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- Title
The National Registry of Retinoblastoma in Japan (1983-2014).
- Authors
The Committee for the National Registry of Retinoblastoma
- Abstract
Purpose: To review the clinical findings of retinoblastoma recorded over a period of 32 years by the National Registry of Retinoblastoma in Japan.Study design: Retrospective.Methods: We reviewed the diagnoses, clinical pictures, and treatment data recorded on a yearly basis from 1983 until 2014 by major Japanese medical facilities.Results: A total of 2360 patients (1225 boys, 1135 girls) were analyzed. Of those, 67.3% had unilateral retinoblastoma, 32.7% had bilateral retinoblastoma, and 6.7% had a family history of retinoblastoma. The average occurrence frequency for retinoblastoma was 1:16,823 births/year. At diagnosis, 89.0% of the patients were aged younger than 3 years and 41.0% were aged younger than 1 year. The most common initial symptom was leukocoria (48.9%), which was followed by cat’s eye (17.1%) and strabismus (14.8%). Of the total 3131 eyes, 53.0% were Reese-Ellsworth group V. As per the International Classification of Retinoblastoma, 33.7% of the eyes were group D, and 30.1% were group E. Enucleation was performed in 1545 eyes (52.2%), and subsequent treatment was administered in 379 eyes. Conservative therapy was performed in 1415 eyes (47.8%) of 926 patients. The number of eyes receiving conservative therapy increased every year, even in about 30% of those with advanced group V eyes. Radiation therapy was the primary conservative therapy before the year 2000 and was thereafter replaced by chemotherapy. In the eyes receiving chemotherapy, anticancer agents were administered systematically in one-third of the eyes, locally in another one-third of the eyes, or as a combination of both in the other one-third. Conservative therapy successfully preserved in over 90% of the eyes of groups I to IV and in 78.1% of the eyes of group V.Conclusions: Retinoblastoma is often diagnosed at an advanced stage. With the development of systemic and local chemotherapy, the number of eyes with advanced retinoblastoma preserved by conservative therapy has been increasing.
- Subjects
RETINOBLASTOMA; CANCER chemotherapy; ANTINEOPLASTIC agents; RETROSPECTIVE studies; PATIENTS; THERAPEUTICS
- Publication
Japanese Journal of Ophthalmology, 2018, Vol 62, Issue 4, p409
- ISSN
0021-5155
- Publication type
Article
- DOI
10.1007/s10384-018-0597-2