Results of Combined Chemotherapy and External Beam Radiotherapy for Unilateral Intra-Orbital Retinoblastoma—A Multi-Institutional Study from Mali

作     者：Boubacar Togo Fousseyni Traore Konimba Diabate Fatou Sylla Aichata Tall Affiatou Simaga Bakarou Kamate Hawa Diall Pierre Togo Abdoul Karim Doumbia Pierre Bey Francis Eshun 

作者机构：Department of PediatricsGabriel Toure HospitalBamakoMali Radiation Therapy ServiceNational Hospital of MaliBamakoMali Institute of Tropical Ophthalmology of Africa(IOTA)BamakoMali Department of HistopathologyHospital of Point GBamakoMali Curie Institute et AMCCParisFrance Phoenix Children’s HospitalPhoenixAZUSA 

出 版 物：《Open Journal of Pediatrics》 (儿科学期刊（英文）)

年 卷 期：2019年第9卷第1期

页      面：39-46页

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Orbital Retinoblastoma Children Mali 

摘      要：Retinoblastoma (RB) treatment aims at saving the life and preserving useful vision. In most low-income countries, because of delays in diagnosis, advanced disease presentation is quite common. This prospective study aimed at evaluating the treatment results of orbital RB with regards to overall survival rate of the patients treated with chemotherapy and radiotherapy. The study was performed from 01 November 2011 to 31 December 2015 in the paediatric oncology unit of Gabriel Touré Teaching Hospital, Bamako, Mali and The Institute of Tropical Ophthalmology of Africa (IOTA), Bamako, Mali. All intra-orbital non-metastatic RB cases not previously treated by chemotherapy or radiotherapy were included in this study. Fourteen patients were included into the study. Median age was 2 years, and sex ratio 2.5 (M = 10;F = 4). Right eye (n = 12, 85.7%) was more often affected than left eye (n = 2;14.3%). Chemotherapy toxicities were mainly haematological including grade 3 anemia (n = 2;7%) and grade 4 neutropenia (n = 3;11%). Twelve patients (86%) were enucleated after neoadjuvant chemotherapy. Two patients (14%) abandoned treatment before enucleation. The IRSS pathology staging was: stage IIIa in 6 patients (50%), and stage II in six patients (50%). Six children (43%) received orbital radiotherapy at total dose of 45 Gy;Six children (43%) achieved complete remission;Overall survival rate was 48% at 4 years (95% CI: 22.5% - 74.4%). In conclusion, the therapeutic strategy based on neoadjuvant chemotherapy followed by enucleation, adjuvant chemotherapy and external beam radiotherapy, was beneficial in patients with stage III disease, achieving an event-free survival rate of 48%.