is a cancer
of the retina
. Development of this tumor is initiated by mutations
that inactivate both copies of the RB1
gene, which codes for the retinoblastoma protein
Until recently the only treatment was to remove the affected eyeball before the cancer spread. Chemotherapy is the treatment of choice for most unilateral cases. However, with locally advanced disease, external beam radiation may be needed, and if both eyes are involved, enucleation
may be the only option. Affected children in developing countries present with advanced features and usually die of metastatic spread. In its initial stages, retinoblastoma is very similar to Coats' disease
, a non-cancerous retina disease. Coats' disease should be ruled out before enucleation is done. A mis-diagnosis of retinoblastoma accounts for the greatest number of Coats' disease eyes being enucleated.
Many children with bilateral retinoblastoma can be treated with a preservation attempt. Tumor chemoreduction with carboplatin and other drugs may reduce the tumor volume making them amenable to local therapies
Local therapies include-
- Laser therapy (Uses infrared laser light to precisely destroy the blood vessels surrounding a tumor.)
- Cryotherapy (use of a cold gas which is injected into the affected part of the retina to shrink the tumor.)
- Thermotherapy (A relatively new technique used mainly in new testing. It uses the principle that if heat is applied to the affected area, a tumor will sustain more damage than healthy cells because healthy cells can cool themselves better using healthy surrounding blood vessels. If this technique is not immediately successful it may increase the efficacy of other treatments such as chemotherapy and focused radiation plaques.)
- Radiotherapy (Generally used as a last resort, radiotherapy was previously the treatment of choice before the above mentioned treatments were developed. Radiotherapy destroys cancerous growths using gamma radiation or x-ray radiation but it carries with it many drawbacks, including:-
- Possibility of secondary cancerous growths which present themselves months or years later.
- Destruction of healthy cells in the area surrounding the treated tumor.
- Bone deformation due to the destruction of the growth plates mainly in the area of the temple.)
It is important that children with retinoblastoma are treated in specialist centers.
It is considered to be one of the most common inherited cancer syndromes.
Brachytherapy with beta-emitting eye applicators have also been a successful major treatment. BEBIG (GmbH-Berlin-Germany) produces various kinds of ruthenium ophthalmic applicators for treating retinoblastoma.
In October 2007, researchers identified the specific cell that causes retinoblastoma.