Division of Paediatric Haematology/Oncology, Children's Medical Institute and Department of Paediatrics, National University Hospital, Singapore.
Ann Acad Med Singap. 2009 Aug;38(8):693-8.
Retinoblastoma is a very rare disease. There were 30 cases of retinoblastoma diagnosed and treated at National University Hospital (NUH).
A retrospective chart review was performed on the medical records of 30 patients who were diagnosed with retinoblastoma between 1995 and 2008 at the Department of Paediatrics, National University Hospital, Singapore.
The median age at diagnosis was 1.6 years (range, 0-5.9) with a median follow-up of 1.8 years (range, 0.1 to 11.6). The median time from presenting signs to the time of diagnosis was 5.2 months (range, 0-25.2). Common presenting signs of retinoblastoma were identified; the most common of which were leukocoria (50.0%), squinting (13.3%), poor vision (10.0%), strabismus (6.6%) and unknown (33.3%). Of the 30 patients, 10 were from Singapore whilst the other 20 patients were from the surrounding countries. Twelve patients had bilateral disease at the time of diagnosis, while 18 had unilateral disease. Staging information was available in 27 patients. Enucleation was performed in 25 of 30 patients. Radiation therapy was given in 3 patients in 1995 (bilateral disease), 2001 (bilateral disease) and 2003 (unilateral disease). At the time of analysis, 19 patients were alive with no evidence of disease. Overall 5-year survival for the cohort was 88.1% [95% confidence interval (CI), 88.0-100] and event-free survival for the whole cohort was 74.2% (95% CI, 55.8-92.6).
In our limited experience, the importance of collaboration and standardisation of the staging system, raising awareness and education of primary healthcare providers and parents are strongly stressed.
视网膜母细胞瘤是一种非常罕见的疾病。在新加坡国立大学医院(NUH)诊断和治疗了 30 例视网膜母细胞瘤患者。
对 1995 年至 2008 年在新加坡国立大学医院儿科诊断为视网膜母细胞瘤的 30 例患者的病历进行回顾性图表审查。
中位诊断年龄为 1.6 岁(范围,0-5.9),中位随访时间为 1.8 年(范围,0.1-11.6)。从出现症状到确诊的中位时间为 5.2 个月(范围,0-25.2)。确定了视网膜母细胞瘤的常见表现症状;其中最常见的是白瞳(50.0%)、斜视(13.3%)、视力不佳(10.0%)、斜视(6.6%)和未知(33.3%)。30 例患者中,10 例来自新加坡,其余 20 例来自周边国家。12 例患者在诊断时患有双侧疾病,18 例患者患有单侧疾病。27 例患者有分期资料。30 例患者中有 25 例行眼球摘除术。1995 年(双侧疾病)、2001 年(双侧疾病)和 2003 年(单侧疾病)对 3 例患者给予放疗。在分析时,19 例患者存活且无疾病证据。该队列的总 5 年生存率为 88.1%[95%置信区间(CI),88.0-100],整个队列的无事件生存率为 74.2%(95%CI,55.8-92.6)。
根据我们的有限经验,强烈强调合作和分期系统标准化、提高初级保健提供者和家长的认识和教育的重要性。