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Eye Retinoblastoma



Alternative Name

Tumor - retina; Cancer - retina

Overview

Retinoblastoma is a relatively uncommon tumor of childhood that arises in the retina, the inner layer of the eye that senses light and sends the image to the brain. Approximately 300 hundred children annually are diagnosed with retinoblastoma in the United States. As tumors of early childhood, retinoblastomas are generally diagnosed in children between 1-2 years of age, but can affect newborns and children up to age 5 as well. Once diagnosed, most children with retinoblastomas generally respond well to therapy with minimal to no loss of vision.


Review Date: 7/13/2011

Reviewed By: Chatchawin Assanasen, MD Assistant Professor Pediatric Hematology-Oncology, CTRC



Signs/Symptoms

Most children do not show any signs of having cancer prior to diagnosis. Often, a parent or family member may identify the tumor as a white tint in the pupil of the child eye. Often this is seen in contrast to "red eye" usually observed during flash photography. Sometimes as the tumor grows within the eye it can be observed altering the shape, color, vision, and normal movements of the eye.

Diagnosis

If retinoblastoma is suspected, the doctor will usually refer your child to a pediatric ophthalmologist, an eye doctor who will examine the retina directly by dilating the eye, sometimes under general anesthesia.

Computed Tomography (CT or CAT) scans use X-rays to determine any evidence of both local and distant tumor spread.

Magnetic resonance imaging (MRI) uses electromagnetic waves and strong magnets to produce detailed pictures of the internal parts of the body.

Laboratory tests such as blood and urine tests check the general health of a patient.

As with many forms of childhood cancer, there is a genetic component to retinoblastoma. A gene mutation named RB1 has been highly associated with retinoblastoma tumors.  Children who carry the genetic mutation (from either a parent or grandparent) usually are younger and get more than one tumor and are likely to develop the disease in both eyes. This is called hereditary ("inherited") retinoblastoma. Those who have no family history of the disease are called sporadic and will usually get it in just one eye, with much less risk of developing retinoblastoma in the other eye.  Currently, there are commercially available tests to screen those who may pass the disease onto their offspring. 

Cancer genetics counseling services are conducted at both the Cancer therapy and Research Center (CTRC) and CHRISTUS Santa Rosa Children's Hospital providing comprehensive, and compassionate state-of-the art clinical care to patients and families at high risk for cancer and expert clinical evaluations to patients referred to the University of Texas Health Science Center at San Antonio (UTHSCSA) and its affiliated health programs. Genetics Counseling Services

Treatment

Treatment for Retinoblastoma is determined by many factors, the most important being the stage of the cancer at diagnosis. In the past the Reese-Ellsworth staging system (developed in the 1960's) provided physicians with the tool to determine how best to save the affected eye.  Recently, the National Childhood Cancer Foundation's Children's Oncology Group recently adopted a more modern staging system that considers a child's disease severity and predicts what type of treatment will be most effective while still preserving the eye.  The faculty and staff at UTHSCSA are pleased to continue to be a part of this cooperative effort advancing knowledge and improving cancer therapies for children.

Most treatment plans for retinoblastoma usually include chemotherapy.  The more advanced stages also may require surgery or radiation therapy. Chemotherapy uses drugs (administered either orally or intravenously) to enter the bloodstream, circulate throughout the body, and kill cancerous cells wherever they may be. Radiation uses high-energy X-ray beams to kill specific cells in different areas of the body. (read more about Radiation Oncology)

Both treatments have short-term and long-term risks. Temporary or short-term discomfort may include nausea and vomiting, loss of appetite, mouth sores, fatigue, loss of hair, weakened immune system (leaving a patient vulnerable to infection), and bleeding or bruising.

Other treatment options include:  

  • Brachytherapy: Radioactive material (pellets or plaques) is placed within the tumor to deliver focal radiation to specific areas. This form of treatment minimizes the damage to surrounding healthy tissue.
  • Cryotherapy: extremely cold substances such as liquid nitrogen are used to freeze and destroy diseased tissue.
  • Laser therapy (thermotherapy): Laser energy (through the use of infrared light) heats up cancer cells and surrounding blood vessels, which kills the cells.  Laser energy can also be delivered to blood vessels surrounding the tumor, causing blood clots and depriving the cancer cells of nutrients.
  • Enucleation: In severe cases of retinoblastoma, the entire eyeball is removed to prevent the spread of cancerous cells to other areas of the body.

Long-term effects of therapy may include the development of secondary cancers (like leukemia) or the weakening of some internal organs, such as the kidney.  All pediatric oncology patients at UTHSCSA continue to be evaluated in our Long Term Follow-up Clinic to identify and intervene early on should anything arise.  However, these risks are minimal ― the benefits of treatment far outweigh potential risks.

Pediatric Hematology / Oncology

If you would like to request an appointment with a physician, or if there is a direct referral from a physician, please call:

Appointments: (210) 704-2187

Fax: (210) 704-2396