Pant Gitika, Verma Nishant, Kumar Archana, Pooniya Vishal, Gupta Sanjiv Kumar
a Pediatric Hematology-Oncology Unit, Department of Pediatrics , King George's Medical University , Lucknow , Uttar Pradesh , India.
b Department of Ophthalmology , King George's Medical University , Lucknow , Uttar Pradesh , India.
Pediatr Hematol Oncol. 2017 Nov;34(8):419-424. doi: 10.1080/08880018.2017.1422060. Epub 2018 Jan 16.
Retinoblastoma (RB) is the most common ocular malignancy in children, and is managed by multimodal treatment. There is a paucity of data regarding the clinical profile and outcome of children with extraocular retinoblastoma from Low Middle Income Countries (LMIC) including India. Case records of children with newly diagnosed extraocular RB from January 2013 to August 2016 treated at our unit were analysed for clinical profile, treatment, and outcome. Over the 44 month study period, 91 children were diagnosed with RB, out of which 41 had extraocular disease. While 26 children had extraocular spread limited to orbit (IRSS stage III), 15 had a distant spread to brain (IRSS stage IV). Median lag period for diagnosis was eight months. Treatment abandonment rates were 38.5% and 46.6% in International Retinoblastoma Staging System (IRSS) stage III and IV respectively. With a median follow up of 31.5 months, the projected overall survival for IRSS III at one, two, and three years was 87.5%, 55.6%, and 39.7%. All patients with stage IV disease died after a median follow up duration of three months. High treatment abandonment rates and limited availability of resources lead to suboptimal survival in children with extraocular RB from LMIC. Initiatives aimed at improving early diagnosis, so that the disease is detected in the intraocular stage, are critical to improve the survival in children with RB.
视网膜母细胞瘤（RB）是儿童中最常见的眼部恶性肿瘤，采用多模式治疗。关于包括印度在内的低收入和中等收入国家（LMIC）患有眼外视网膜母细胞瘤儿童的临床特征和预后的数据很少。分析了2013年1月至2016年8月在我们科室接受治疗的新诊断眼外RB儿童的病例记录，以了解其临床特征、治疗和预后。在44个月的研究期间，91名儿童被诊断为RB，其中41名患有眼外疾病。26名儿童的眼外扩散局限于眼眶（国际视网膜母细胞瘤分期系统（IRSS）III期），15名儿童有远处扩散至脑部（IRSS IV期）。诊断的中位延迟期为8个月。在IRSS III期和IV期，治疗放弃率分别为38.5%和46.6%。中位随访31.5个月，IRSS III期1年、2年和3年的预计总生存率分别为87.5%、55.6%和39.7%。所有IV期疾病患者在中位随访3个月后死亡。高治疗放弃率和资源可用性有限导致LMIC眼外RB儿童的生存未达最佳状态。旨在改善早期诊断以便在眼内期检测到疾病的举措对于提高RB儿童的生存率至关重要。
