Familial retinoblastoma: where and when?
OBJECTIVE
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.


CONCLUSIONS
The timing, location, and number of new retinoblastoma tumors follows a predictable pattern.
Ophthalmic Oncology Center, New York Hospital-Cornell Medical Center, NY, USA.
D H
DH
Abramson
M E
ME
Mendelsohn
C A
CA
Servodidio
T
T
Tretter
D S
DS
Gombos
D017668:Age of Onset; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009518:New York / Q000453:epidemiology; D019572:Retinal Neoplasms / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D012175:Retinoblastoma / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D012189:Retrospective Studies