Eye Cancer (Retinoblastoma)

Retinoblastoma is a malignant tumor in the retina of a child’s eye. While it’s not common to find hospitals that specialize in this rare cancer, the pediatric ophthalmology specialists at the University of Michigan Kellogg Eye Center do, diagnosing and treating retinoblastoma using the latest treatment options, as well as discovering new treatments to comprehensively care for our patients. These ophthalmologists work in tandem with Kellogg Eye Center’s newest service: the Orbital and Ocular Oncology Service.  They also work with physicians from pediatric hematology-oncology and interventional radiology.

Most often, retinoblastoma is diagnosed early in life, often by a parent or a pediatrician noting a “white pupil”, known as leukocoria – a white pupil due to reflection of light off of a tumor located behind the lens of the eye. Sometimes a parent notices something is wrong when the white pupil is seen in a flash photograph. Retinoblastoma can occur randomly or it can be hereditary. When retinoblastoma occurs in only one eye, it is usually not hereditary. When the disease occurs in both eyes, it is always hereditary.

Once the symptom is noticed, the child is referred to a pediatric ophthalmology specialist at the Kellogg Eye Center. The ophthalmologist will perform a fundus examination, which means examining the interior lining of the eyeball that includes the retina. Once retinoblastoma is diagnosed, the pediatric ophthalmology specialist will order tests to determine the size of the tumor and whether it has spread to other parts of the body. Tests may include an MRI or CT scan, bone marrow test and/or a spinal tap. Our multidisciplinary program combines the efforts of Pediatric Ophthalmology, Ocular Oncology, Radiation Oncology, Hematology/Oncology and the Rogel Cancer Center.

Typically, the survival rate for a child with retinoblastoma is greater than 90 percent. The type of treatment given depends on the extent of the disease within the eye, whether the disease is in one or both eyes, and whether the disease has spread beyond the eye.

Retinoblastoma Treatment

Treatment options focus on both cure and preservation of sight, and include the following:

  • enucleation – surgery to remove the eye when the tumor is large or when vision is permanently lost
  • cryotherapy – the use of extreme cold to destroy cancer cells
  • laser photocoagulation therapy – the use of laser light to destroy blood vessels that feed the tumor
  • chemotherapy, which can be delivered by three routes:
    • systemic (into a vein) when the tumor is large or when it has spread beyond the eye
    • intra-arterial (into a small artery feeding the eye) for treating tumors for which the prospect of preserving vision remains good
    • intra-vitreal (into the fluid within the back part of the eye) for treating tumor cell clusters which are floating within that fluid

Often intra-arterial and intra-vitreal chemotherapies are administered at the same time.

Patients often receive a combination of therapies to treat the tumor(s).

Cutting-edge clinical trials are available to patients who qualify through the Children’s Oncology Group.

Because of the hereditary factor, brothers and sisters of children with retinoblastoma should be examined to find out if they may develop the disease.

Retinoblastoma survivors can also pass this form of cancer on to their children. If your child has retinoblastoma, particularly the hereditary type, there is an increased chance that he or she may develop a second cancer in later years. We offer genetic counseling to answer all your questions about inheritance risks, providing comprehensive evaluation, education, and support.

Take the Next Step

Schedule an appointment by calling the Kellogg Eye Center at 734-764-7558. 

You may also contact the pediatric hematology-oncology clinic at C.S. Mott Children’s Hospital at 734-936-9814.