• Ocular Pathology/Oncology, Pediatric Ophth/Strabismus, Retina/Vitreous

    Researchers used the 8th edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual to evaluate metastatic and mortality rates among children with retinoblastoma.

    Study design

    This is a multicenter, international, retrospective review of 2,085 patients (2,905 eyes) with retinoblastoma from 18 ophthalmic oncology centers in 13 countries.


    Among the patients assessed, 2.6% of tumors were categorized as cT1a, 8% were cT1b, 9% were cT2a, 39% were cT2b, 40% were cT3 and 0.9% were cT4. Approximately 5% of patients (109) developed metastases and died. The 5-year Kaplan-Meier cumulative survival estimates by clinical tumor categories were 100% for category cT1a, 98% for cT1b and cT2a, 96% for cT2b, 89% for cT3 and 45% for cT4 tumors.

    The staging system revealed increased risk of mortality with increased tumor staging. No other data points gathered were associated with increased mortality besides tumor stage.


    This retrospective multicenter study was limited by the quality of data that could be mined from the database. As stated in the paper, since different centers used different classification systems, it was difficult to compare centers. There may be a selection bias from centers who were willing to participate.

    Clinical significance

    This paper validates a retinoblastoma classifications system and describes factors affecting mortality of patients with retinoblastoma based on their clinical stage. Using the AJCC staging system could help compare data between centers.