According to the National Cancer Institute retinoblastoma is a rare cancer that occurs in only about 300 children and adolescents aged less than 2- years in the United States.

Derek Fisher had a tough decision to make. The usual treatment for retinoblastoma is to remove the affected eye, but Fisher opted to go for an innovative chemotherapy treatment pioneered by Dr. David Abramson and Dr. Pierre Gobin of the Memorial Sloan-Kettering Cancer Center. The doctors told The New York Times that the treatment had been tried in just 14 cases, but they opted to try intra-arterial chemotherapy in Tatum Fisher's case as well. The prognosis is guarded and there are many more appointments for the tiny tot before she can be pronounced cancer free.

Newslocale.org is attempting to throw light on this rare cancer of the eye through the following questions and answers

What is retinoblastoma?
Retinoblastoma is a cancer that develops in the tissues of the retina. The retina is the light-sensitive layer of nervous tissue located on the back of the eye. It has elements called rods and cones that are essential for vision. According to the National Cancer Institute, retinoblastoma usually occurs in children younger than 5 years.

What are the important statistics related to retinoblastoma?
Retinoblastoma tumors occur in very young children. The National Cancer Institute says 63 percent of the cases develop in children younger than two years of age and almost 93 percent of the cancer develops in children under five years of age.

Furthermore the incidence of the cancer appears to be evenly distributed among the genders. In males retinoblastoma occurs in 3.7 cases per million, while in females it occurs in 3.8 cases per million children. Among whites retinoblastoma is seen in 3.7 cases per million and it occurs in 4.0 cases per million children among blacks.

Is retinoblastoma inherited?
Yes, retinoblastoma is inherited and a gene called RB1-gene is mutated in the condition. This gene codes for the Rb-1 protein. A mutation in the gene inactivates two copies of the protein leading to the development of retinoblastoma. The gene is located on long arm of chromosome 13.

Every child of a parent having developed the condition has a 50% risk of inheriting the retinoblastoma gene. And tin children those who inherit the gene, there is a 90 percent chance of developing the cancer. Another notable condition, according to the NCI, is the sporadic heritable retinoblastoma, wherein a new mutation predisposes the child to the risk of developing the cancer.

Does retinoblastoma occur in both eyes?
Bilateral retinoblastomas occur during the first year of life and are directly caused by the genetic mutation. Among children aged less than a year, 42 percent of retinoblastomas are bilateral, while the incidence drops to 21 percent after one year of age. In older children, retinoblastoma occurs in both eyes in just 9 percent of cases.

What are the complications of retinoblastoma?
Retinoblastoma can spread to the brain because of its closeness to the optic nerve.

How is retinoblastoma treated?
The most common method is to remove the affected eye. However there are several alternatives like laser therapy, chemotherapy and radiation therapy to conserve the eye.

What is the new treatment being discussed in Tatum Fisher's case?
Dr. David Abramson and Dr. Pierre Gobin of the Memorial Sloan-Kettering Cancer Center have developed a new treatment called intra-arterial chemotherapy, which can save the eye. It involves injecting a high dose of chemotherapy into the artery of the retina.

Is the treatment safe and effective?
At this point, the treatment has only been tried in 14 cases and as yet the doctors have not detailed it anywhere. So it is difficult to know if the treatment is safe and effective.

What are the survival rates in retinoblastoma?
The prognosis is quite favorable with 93 percent five-year survival rate among children. Males and females have had equal survival rates according to the NCI.

The above Q&A is for inormational purposes only. Users are advised to consult their doctors for more information. 

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