Retinoblastoma is a cancer of the eye that begins in the retina. The retina is the light-sensitive lining on the inside of the eye that communicates with the brain via the optic nerve. Retinoblastoma most commonly affects young children and rarely occurs in adults.The survival rate of retinoblastoma: The 5-year survival rate for children with retinoblastoma is 96%. Usually, nine out of 10 children with retinoblastoma can be cured. The survival rate depends on whether the cancer has spread from the eye to other parts of the body. The long-term survival rate is much higher if the tumor has not spread beyond the eye.
What causes retinoblastoma?
Retinoblastoma occurs when there are genetic mutations in the nerve cells in the retina. The mutations cause the cells in the retina to grow and multiply rapidly. These cells accumulate forming a tumor. Retinoblastoma cells can invade into the eye and destroy the structure of the eye. The structures around the eye get damaged by the tumor. The tumor can spread to other parts of the body (metastasis) and can involve the brain and spine. The exact cause of the genetic mutations causing retinoblastoma is unknown, but it's most likely to be inherited.
Hereditary retinoblastoma is an autosomal dominant genetic disorder that is inherited. This means either one of the parents needs to pass on only a single copy of the mutated gene to the child to increase the risk of retinoblastoma in the child. If anyone of the parents carries a mutated gene, there is a 50% chance that the child will be inheriting the gene. Though the genetic mutation increases a child's risk of retinoblastoma, it does not mean the child would definitely develop cancer. Hereditary retinoblastoma usually occurs in both eyes. Children who inherit hereditary retinoblastoma may be at risk of developing other cancers.
What are the signs and symptoms of retinoblastoma?
Since retinoblastoma usually affects infants and small children, they are usually unable to explain their symptoms. The following signs in the child can indicate retinoblastoma:
- White color in the pupil (the dark circular portion within the iris or the colored part of the eye) when light is shone over the eye or while taking a flash photograph.
- The eyes appear to be looking in different directions
- Redness of the eye
- Swelling of the eye
How is retinoblastoma treated?
The treatments of retinoblastoma depend on the size, location, and extent of the tumor, as well as the overall health of patients and their preferences. Treatment options include:
Chemotherapy: Chemotherapy is a medical treatment that uses medication to kill cancer cells. They may be taken orally or intravenously. Chemotherapy is also beneficial for tumors that have spread beyond the eyeball or to other parts of the body. Intravitreal chemotherapy is a type of chemotherapy where the medications are directly injected into the eyes.
Radiation therapy: Radiation therapy involved using high-energy radiation beams, such as X-rays and protons, to kill cancer cells and shrink the tumor.
Laser photocoagulation or laser photocoagulation: In laser therapy, a laser is used to destroy blood vessels. This cuts off the supply of oxygen and nutrients to the tumor, killing the tumor cells.
Cold treatments (cryotherapy): Cryotherapy involves using substances, such as liquid nitrogen, at an extremely low temperature to freeze and thaw the cancer cells repeatedly to kill cancer cells.
Surgery: Large tumors that can’t be treated by other methods require surgery. Surgery for retinoblastoma includes:
- Enucleation: This surgery involves the removal of the affected eyeball. Part of the optic nerve, which is the nerve extending from the back of the eye and into the brain, is also removed.
- Surgery to place an eye implant: Immediately after enucleation surgery, the surgeon places a special implant made of plastic or other materials in the eye socket. The eye implant is connected to the muscles around the eye that control eye movement. The implant does not provide vision but moves like a natural eye.
- Fitting an artificial eye: A few weeks after surgery and as the eye heals, a custom-made artificial eye is placed over the eye implant, which matches the healthy eye. The artificial eye is clipped onto the eye implant. The artificial eye moves like the natural eye. Vision is not restored but most children adapt to having vision in only one eye, without any significant discomfort.
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