Study: Family history, dilated screening leads to best ocular survival in retinoblastoma

The best ocular survival in retinoblastoma is seen in patients with a family history of the disease who are clinically screened for retinal tumors from birth, a large retrospective study found. Despite routine pediatric screening for leukocoria, most retinoblastomas are diagnosed only after a parent detects the presence of leukocoria, the study found.

David H. Abramson, MD, and colleagues at the New York Presbyterian Hospital-Weill Medical College of Cornell University conducted the retrospective study, which included 1,831 eyes diagnosed with retinoblastoma from 1914 to 2000.

Leukocoria was correlated with a good 5-year patient survival rate (86%) but a poor ocular survival rate: 4% for unilateral patients and 29% for bilateral patients. Of the 308 patients who initially presented with strabismus, patient survival rate was 90% at 5 years, but ocular survival was poor, at 17%.

Patients with a family history of retinoblastoma who were screened for tumors from birth with dilated fundus exams were diagnosed earlier and younger and had better rates of ocular survival than nonscreened patients with family history, the study found.

The study was published in Pediatrics.