What is Retinoblastoma?

Retinoblastoma is a rare form of malignancy that affects the eye's retina. Frequently diagnosed in children, retinoblastoma carries a high risk for recurrence in the wake of remission and can increase a child’s chances for developing other forms of cancer later in life. Treatment for retinoblastoma is entirely dependent on the location, staging and size of the neoplastic growth and may include the administration of various therapies, such as chemo, radiation and laser. Some cases of retinoblastoma may necessitate surgery to remove the affected eye and prevent the cancer from metastasizing throughout the body.

Originating as a cell mutation within the retina, retinoblastoma occurs when unchecked cell growth contributes to the formation of a malignant neoplasm, or tumor. There is no established, definitive cause for the rapid cell growth that leads to the development of this form of eye cancer. Some medical organizations, such as the Mayo Clinic, have asserted that there may be a genetic factor that contributes to the development of some cases.

A diagnosis of retinoblastoma is often established following the administration of an eye exam and imaging testing. Following a complete eye exam, the ophthalmologist may recommend imaging tests, including magnetic resonance imaging (MRI), to determine the extent of the malignancy and whether it has become invasive to surrounding tissues. Once a diagnosis has been confirmed, additional testing may be performed by specialists to determine the staging of the cancer and determine an appropriate course of treatment.

Individuals who develop retinoblastoma may exhibit physical signs including eye swelling and redness and pupil discoloration. It is not uncommon for symptomatic individuals to lose the ability to control their eye movements, such as the sudden development of involuntary eye crossing. Additionally, he or she may develop eye pain and experience pronounced changes in his or her vision.

Treatment often involves the administration of a variety of drug therapies to shrink the tumor and inhibit its growth. Depending on the location, size and staging of the neoplastic growth, chemo and radiation therapies are generally administered as initial treatment. Chemotherapy involves the administration of anticancer drugs and can cause side effects that include fatigue, nausea, and weight loss. Radiation therapy is the utilization of focused beams of concentrated energy administered directly to the malignant growth. The application of radiation therapy may be accomplished through either internal or external administration and can induce side effects such as irritation at the administration site and fatigue.

Certain cases of eye cancer may necessitate treatment beyond the traditional application of chemo and radiation therapies, such as the use of laser, cryo- or thermo-therapies. Each form of therapy uses a certain medium, such as extreme temperature, to eliminate cancer cells and inhibit the growth of new ones. In cases where the neoplastic growth is large or has become invasive to surrounding tissues, surgery may be necessary to remove the eye, a procedure known as enucleation, as a means of preventing the malignancy from metastasizing.