Pediatric Retinoblastoma Staging

After a diagnosis is made, doctors will perform pediatric retinoblastoma staging to determine the size and scope of the cancer. Pediatric retinoblastoma staging will help to determine whether or not the cancer has spread beyond the initial tumor site, such as to the brain or bone marrow. There are several systems in place for pediatric retinoblastoma staging. After pediatric retinoblastoma staging takes place, doctors can build a treatment plan that is specific to each patient’s case.

Basic Pediatric Retinoblastoma Staging

The primary goal of pediatric retinoblastoma staging is to determine if the cancer is localized to the original tumors in the retina, or if it has spread elsewhere. When the cancer spreads, the disease is often more advanced. As a result, treatment may be more difficult. Pediatric retinoblastoma staging can help doctors decide which treatment plan would be most effective depending on the stage of the cancer.

In its most basic form, pediatric retinoblastoma staging is based on three categories:

  1. Intraocular retinoblastoma, which is still contained in the eye
  2. Orbital retinoblastoma, where the cancer has spread to the child’s eye socket
  3. Metastatic or extraocular retinoblastoma, during which the cancer has spread to more distant areas of the body

Reese-Ellsworth Staging System

The Reese-Ellsworth staging system was developed in the 1960s for staging intraocular retinoblastoma, or retinoblastoma which has not yet spread. This system divides intraocular retinoblastoma into five groups. In more advanced pediatric retinoblastoma stages, it is more likely that the child will experience vision damage or loss. These five groups discuss the likelihood that each patient’s eye and vision will be preserved.

The Reese-Ellsworth system classifies the following:

  • Group 1: very favorable for eye preservation. Tumors are smaller and located toward the back of the eye.
  • Group 2: favorable for eye preservation. Tumors are smaller, but may be toward the middle of the eye.
  • Group 3: doubtful for eye preservation. Tumors are toward the front of the eye, or larger tumors are toward the back of the eye.
  • Group 4: unfavorable for eye preservation. Tumors are large or extending toward the front of the eye.
  • Group 5: very unfavorable for eye preservation. More than half of the patient’s retina is involved. Vitreous seeing may occur. Vitreous seeding involves the spread of the tumor into the vitreous humor, or gelatinous material which fills the eye.

AJCC Pediatric Retinoblastoma Staging

The American Joint Commission on Cancer (AJCC) also developed a pediatric retinoblastoma staging system. This system observes three main points of interest while determining the cancer’s stage. The AJCC system discusses both intraocular and extraocular retinoblastoma.

The three keys to the AJCC system are T, N, M:

  1. Tumor: the primary tumor’s size and its growth within and outside the eye
  2. Nodes: whether or not cancerous cells reached the patient’s lymph nodes, or the small collections of immune cells in the body
  3. Metastasizing: whether or not it has metastasized, or spread to other areas such as the skull, brain, bone marrow, or long bones


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