Clinical manifestations and treatment of retinoblastoma in Kobe children's hospital for 16 years.

Purpose: To report the clinical manifestations and therapeutic outcomes of retinoblastoma during 16 years.

Methods: Forty-three eyes of 34 patients with retinoblastoma from 1996 to 2011 were retrospectively examined for eye preservation rates based on the disease stage, and the treatment outcomes between monocular and binocular cases were compared. In addition, the relationship between age at initial examination and disease stage and the difference of age at enucleation with or without preservation therapy were examined.

Results: Twenty-five patients had monocular retinoblastoma and 9 patients had binocular retinoblastoma. Three patients had a family history of retinoblastoma. The mean observation period was 106.6 ± 53.0 months. Median age at initial examination was 13.5 months. There was no correlation between age at initial examination and disease stage. Preservation therapy was performed for 24 eyes. Nineteen eyes were enucleated without performing preservation therapy. There was a significant difference in age at enucleation between the preservation therapy group (finally enucleated 13 eyes, median = 30.0 months) and the non-preservation therapy group (19 eyes, median = 13.0 months). Eye preservation rates according to the International Classification of Retinoblastoma (ICRB) were as follows: 100% for group B, 100% for group C, 33% for group D, and 0% for group E. In eyes with ICRB group D, the ratio of induction with preservation therapy was 50% of monocular cases and 75% of binocular cases.

Conclusions: Preservation therapy was able to significantly delay enucleation. Eye preservation therapy was performed for a higher proportion of advanced cases among binocular cases than among monocular cases.