Wednesday, March 4, 2009

Retinoblastoma

Retinoblastoma is a type of intraocular cancer that occurs most often in children. Only about 300 children are diagnosed in the US per year. Basically it forms in the retina and is caused by a mutation in the genes. The chemicals in our DNA are vital to how our cells will function. For example, a chemical called ‘oncogenes’ tell cells how fast to divide. Then there is a chemical known as the ‘tumor suppressor’ chemical. Mutations in these genes can tell them to do the opposite, this turning off the ‘tumor suppressor’ chemical, which can very easily cause cancers.

Retinoblastoma itself affects the tumor suppressor gene Rb. This is the gene that stops uncontrolled cell growth. So when that gene becomes ineffective, if unchecked the cells can then very easily become cancerous.

Nearly three-fourths of children who are diagnosed have a white pupil, also known as leukocoria. They may also have symptons such as poorly aligned eyes are red and swollen eyes. These symptoms mentioned in the previous sentence could also be mistaken for other pathologies, such as congenital cataract, or coats disease. The main way to tell the difference is through testing; blood tests, CT’s, digital photography, or biopsy. Though biopsy is a 100% effective, doctors tend to veer away with this form of diagnosis because it could potentially spread the cancer cells elsewhere.

Since the nineteen seventies doctors and scientist have developed treatments that have gone from total eye removal, to chemo-based therapy treatments, or radiotherapy. The chemo can shrink them and from there they can use laser therapy or freezing therapy.

Notice in the picture below of a axial CT image the calcification.
http://www.eyecancer.com/ViewImage.aspx?sImgSource=2005719183420.gif&sDesc=A+CT+scan+of+a+retinoblastoma+demonstrates+calcification+within+the+right+eye+(arrow).


These are "seeds" of the disease showing through the iris.
http://www.eyecancer.com/ViewImage.aspx?sImgSource=2005719183229.gif&sDesc="Seeds"+of+retinoblastoma+have+migrated+onto+the+iris+surface.
This is the white pupil appearence 75% of children with this disease get.

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