Pediatric Department, Pediatric Oncology Unit, CHU Gabriel Touré, Bamako, Mali.
Pediatric Ophtalmology Department, Institute of African Tropical Ophthalmology, Bamako, Mali.
Pediatr Blood Cancer. 2018 Aug;65(8):e27101. doi: 10.1002/pbc.27101. Epub 2018 Apr 26.
Retinoblastoma (Rb) is the most common intraocular primary malignancy in children. In industrialised countries, the cure rate is about 95%. We present the results of a prospective study on the management of Rb in the paediatric oncology unit of Gabriel Touré Teaching Hospital and African Institute of Tropical Ophthalmology, from November 1, 2011 to December 31, 2015.
The aims of this prospective study were to evaluate the treatment of localised Rb, ocular prosthesis after enucleation, conservative management for bilateral Rb as well as survival rates in all patients. Patients with early stage Rb at diagnosis were included. The treatment was performed according to the retinoblastoma treatment guidelines of the French-African Paediatric Oncology Group.
Eighty-eight patients were included in the study. Sex ratio was 1:1 (M = 44, F = 44). Median age at diagnosis was 3 years (range: 2 months-5 years). Unilateral intraocular Rb was predominant (n = 50; 56.8%). Conservative treatments were performed on nine eyes in nine patients. Overall survival and event-free survival of the entire cohort at the end of 4 years were 73% (95% CI 60.8-81.2%) and 59% (95% CI 47.9-69.5%), respectively, with a median follow-up of 3.7 years (0.1-5.6 years). In conclusion, early enucleation in early stage of Rb can improve outcomes in resource-limited countries. Delayed enucleation and refusal of adherence to treatment are still major concerns and remain a barrier to improving overall patient survival.
视网膜母细胞瘤（Rb）是儿童中最常见的眼内原发性恶性肿瘤。在工业化国家，治愈率约为 95%。我们报告了 2011 年 11 月 1 日至 2015 年 12 月 31 日期间，在加布里埃尔·图雷教学医院和非洲热带眼科研究所儿科肿瘤病房对 Rb 进行的前瞻性研究结果。
本前瞻性研究的目的是评估局部 Rb、眼球摘除术后眼假体、双侧 Rb 的保守治疗以及所有患者的生存率。纳入诊断时处于早期 Rb 的患者。治疗根据法国-非洲儿科肿瘤学组的 Rb 治疗指南进行。
本研究共纳入 88 例患者。男女比例为 1:1（M=44，F=44）。中位诊断年龄为 3 岁（范围：2 个月至 5 岁）。单侧眼内 Rb 更为常见（n=50；56.8%）。对 9 名患者的 9 只眼进行了保守治疗。整个队列在 4 年结束时的总生存率和无事件生存率分别为 73%（95%CI 60.8-81.2%）和 59%（95%CI 47.9-69.5%），中位随访时间为 3.7 年（0.1-5.6 年）。总之，在资源有限的国家，早期 Rb 的早期眼球摘除可以改善结局。延迟眼球摘除和拒绝接受治疗仍然是主要关注点，仍然是提高总体患者生存率的障碍。
