Retinoblastoma in Taiwan: survival and clinical characteristics 1978-2000.

PURPOSE Few estimates of the survival rates ofwas retinoblastoma have been reported from the Asia region. In this study, we aim to describe the survival and clinical characteristics of 96 retinoblastoma cases treated at Chang Gung Medical Center, Taipei, between 1978 and 2000. METHODS We retrospectively analyzed the clinical records of 96 children (116 eyes) diagnosed with retinoblastoma and treated between 1978 and 2000. Information on sex, laterality, age at diagnosis, presenting signs, spread of tumor, treatment modality, survival rate, and family history were collected. RESULTS Seventy-six (79.2%) cases were unilateral and 20 (20.8%) were bilateral. The mean age overall at the time of diagnosis was 24.7 months; in unilateral cases, 27.1 months; and in bilateral cases, 15.6 months. The most common presenting signs were leukocoria (75 cases, 78.1.0%), buphthalmos (34 cases, 35.4%), proptosis (16 cases, 16.7%), and strabismus (12 cases, 12.5%). Forty-two eyes had orbital extension, 27 patients had central nervous system invasion, 16 cases exhibited bone marrow involvement, and 3 cases had liver metastasis. Three (3.1%) patients had a family history of retinoblastoma. None of the cases developed a secondary neoplasm. The 3-year cumulative survival rate of the 96 patients was 64.41% (unilateral, 71.97%; bilateral 40.01%). CONCLUSIONS The mortality was much higher than that in reports on Western and Japanese patients. Delayed diagnosis with frequent extraocular spread at the time of diagnosis caused the low survival rate. Fewer familial cases were encountered in our study than in other studies.