is to preserve the life of the child, then to preserve vision, and then to 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 paediatric oncologist. Children with involvement of both eyes at diagnosis usually require multimodality therapy ([[chemotherapy]] and local therapies).<ref name="wiki">  Retinoblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Retinoblastoma Accessed on October 10 2015</ref>  
The goals of therapy are the following:<ref name="NIH">  Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13 2015</ref>  
The goals of therapy are the following:<ref name="NIH">  Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13, 2015</ref>  
*Eradicate the disease to save the patient's life
*Eradicate the disease to save the patient's life



Revision as of 18:19, 18 October 2015

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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 optimal therapy for retinoblastoma depends on several factors such as tumor size, tumor location, presence or absence of vitreous or subretinal seeds, and patient age. The various treatment modalities for retinoblastoma include enucleation, external beam radiation therapy, radioactive plaques (I-125 brachytherapy), cryotherapy, laser photocoagulation, thermotherapy, and chemotherapy (which includes systemic, intra-arterial, and intravitreal).[1]

Medical therapy for intraocular tumors

The priority of retinoblastoma treatment is to preserve the life of the child, then to preserve vision, and then to 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 paediatric oncologist. Children with involvement of both eyes at diagnosis usually require multimodality therapy (chemotherapy and local therapies).[1] The goals of therapy are the following:[2]

  • Eradicate the disease to save the patient's life
  • Preserve as much vision as possible
  • Decrease risk of late sequelae from treatment, particularly subsequent neoplasms

The choice of therapy depends upon several factors such as:[3]

  • Tumor size
  • Tumor location
  • Presence or absence of vitreous or subretinal seeds
  • Patient age

The various treatment modalities 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 (EBR)

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.

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.

Patient Group Treatment Line Treatment
With vitreous seeding
With gross anterior chamber involvement or neovascular glaucoma or orbital inflammation First Enucleation
Adjunct Postoperative systemic chemotherapy
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
Without vitreous seeding
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
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
Metastatic disease First Multimodal therapy
Recurrence
Post globe-salvaging therapy First Brachytherapy
Post enucleation First External beam radiation therapy
Adjunct Systemic chemotherapy

References

  1. 1.0 1.1 Retinoblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Retinoblastoma Accessed on October 10 2015
  2. Retinoblastoma Treatment. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#section/_53 Accessed on October 13, 2015
  3. 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.

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