• Written By: Patricia Chévez-Barrios, MD
    Ocular Pathology/Oncology

    This study's authors performed a literature search and meta-analysis to document the spectrum of second primary tumors in retinoblastoma patients and identify relationships between patient age, radiation field, tumor location and tumor type. Using data from 128 published articles on 676 secondary primary tumors in 602 patients, this is the largest study to date analyzing secondary primary tumors in this high-risk population.

    The authors found that median age at diagnosis of a second primary tumor was 13.0 years (range, 0.3 to 60.4 years) for all secondary primary tumors, 2.7 years for midline intracranial primitive neuroectodermal tumors, 13.0 years for sarcomas, 27.0 years for melanomas and 29.0 years for carcinomas. The median age at which secondary primary tumors occurred inside the radiation field was younger than for those that occurred outside the radiation field or in patients who did not undergo irradiation (P < 0.001). Sarcomas occurred more commonly inside the radiation field (P < 0.001). Melanomas, lipomas, leukemias and lymphomas occurred more commonly outside the radiation field or in patients who did not undergo irradiation (P < 0.001).

    The authors conclude that retinoblastoma patients are susceptible to a broad range of secondary primary tumors throughout their lives, and that the median age at diagnosis changes according to tumor type. They say this data offers new insights for studying the underlying genetic predisposition to secondary primary tumors and will aid healthcare providers in monitoring this high-risk group.