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

@entity1 @entity5 @entity26681 @entity3 @entity995
PURPOSE: Few estimates of the survival rates ofwas @entity995 have been reported from the Asia region. In this study, we aim to describe the survival and clinical characteristics of 96 @entity995 cases treated at Chang Gung Medical Center, Taipei, between 1978 and 2000. METHODS: We retrospectively analyzed the clinical records of 96 @entity1 (116 eyes) diagnosed with @entity995 and treated between 1978 and 2000. Information on sex, laterality, age at diagnosis, presenting signs, @entity5 , 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%), @entity26681 (34 cases, 35.4%), proptosis (16 cases, 16.7%), and strabismus (12 cases, 12.5%). Forty-two eyes had orbital extension, 27 @entity1 had central nervous system invasion, 16 cases exhibited bone marrow involvement, and 3 cases had liver @entity3 . Three (3.1%) @entity1 had a family history of @entity995 . None of the cases developed a secondary @entity5 . The 3-year cumulative survival rate of the 96 @entity1 was 64.41% (unilateral, 71.97%; bilateral 40.01%). CONCLUSIONS: The mortality was much higher than that in reports on Western and Japanese @entity1 . 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.
