Retinoblastoma in Asia
Swathi Kaliki, Vijitha S. Vempuluru, Ashik Mohamed, Mazin Faisal Al‐Jadiry, Richard Bowman, Bhavna Chawla, Syed Ahmer Hamid, Xunda Ji, Noa Kapelushnik, Rejin Kebudi, Purnima Rajkarnikar Sthapit, Duangnate Rojanaporn, Rita S. Sitorus, Yacoub A. Yousef, Ido Didi Fabian, Swathi Kaliki, Vijitha S. Vempuluru, Ashik Mohamed, Rula Ahmed Abdulqader, Priyanka Aggarwal, Alia Ahmad, Marliyanti Nur Rahmah Akib, Saleh Al Mesfer, Mouroge Hashim Al Ani, Safa A. Faraj, Ana Patricia Alcasabas, Saad Al-Dahmash, Christiane Al‐Haddad, Hamoud Hodeish Yahya Al-Hussaini, Mazin Faisal Al‐Jadiry, Usama Al‐Jumaily, Hind M. Alkatan, Ali Abdul Razzaq Mahmood Al-Mafrachi, Athar Abdul Samad Majeed Al-Shaheen, Entissar Hadi Al-Shammary, Primawita Oktarima Amiruddin, Inggar Armytasari, Nick Astbury, Hatice Tuba Atalay, Eda Ataseven, La‐ongsri Atchaneeyasakul, Ruhengiz Balayeva, Covadonga Bascarán, Ainura Suranovna Begimkulova, Anirban Bhaduri, Sunil Bhat, Arpita Bhattacharyya, Sharon Blum, Richard Bowman, Jassada Buaboonnam, Matthew J. Burton, Shani Caspi, Shabana Chaudhry, Bhavna Chawla, Wensi Chen, Tsengelmaa Chuluunbat, Wantanee Dangboon, Anirban Das, Pranab Das, Sima Das, Yi Du, Gagan Dudeja, I Wayan Eka Sutyawan, Zehra Fadoo, Mohammad Faranoush, Allen Foster, Shahar Frenkel, Fariba Ghassemi, Nir Gomel, Sanjeeva Gunasekera, Kaan Gündüz, Himika Gupta, SK Gupta, Vineeta Gupta, Syed Ahmer Hamid, Norhafizah Hamzah, Murat Hasanreisoğlu, Shadab Hassan, Huda Awni Haydar, Suradej Hongeng, Allawi Noor Hussein Al-Janabi, Ziyavuddin Islamov, Teyyeb Azeem Janjua, Irfan Jeeva, Xunda Ji, Dong Hyun Jo, Mehmet Kantar, Noa Kapelushnik, Rejin Kebudi, Jonny Keomisy, Zohora Jameela Khan, Hussain Ahmed Khaqan, Vikas Khetan, Alireza Khodabande, Jeong Hun Kim, Hayyam Kıratlı, İrem Koç, Kittisak Kulvichit, Mayasari Wahyu Kuntorini, Cairui Li
Abstract
PURPOSE: To describe the clinical presentation and treatment outcomes of children who received a diagnosis of retinoblastoma in 2017 throughout Asia. DESIGN: Multinational, prospective study including treatment-naïve patients in Asia who received a diagnosis of retinoblastoma in 2017 and were followed up thereafter. PARTICIPANTS: A total of 2112 patients (2797 eyes) from 96 retinoblastoma treatment centers in 33 Asian countries. INTERVENTIONS: Chemotherapy, radiotherapy, enucleation, and orbital exenteration. MAIN OUTCOME MEASURES: Enucleation and death. RESULTS: Within the cohort, 1021 patients (48%) were from South Asia (SA), 503 patients (24%) were from East Asia (EA), 310 patients (15%) were from Southeast Asia (SEA), 218 patients (10%) were from West Asia (WA), and 60 patients (3%) were from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, < 1-261 months). The cohort included 1195 male patients (57%) and 917 female patients (43%). The most common presenting symptoms were leukocoria (72%) and strabismus (13%). Using the American Joint Committee on Cancer Staging Manual, Eighth Edition, classification, tumors were staged as cT1 (n = 441 [16%]), cT2 (n = 951 [34%]), cT3 (n = 1136 [41%]), cT4 (n = 267 [10%]), N1 (n = 48 [2%]), and M1 (n = 129 [6%]) at presentation. Retinoblastoma was treated with intravenous chemotherapy in 1450 eyes (52%) and 857 eyes (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, and 20% and 11% for WA (P < 0.0001 and P < 0.0001), respectively. CONCLUSIONS: At the conclusion of this study, significant heterogeneity was found in treatment outcomes of retinoblastoma among the regions of Asia. East Asia displayed better outcomes with higher rates of globe and life salvage, whereas Southeast Asia showed poorer outcomes compared with the rest of Asia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.