Familial retinoblastoma: where and when?

Acta Ophthalmol Scand. 1998 Jun;76(3):334-8. doi: 10.1034/j.1600-0420.1998.760316.x.

Abstract

Purpose: To determine when patients with a family history of retinoblastoma and previously normal eye exam develop intraocular disease, and where the new retinoblastoma tumors occur.

Methods: A retrospective chart review of retinoblastoma patients.

Results: Sixty-two percent of the first eyes (eyes having a previously normal examination) were diagnosed with retinoblastoma by 6 months of age, 90% by 12 months and 100% by 28 months. For the second eye, 27% were identified by 6 months, 64% by 12 months, 91% by 30 months and 100% by 44 months. The younger the age at initial diagnosis of retinoblastoma, the greater the likelihood that tumors will initially be found in the posterior pole. Macular tumors were diagnosed very early (mean 4 months) and once a retinoblastoma focus had appeared in one eye no new tumors developed in the macula of either eye.

Conclusion: The timing, location, and number of new retinoblastoma tumors follows a predictable pattern.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age of Onset
  • Child, Preschool
  • Humans
  • Infant
  • New York / epidemiology
  • Retinal Neoplasms / epidemiology*
  • Retinal Neoplasms / genetics
  • Retinal Neoplasms / pathology
  • Retinoblastoma / epidemiology*
  • Retinoblastoma / genetics
  • Retinoblastoma / pathology
  • Retrospective Studies