在孟加拉国三级眼科中心成立“视网膜母细胞瘤中心”——视网膜母细胞瘤患者的新希望

S. Roy, Murtuza Nuruddin, M. Osmani
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摘要

视网膜母细胞瘤是儿童最常见的原发性眼内肿瘤,其发生率为1.16万至1.8万活产婴儿。全世界每年新发现的病例约为8000例,印度约为1400例。它占出生后第一年发生的癌症的11%。目前革命性的治疗策略已使发达国家视网膜母细胞瘤的存活率提高到95%以上,是所有儿童癌症中最高的。但它仍然是世界范围内致命的癌症。基于收入的视网膜母细胞瘤存活率>90% vs 40%(在高收入国家和低收入国家)。转移的发生率在低收入国家较高(32%对12%的中等收入国家)。全球负担的43%生活在6个亚洲国家(印度、中国、印度尼西亚、巴基斯坦、孟加拉国和菲律宾)。各大洲的死亡率各不相同。据估计,全世界的死亡率超过40%,其中大多数来自亚洲和非洲。孟加拉国是东南亚地区的发展中国家之一,视网膜母细胞瘤占4岁以下儿童癌症的83%。为使视网膜母细胞瘤的治疗达到国际标准,需要建立由眼科肿瘤学家、临床肿瘤学家、放射肿瘤学家、儿科医生、眼整形外科医生、视网膜专科医生、儿科眼科医生和眼科医生组成的视网膜母细胞瘤中心。管理包括适当的诊断、治疗疾病、遗传咨询、定期随访、幸存者康复和兄弟姐妹筛查。吉大港眼科医院和培训中心是孟加拉国的三级中心和转诊中心之一,自成立以来一直在治疗视网膜母细胞瘤。由于时间的需要,医院从2017年开始对各种设施进行重组,以团队方式为视网膜母细胞瘤患者提供服务。2017年1月至2022年3月共诊断304例患者。其中132例接受长春新碱、依托泊苷、卡铂VEC化疗,79例行长视神经去核术。医院还对兄弟姐妹进行筛查,为RB幸存者提供视觉和心理社会康复,并在社区一级开展宣传项目。
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Establishment of “Retinoblastoma Center” in A Tertiary Eye Care Center of Bangladesh – A New Hope for Retinoblastoma Patients
Retinoblastoma is the most common primary intraocular tumor in children with an incidence of 1: 16,000 to 18,000 live birth. Every year worldwide newly detected cases are about 8000 and in India about 1400. It represents 11% of cancer that develop in the first year of life. Current revolutionary management strategy has increased the survival rate of retinoblastoma above 95% in developed country and it is the highest among all pediatric cancer. But still it is a deadly cancer worldwide. Survival from retinoblastoma based on income >90% vs 40% (in high to low income countries). Occurrence of metastases is higher in low-income countries (32% vs 12% in middle-income). Forty three (43%) of global burden lives in 6 countries of Asia (India, China, Indonesia, Pakistan, Bangladesh & Philippines). Mortality rate varies in different continents. An estimated worldwide death rate is more than 40% and most of them from Asia and Africa. Bangladesh is one of the developing countries of the South- East Asia region and retinoblastoma constitutes 83% of all pediatric cancer bellow 4 years of age. For proper management of retinoblastoma with an international standard, establishment of retinoblastoma center consisting of Ocular oncologist, Clinical oncologist, Radiation oncologist, Pediatrician, Oculoplastic surgeon, Retina specialist, Pediatric ophthalmologist, and Ocularist is needed. Management include proper diagnosis, treatment of the disease, genetic counseling, regular follow up, rehabilitation of survivor and screening of sibling. Chittagong Eye Infirmary & Training Complex which is a tertiary center and one of the referral centers of the Bangladesh is treating retinoblastoma since its inception. Due to demand of time the hospital has been reorganized the various facilities to serve retinoblastoma patients with a team approach from 2017. From January 2017 to March 2022 total 304 patients were diagnosed. Among them 132 received VEC (Vincrisrine, Etoposide, Carbplatin) chemotherapy from this center and 79 underwent enucleation with long optic nerve. The hospitals also screen sibling, provide visual and psycho-social rehabilitation of the RB survivors and run awareness program in community level.
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