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首页> 外文期刊>Pediatric blood & cancer >Retinoblastoma in Central America: Report from the Central American Association of Pediatric Hematology Oncology (AHOPCA)
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Retinoblastoma in Central America: Report from the Central American Association of Pediatric Hematology Oncology (AHOPCA)

机译：中美洲视网膜母细胞瘤：中美洲小儿血液肿瘤学协会（AHOPCA）的报告

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Background: Retinoblastoma is highly curable in high income countries. Low income countries have poor results due to advanced disease and lack of resources. Central American Association of Pediatric Hematology Oncology (AHOPCA) aimed to standardize the approach and to improve outcomes of patients with retinoblastoma. Procedure: One hundred seventy-one patients, age <18 years newly diagnosed with retinoblastoma were treated according to laterality and stage. Therapeutic modalities were: surgery (enucleation), local control (laser therapy, cryotherapy), chemotherapy, and radiation therapy. Chemotherapy consisted of vincristine, etoposide, and carboplatin (6 cycles). Outcomes were measured by overall survival. Events were abandonment of therapy and death. Results: One hundred seventy-one patients (129 unilateral, 42 bilateral) were treated. Median age was 2 years 4 months; 112 (66%) were diagnosed before 3 years of age. 119 (92%) eyes in patients with unilateral disease were Reese-Ellsworth IV or V versus 52 (62%) eyes in patients with bilateral disease. Extraocular disease was more prevalent in unilateral disease (65% vs. 50%). Older age at diagnosis correlated with higher stage. Estimated overall survival at 60 months was 0.48±0.04. Outcome of patients with bilateral disease was significantly better than unilateral (62%±0.09 vs. 42%±0.05, P=0.0006). Thirty-eight patients (22%) refused or abandoned therapy. Conclusions: Protocol-directed therapy for retinoblastoma in Central America is possible. Patients present with advanced disease and outcome is significantly worse than in middle and high-income countries. Refusal and abandonment of therapy are societal events that affect outcome. Initiatives aimed at improving early diagnosis, while dedicated treatment centers are developed, are critical.
机译：背景：视网膜母细胞瘤在高收入国家可高度治愈。低收入国家由于疾病晚期和缺乏资源而收成不好。中美洲小儿血液肿瘤学协会（AHOPCA）旨在标准化该方法并改善视网膜母细胞瘤患者的预后。程序：根据偏侧性和分期对新诊断为视网膜母细胞瘤的171名年龄<18岁的患者进行了治疗。治疗方式为：手术（摘除术），局部对照（激光治疗，冷冻治疗），化学疗法和放射疗法。化学疗法包括长春新碱，依托泊苷和卡铂（6个周期）。结果通过总体生存率来衡量。事件是放弃治疗和死亡。结果：共治疗了171例患者（单侧129例，双侧42例）。中位年龄为2岁4个月; 3岁之前被诊断出112例（66％）。单侧疾病患者的119眼（92％）为Reese-Ellsworth IV或V，而双侧疾病患者的52眼（62％）。眼外疾病在单侧疾病中更为普遍（65％比50％）。诊断时年龄越大，阶段越高。估计的60个月总生存率为0.48±0.04。双侧疾病患者的结果显着优于单侧患者（62％±0.09 vs. 42％±0.05，P = 0.0006）。 38位患者（22％）拒绝或放弃了治疗。结论：中美洲视网膜母细胞瘤的方案指导治疗是可能的。患有晚期疾病和转归的患者明显比中高收入国家差。拒绝和放弃治疗是影响结果的社会事件。在建立专门的治疗中心的同时，旨在改善早期诊断的举措至关重要。

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