Establishment of “Retinoblastoma center” in A Tertiary Eye Care Center of Bangladesh – A New Hope for Retinoblastoma Patients

Rani Roy Soma
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Abstract

Retinoblastoma is the most common primary intraocular tumor in children with an incidence of 1:16,000 to 18,000 live birth. Worldwide newly detected cases per year are about 8000 and in India above 1400. It represents 11% of cancer that develops in the first year of life. The revolutionary management strategy has increased the survival rate of retinoblastoma above 95% in developed countries, and this rate is the highest among all pediatric cancers. But still, it is deadly cancer worldwide. Survival from retinoblastoma based on income >90% vs. 40% (in high to low-income countries). The incidence of metastasis is more in lower-income countries (32% vs. 12% in middle-income). Notably, Forty-three percent of the world’s estimated cases reside in only 6 countries in Asia (China, Indonesia, Philippines, India, Pakistan, and Bangladesh). The mortality rate varies on different continents. Worldwide estimated death from retinoblastoma is more than 40%, and most of them are from Asia and Africa. Bangladesh is one of the developing countries in the South-East Asia region, and retinoblastoma constitutes 83% of all pediatric cancer under 4 years of age. For proper management of retinoblastoma with an international standard, the establishment of a retinoblastoma center consisting of ocular oncologist, clinical oncologist, radiation oncologist, pediatrician, oculoplastic surgeon, retina specialist, pediatric ophthalmologist, and ocularist is needed. Management includes proper diagnosis, treatment of the disease, genetic counseling, regular follow-up, rehabilitation of survivors, and screening of siblings. Chittagong Eye Infirmary & Training Complex is a tertiary eye care center and one of the referral centers of Bangladesh and is treating retinoblastoma since its inception. Due to the demand of time, the hospital has been reorganized with various facilities to serve retinoblastoma patients with a team approach in 2017. From January 2017 to March 2022, a total of 304 patients were diagnosed. Among them, 132 received vincristine, etoposide, and carboplatin (VEC) chemotherapy from this center, and 79 underwent enucleation with the long optic nerve. Besides treatment, the hospital is conducting sibling screening, visual and psycho-social rehabilitation for the RB survivors, and community awareness programs.
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在孟加拉国三级眼科中心建立“视网膜母细胞瘤中心”——视网膜母细胞瘤患者的新希望
视网膜母细胞瘤是儿童中最常见的原发性眼内肿瘤,其活产率为1:16 000至18000。全世界每年新发现的病例约为8000例,印度超过1400例。它占出生后第一年发生的癌症的11%。在发达国家,这种革命性的治疗策略使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为90% vs. 40%(在高收入和低收入国家)。转移的发生率在低收入国家更高(32%对12%的中等收入国家)。值得注意的是,全世界估计病例的43%仅居住在亚洲6个国家(中国、印度尼西亚、菲律宾、印度、巴基斯坦和孟加拉国)。各大洲的死亡率各不相同。全世界视网膜母细胞瘤的死亡率估计超过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,视网膜母细胞瘤占所有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,需要建立一个由眼科肿瘤学家、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、儿童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、疾病治疗、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一个三级眼科保健中心,也是孟加拉国的转诊中心之一,自成立以来一直在治疗视网膜母细胞瘤。由于时间的需要,医院于2017年进行了重组,配备了各种设施,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例在该中心接受长春新碱、依托泊苷、卡铂(VEC)化疗,79例视神经长段去核。除了治疗之外,医院还开展了兄弟姐妹筛查、RB幸存者的视觉和心理社会康复以及社区意识项目。
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