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Retinoblastoma

What is a retinoblastoma?

Retinoblastoma is a type of cancer that affects the cells of the retina of the eyes. In developed countries retinoblastoma has very good cure rate of about 95 to 98%. Approximately 9 out of 10 children suffering from retinoblastoma lead a healthy adult life. There are two forms of retinoblastoma one is hereditaryand other form is not inherited.

In two out of three cases of retinoblastoma single eye is affected and in the remaining cases both the eyes are affected. Children suffering from heritable form of retinoblastoma are at increased risk of suffering from cancer in other parts of the body. Treatment options include anticancer drugs, radiotherapy, heat therapy and surgery,

Retinoblastoma Symptoms

Common symptoms of retinoblastoma are :

  • The pupil of the affected eye appears white and a whitish glow is seen when photographs of the eye are taken with flash. This whitish pupil is known as leukocoria or amaurotic cat’s eye reflex.
  • Poor vision, red eye with raised intraocular pressure (glaucoma) and pain.
  • Growth retardation or delayed development.
  • Sometimes the children suffer from double vision (squint), this condition is known as “cross eyed” or “wall-eyed”.

Retinoblastoma commonly occurs in children less than 6 years of age but more commonly children between 1 and 2 years suffer from this condition. Common complications of retinoblastoma include increased risk of recurrence of retinoblastoma around the same or the other eye. Therefore regular check ups are required. Children having past history of retinoblastoma are also at increased risk of developing cancer in other organs.

Causes of Retinoblastoma

Retinoblastoma is the most common type of eye cancer occurring in children. Retina consists of nerves which sense the light as it passes through the front of the eye and sends this signal through the optic nerve to the brain. In the brain these light signals are interpreted as visual images.

In normal human body older cells are replaced by programmed cell death (apoptosis) and newer cells are produced by cell division. Both these processes of cell division and apoptosis are highly controlled by certain factors. In cancer patients sudden irreversible change in the genetic material (mutation) occur which may lead to complete inhibition of apoptosis and uninhibition of cell division. As a result immortal cells develop with capacity to spread to other organs.

In retinoblastoma mutation in the cells of retina leads to development of cancerous tumor in the eye. The exact cause of mutation is not exactly know,  however, in some patients of retinoblastoma children may inherit the defective gene (RB 1) from parents. Inherited form of retinoblastoma is an autosomal dominant disease where inheritance of single copy of the defective gene will lead to disease manifestation. Inherited variety of retinoblastoma usually affect both the eyes and affects children at early age.

Risk factors

Children with family history of retinoblastoma are at increased risk of suffering from the disease however in many of the children suffering from retinoblastoma there may not be any family history of retinoblastoma.

Retinoblastoma Treatment

Treatment options depend upon the size, location of the tumor along with preference of the child.

  • Chemotherapy: anticancer drugs are used to destroy the cancerous cells.
  • Radiotherapy: radiation is used to kill the defective cells.
  • Laser therapy, cold therapy or heat therapy are the other options.

In case of large tumor surgery is done to remove the diseased eye (enucleation) and placing an eye implant.


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