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Retinoblastoma in Children

Retinoblastoma in Children

Sheba Medical Center in Israel offers comprehensive care to patients diagnosed with retinoblastoma. As the largest hospital in the region, Sheba provides access to a multidisciplinary team of highly skilled medical professionals and pioneering treatments. Our approach encompasses all aspects of patient care, ensuring exceptional treatment for individuals from around the globe.

Patients with Retinoblastoma receive specialized care from a team of experts who have received training at esteemed cancer centers worldwide. Recognizing that each case is unique, our experienced doctors collaborate to accurately diagnose, stage and develop personalized treatment plans for every patient.

Retinoblastoma in Children


Retinoblastoma is an eye cancer that begins in the retina — the light-sensitive nerve tissue lining the inside of your eye. The retina sends signals through your optic nerve to your brain, where these signals are interpreted as images. It is the most common type of eye cancer in children, comprising about 2% of all pediatric cancers. Fortunately, due to advanced diagnostics and modern treatments, more than 95% of children with retinoblastoma can now be cured.


The eyes begin to develop in utero, when cells called retinoblasts rapidly multiply to create new cells for the retina. After this early stage of the eye’s development, retinoblasts cease to reproduce and instead grow into mature retinal cells. When this process goes wrong and the retinoblasts grow out of control, the result is retinoblastoma. Usually, the course of events that leads to retinoblastoma starts with a mutation in the RB1 gene, which is the gene that prevents cells from growing uncontrollably.

Retinoblastoma occurs when nerve cells in the retina develop genetic mutations. These mutations cause the cells to continue growing and multiplying when healthy cells would normally die. This accumulating mass of cells forms a tumor. Retinoblastoma cells can then invade further into the eye and nearby structures, and at times also spread (metastasize) to other areas of the body, including the brain and spine.

Risk Factors

There are very few known risk factors exist for retinoblastoma, but some possible factors include:
  • Age: Most diagnoses occur before the child is three years old, and rarely after age six.
  • Heredity: Having a parent who had congenital retinoblastoma; however, retinoblastoma may also occur in a child without any family history of the cancer.
  • Parental lifestyle: Exposure to chemicals in diesel or gasoline exhaust during pregnancy; the mother not eating enough fruits and vegetables during pregnancy; exposure of fathers to radiation; older age of the father.


In general, the symptoms of retinoblastoma in children present before age two, and they are noticed most frequently by a parent or doctor.
The most common signs include:
  • Leukocoria: Also called “white pupillary reflex,” is the most typical symptom of retinoblastoma; it refers to when the eye’s pupil looks white when light shines into it (instead of red because of blood vessels at the back of the eye)
  • Nystagmus: Involuntary eye movements
  • Different colors in each iris
  • Eye pain
  • Strabismus: A problem with eye teaming, also called “lazy eye”
  • Redness of the white part of the eye or around the eye
  • Weak vision or a change in vision


No screening tests are in practice for retinoblastoma, namely because it is so rare.

However, if retinoblastoma is suspected, doctors may perform a thorough physical examination of the eyes and body. In addition, doctors will also ask about a family history of retinoblastoma and other types of cancer. Family history is important because it can help to determine if other relatives could pass the RB1 gene mutation to their children. If the exam points to a possible diagnosis of retinoblastoma, imaging tests will be performed to confirm it.

When a diagnosis of retinoblastoma is confirmed, genetic testing is helpful for determining if the retinoblastoma is heritable or not. Non-heritable retinoblastoma typically arises sporadically, without being passed down through family genetics. Knowing which form of the disease a child has is significant because it can provide information about their risk of developing other cancers.

Bilateral retinoblastoma refers to the presence of tumors in both eyes, indicating a higher risk for the hereditary form of this eye cancer, while Unilateral retinoblastoma is the development of tumors in only one eye, typically indicating a lower risk for hereditary factors.


Retinoblastoma is classified into three main stages:
  • Intraocular retinoblastoma: The cancer is limited to the eye.
  • Extraocular retinoblastoma: The cancer has spread beyond the eye. Orbital retinoblastomas are when cancer has only moved into the eye socket, versus metastatic retinoblastomas, which have spread to other distant organs.
  • Recurrent retinoblastoma: The cancer has returned – either to the eye or in other body parts. The stage of retinoblastoma is strongly associated with the prognosis for children who have this cancer. Staging is also significant for designing the most effective treatment program.

Possible Complications

When left untreated, retinoblastoma tumors can grow to fill most of the eyeball. If cells break away from the main tumor on the retina, they can form additional tumors on other parts of the eye. Over time, retinoblastoma can also spread beyond the eyeball to lymph nodes and other organs. However, the vast majority of cases are detected and treated when retinoblastoma is present only in the eyeball.


While it is the most common type of ocular cancer in children, the cure rate is fortunately very high due to the development of precise, modern diagnostics and state-of-the-art medical treatments. The goals of retinoblastoma treatment are to destroy the cancer cells, preserve the vision if possible and limit any long-term effects caused by cancer. The treatments options are:
  • Surgery (enucleation) - While it is generally not needed for small retinoblastomas, in cases where the tumor has grown before being detected and if other treatments fail and there is irreversible damage to vision, an enucleation (surgery to remove the entire eye) will be needed.
  • Laser therapy - There are different types of laser therapy that may be used for small retinoblastoma tumors, such as photocoagulation and transpupillary thermal therapy.
Get more information about the benefits of treating Retinoblastoma in Israel.


At present, there are no known avoidable risk factors for retinoblastoma. In adults, the risk for any cancer can be reduced by avoiding certain risk factors, such as smoking.

In children, however, there are no risk factors that aren’t hereditary.

Prognosis and Survival Rate

Retinoblastoma can be fatal if left untreated, so early diagnosis can save both a child’s life and vision. Fortunately, the five-year survival rate is very high (96.5% with treatment) due to the development of precise, modern diagnostics and state-of-the-art treatments. A treatment plan for retinoblastoma must take into consideration the tumor’s type, size and location, and may include laser therapy (photocoagulation or thermotherapy), chemotherapy, radiation therapy and surgery.

Learn about baby Stephen, who received life-saving treatment at Sheba that cured his Bilateral retinoblastoma.

Our Experts

While retinoblastoma is the most common intraocular malignancy in children, it is still a relatively rare malignancy, as according to the latest estimations only 8,000 new cases are diagnosed every year. Therefore, choosing a hospital with state-of-the-art technologies and expert specialists is critical.

The Ocular Oncology Center at Sheba Medical Center offers advanced treatment for retinoblastoma. Our center is a national and international referral center for the diagnosis and treatment of various ocular tumors and associated conditions. Working in close collaboration with the experts at our Goldschleger Eye Institute, the largest facility for ocular medicine in Israel, we strive to preserve the overall health and vision of adult and pediatric patients with retinoblastoma. Sheba’s compassionate team of retinoblastoma experts will ensure you and your family are well-informed throughout your journey to recovery.

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Sheba Medical Center provides innovative, personalized medical care to patients from around the world. We are the largest, most comprehensive hospital in the Middle East and dedicated to providing advanced and compassionate medicine for everyone. We welcome all cases, including the rarest and the most challenging. Our medical teams collaborate to provide the best possible health outcomes. From your initial inquiry through the long-term follow-up care, we are here for you.

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