Types of Pediatric Retinoblastoma

Pediatric retinoblastoma is a type of tumor that grows on the retina of the eye. Pediatric retinoblastoma most commonly affects children under six years of age, and is often developed while in the womb. The first sign that parents usually notice that may indicate that a child has pediatric retinoblastoma is a whitish spot in the pupil. This white spot is often noticeable in pictures. Since the pupil is translucent, this visible white spot is actually a glimpse of the tumor at the back of the eye.

The Two Types of Pediatric Retinoblastoma

The two primary types of pediatric retinoblastoma are hereditary and non-hereditary retinoblastoma. Retinoblastoma may also be unilateral or bilateral. Unilateral retinoblastoma occurs in one eye. Bilateral retinoblastoma is present in both eyes.

Hereditary Retinoblastoma

Cases of heritable retinoblastoma make up about 40 percent of all cases of retinoblastoma. A specific gene is present in hereditary retinoblastoma that helps doctors to identify the disease as such, even if there is no family history of the disease. Hereditary retinoblastoma is often developed very shortly after birth or in the womb.

Hereditary retinoblastoma can affect both eyes, in which case it is referred to as bilateral. Children with heritable retinoblastoma may develop new tumors, so it is important to take the child in for frequent screenings even after successful treatment. The risk of developing new tumors decreases substantially as the child ages.

Non-hereditary Retinoblastoma

Non-hereditary retinoblastoma is also referred to as sporadic retinoblastoma. This disease typically only takes root in one eye, in which case it is referred to as unilateral. Non-hereditary retinoblastoma often develops later in the child’s life than hereditary retinoblastoma, though most patients are still under the age of five. This type of retinoblastoma makes up about 60 percent of retinoblastoma cases. Researchers and physicians do not know what causes non-hereditary retinoblastoma.

Pediatric Retinoblastoma Symptoms and Diagnosis

Once symptoms have been identified, the physician will usually schedule a scan test such as an ultrasound or a CT scan to view the eye and brain. This allows the physician to see the tumor and may help with staging. A complete physical exam will also be conducted to see if the cancer has spread to other parts of the body.

Symptoms that are often noticed during pediatric exams which indicate retinoblastoma include:

  • Whiteness or redness in the pupil
  • Poor vision
  • Pain and redness in the eyes
  • Different colored irises
  • Crossed or floating eyes
  • Noticeably enlarged pupil

Staging Pediatric Retinoblastoma

Once retinoblastoma has been diagnosed, the physician will conduct further tests to determine what stage of retinoblastoma the patient has. The staging system that is used is called the International Classification for Intraocular Retinoblastoma. This system uses the letters A through E to classify the extent of the disease, the higher the letter the worse the condition. Physicians then use this staging to determine the best course of treatment for the patient.


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