Retinoblastoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Retinoblastoma is a cancer of the retina. Development of this tumor is initiated by mutations[1] that inactivate both copies of the RB1 gene, which codes for the retinoblastoma protein.[2]
It occurs mostly in children younger than 5 years and accounts for about 3% of the cancers occurring in children younger than 15 years. Adult cases have also been clinically recorded.[3] The estimated annual incidence is approximately 4 per million children.[4] It begins with white blotches in one or both eyes (leukocoria) which can be seen in photographs (this is distinct from the red-eye effect which is normal); or when light reflects off the eye, as when watching television.
The tumor may begin in one or both eyes. Retinoblastoma is usually confined to the eye but can spread to the brain via the optic nerve.
As the retina is the light-sensitive part of the eye necessary for vision, loss of vision occurs.
The condition reached the headlines in May 2007, when Utah Jazz point guard Derek Fisher revealed that his 10-month-old daughter had the condition. Fisher missed Game 1 and half of Game 2 of the 2007 NBA playoff series versus the Golden State Warriors to be with his daughter for her surgery in New York City.
Treatment
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 [5]
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 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.
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.
See Ben Underwood for a case who compensated for the resulting blindness by developing human echolocation.
In October 2007, researchers have identified the specific cell that causes retinoblastoma.[3]
References
- ↑ Knudson A (1971). "Mutation and cancer: statistical study of retinoblastoma". Proc Natl Acad Sci U S A. 68 (4): 820–3. PMID 5279gadgqetqer523 Check
|pmid=value (help). - ↑ Friend S, Bernards R, Rogelj S, Weinberg R, Rapaport J, Albert D, Dryja T. "A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma". Nature. 323 (6089): 643–6. PMID 2877398.
- ↑ Takahashi T, Tamura S, Inoue M, Isayama Y, Sashikata T (1983). "Retinoblastoma in a 26-year-old adult". Ophthalmology. 90 (2): 179–83. PMID 6856254.
- ↑ cancer.org
- ↑ Dunkel IJ, Lee TC, Shi W, Beaverson KL, Novetsky D, Lyden D, Finlay JL, McCormick B, Abramson DH. "A phase II trial of carboplatin for intraocular retinoblastoma". Pediatr Blood Cancer. PMID 17301956.
See also
External links
- The National Cancer Institute's page on Retinoblastoma
- Retinoblastoma International
- retinoblastoma at NIH/UW GeneTests
- Retinoblastoma Genetics
- At What Age Could Screening Be Stopped
- Visual Fields in Retinoblastoma Survivors
- Building on a Legacy of Innovation and Collaboration: Better Treatments for Retinoblastoma
- Abramson D, Frank C (1998). "Second nonocular tumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk". Ophthalmology. 105 (4): 573–9, discussion 579-80. PMID 9544627.
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