Pediatric Eye Care

Pediatric retinoblastoma is a rare eye cancer that develops in children. It is estimated that kids’ retinoblastoma accounts for roughly 3 percent of all cancers in children under 15. Kids’ retinoblastoma is caused by a certain genetic defect that may be hereditary or non-hereditary. This type of eye cancer may develop in one or both eyes.

Types of Kids’ Retinoblastoma

Kids’ retinoblastoma occurs in two main forms: hereditary and non-hereditary. Hereditary retinoblastoma occurs when the child inherits a genetic mutation from one or both parents. It is estimated that roughly 40 percent of kids’ retinoblastoma cases are hereditary. Non-hereditary retinoblastoma is caused by an isolated genetic mutation that was not inherited by either of the child’s parents.

Unilateral and Bilateral Retinoblastoma

Additionally, kids’ retinoblastoma may develop in one or both eyes. Kids’ retinoblastoma in one eye is referred to as unilateral retinoblastoma. Kids’ retinoblastoma in both eyes is referred to as bilateral retinoblastoma.

Kids’ Retinoblastoma Symptoms

Kids’ retinoblastoma symptoms may include:

  • Leukocoria, or white pupils
  • Strabismus, or misaligned eyes
  • Red and swollen eyes
  • Poor vision
  • Different-colored irises
  • Pain in the affected eye

Kids’ Retinoblastoma Causes

In simple terms, kids’ retinoblastoma is caused by the uncontrolled division of cells called retinoblasts. Retinoblasts are immature cells of the eye’s retina. The retina is the back inner portion of the eyeball that plays a significant role in vision. When these retinoblasts reproduce in large numbers, the excess cells form retinoblastoma tumors.

Genetic Causes

Like other forms of cancer, kids’ retinoblastoma has a genetic component linked to the cause. Research shows that kids’ retinoblastoma is linked to a gene called RB1. RB1 is a tumor suppressor gene. This means that RB1 is designed to control the division of cells, which in turn prevents the development of tumors. Each cell in the body contains two copies of the RB1 gene, one copy from each parent. Kids’ retinoblastoma develops when at least one retinoblast, or immature retina cell, experiences a mutation in both copies of its RB1 gene.

Kids’ Retinoblastoma Complications

In advanced forms, kids’ retinoblastoma can cause vision problems. Vision may be restored if proper treatment is given to kill the cancer and reverse vision loss. If the child does not receive proper and timely retinoblastoma treatment, blindness can occur.

Retinoblastoma Prognosis

Kids’ retinoblastoma prognosis is typically dependent on how early the cancer is diagnosed and treated. The National Cancer Institute states that roughly 95 percent of kids’ retinoblastoma cases are diagnosed at age five or younger. Two-thirds of these cases receive diagnosis at age two or younger. Generally speaking, the later the retinoblastoma diagnosis, the more time the cancer has to potentially grow and spread. Therefore, later diagnosis is typically associated with poorer prognosis.




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Benedict, William F., et al. “Gene for hereditary retinoblastoma assigned to human chromosome 13 by linkage to esterase D.” Science 219 (1983): 971+. Academic OneFile. Web. 29 Nov. 2013.

de Francesco, Sonia, Theodora Hadjistilianou, and Domenico Mastrangelo. “Age at diagnosis and bilateralization of unilateral retinoblastoma.” The Internet Journal of Ophthalmology and Visual Science 26 Aug. 2006.Academic OneFile. Web. 29 Nov. 2013.

Dommering, Charlotte J., et al. “RB1 mutations and second primary malignancies after hereditary retinoblastoma.” Familial Cancer. 11.2 (2012): 225-233. Print.