Retinoblastoma
From Wikipedia, the free encyclopedia
|
| Leukocoria of right eye indicating presence of tumor | |
| ICD-10 | C69.2 |
| ICD-9 | 190.5 |
| ICD-O: | M9510/3 |
| OMIM | 180200 |
| DiseasesDB | 11434 |
| MedlinePlus | 001030 |
| eMedicine | oph/346 |
Retinoblastoma 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 Rb-1 protein. It occurs mostly in children before the age 5 years and accounts for about 3% of the cancers occurring in children younger than 15 years. However, adults cases have also been clinically recorded[1]. The estimated annual incidence is approximately 4 per million children [2].
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.
[edit] Treatment
In former times the only treatment was to remove the affected eyeball before the cancer spread. This resulted all too often in the distressing situation of a small child having to be taken to see all the beautiful things possible before his/her sight vanished for ever. Nowaways treatment by fastening a small radioactive pellet to the outside of the eyeball over the growth will kill the growth and save most of the eye's sight, if the growth is detected in time. Other possible treatments include chemotherapy (the use of chemicals to reduce the tumour), laser therapy (to destroy small tumours using laser light) and cyotherapy (using cold temperatures to destroy tumours).
[edit] References
- Friend SH, Bernards R, Rogelj S, Weinberg RA, Rapaport JM, Albert DM, Dryja TP. A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature 1986;323:643-6. PMID 2877398.
- Knudson AG Jr. Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci U S A 1971;68:820-3. PMID 5279523.
- Retinoblastoma in a 26-year-old adult. Takahashi T, Tamura S, Inoue M, Isayama Y, Sashikata T. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6856254&dopt=Citation
