RETINOBLASTOMA

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Last Updated : 1/3/2011
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Bharat R Agarwal
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Tumor Staging

For the purpose of treatment, retinoblastoma is divided into intraocular and extraocular disease.
Intraocular: Tumor is localized to the eye, it does not extend beyond the eye or to other parts of the body.
Extraocular: Tumor extends beyond the eye. It may be limited to the tissue around the eye, or it may spread to the CNS or other parts of the body.

Reese and Ellsworth have classified intraocular Retinoblastoma as follows:
Group 1 : very favorable for maintenance of sight.
- Solitary tumor, smaller than 4 disc diameters, all at or beyond the equator.
- Multiple tumors, none bigger than 4 disc diameters, all at or beyond the equator.
Group 2 : favorable for maintenance of sight.
- Solitary tumor, 4-10 disc diameter in size, at or beyond the equator.
- Multiple tumors, 4-10 disc diameter in size, behind the equator.
Group 3: Possible for maintenance of sight.
- Any lesion anterior to the equator
- Solitary tumor larger than 10 disc diameter, posterior to the equator.
Group 4: Unfavorable for maintenance of sight
- Multiple tumors, some larger that 10 disc diameter in size
- Any lesion extending anterior to the ora-serrata.
Group 5: Very unfavorable for maintenance of sight
- Massive tumors involving more than one half of the retina.
- Vitreous seeding
(About 90% of patients present with Group5 disease in one or both the eyes)

Treatment
The goals of therapy are:
- To cure the disease
- To preserve as much sight as possible

I]

TREATMENT FOR INTRAOCULAR RETINOBLASTOMA


The various options are:
- Enucleation: if tumor is large or useful vision is not expected.
- External beam radiation between 3500 to 4600 cGY, delivered in 17-25 fractions over 4-5 week period.
- Cryotherapy: For lesion smaller than 4-disc diameter in the anterior portion of retina, in addition to radiation or in place of photocoagulation.
- Laser Photocoagulation: For posteriorly located tumors smaller than 4-disc diameter distinct from the optic nerve head and macula.
- Systemic Chemotherapy: To reduce tumor load and avoid long-term effects of radiation therapy for patients with intraocular tumors not amenable to cryotherapy or photocoagulation alone.

Unilateral disease
- Enucleation: It is the standard therapy as most unilateral tumors are massive.
- Radiation, cryotherapy, photocoagulation, systemic chemotherapy are useful for smaller size tumors.

Bilateral disease
- Since one eye may be more severely involved as compared to the other eye, the standard treatment is to enucleate the more involved eye. However, if there is a potential for sight in both eyes, bilateral irradiation with close follow-up for response is indicated.
- Systemic chemotherapy followed by cryotherapy/photocoagulation is still experimental.

II] TREATMENT FOR EXTRAOCULAR RETINOBLASTOMA
There is no clear-cut therapy, however orbital irradiation with chemotherapy may be useful with/without intrathecal methotrexate.

Prevention
All siblings of

patients with Retinoblastoma

should be examined periodically and DNA polymorphism analysis should be done.



Contributor Information and Disclosures Bharat R Agarwal
Pediatric Hematologist- Oncologist, Division of Pediatric Hem-Onco, B.J. Wadia Hospital for Children, Mumbai, India


First Created : 1/3/2001
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