Q. How is retinoblastoma treated?

A. 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.

Q. What are the complications of Retinoblastoma?

A. Apart from loss of vision and systemic spread of the disease, it has been found that 5% to 15% of children with either familial, multifocal or bilateral Retinoblastoma may develop an intracranial neuroblastic tumor (pinealoma). Also, patients with germline type of tumor may have increased risk of second malignant neoplasms e.g. osteosarcomas, soft tissue sarcomas & melanomas. Children with germline tumor may develop bilateral disease even if only one eye is involved at presentation. Hence, they should be examined frequently for the development of new Retinoblastoma tumors. It has been recommended that they should be examined every 2 to 4 months for at least 28 months and following treatment followed up until 7 years of age.

Q. How is Retinoblastoma prevented?

A. All siblings of patients with Retinoblastoma should be examined periodically and DNA polymorphism analysis should be done.

Q. How is a relapse treated?

A. If the relapse is confined to the eye and tumor is small, it can be treated with local therapy alone. The prognosis for vision and survival is excellent.

If relapse is confined to the eye, but is extensive, treatment with etoposide & carboplatin can be tired. The prognosis for vision is poor but survival remains excellent. If relapse is extraocular, the prognosis is less than 50%.

Last created on 13-07-2001
Last updated on 01-07-2006