Abstract

Purpose To investigate the clinical presentations and outcomes of retinoblastoma in relation to the advent of new multimodal treatments in Thailand. Patients and Methods. Retrospective case series. We evaluated the clinical presentation, staging, details of treatment, and treatment outcomes of retinoblastoma patients who were treated at Ramathibodi Hospital, Bangkok, Thailand, between January 1, 2007, and December 31, 2018. The log-rank test was used to explore clinical characteristics and treatment modalities that affected globe salvage and survival curves. Results This study included 124 eyes of 81 patients with retinoblastoma. Forty-three patients (53.1%) had bilateral retinoblastoma. The median age at diagnosis was 8 months (range, 1–48 months). Of 124 eyes, 9 eyes (7.3%) had extraocular retinoblastoma and 115 eyes (92.7%) had intraocular retinoblastoma, which were classified by the International Classification of Retinoblastoma (ICRB) as group A, 4 eyes (3.5%); group B, 19 eyes (16.5%); group C, 6 eyes (5.2%); group D, 31 eyes (27%); and group E, 56 eyes (47.8%). Treatment included systemic chemotherapy, intra-arterial chemotherapy, ruthenium-106 plaque brachytherapy, external beam radiation therapy, cryotherapy, transpupillary thermotherapy, subtenon chemotherapy, and intravitreal chemotherapy. At the median follow-up period of 38.4 months (range, 0.2–148.2 months), the overall globe salvage rate of intraocular retinoblastoma was 51.7%. For unilateral retinoblastoma, globe salvage rate was 37.5% (group B, 100%; group C, 100%; group D, 50%; and group E, 18.8%). For bilateral intraocular retinoblastoma, the globe salvage rate was 57.8% (group A, 100 %; group B, 94.4%; group C, 100%; group D, 64.7%; and group E, 28.2%). The overall survival rate was 93.8%. Conclusions Recent advanced treatment modalities have improved the probability of globe salvage. However, enucleation remains an important life-saving intervention in many advanced cases.

Highlights

  • Retinoblastoma is the most common primary intraocular malignant tumor in children [1], with a global incidence of approximately 7,202–8,102 annually [2]

  • Numerous studies worldwide, including Asia [7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30] and ailand [4,5,6, 31,32,33,34], have described the clinical manifestations and treatment outcomes of retinoblastoma, but these studies did not use IAC, brachytherapy, or intravitreal chemotherapy as part of retinoblastoma treatment [5,6,7,8, 11,12,13, 17,18,19, 23,24,25, 30,31,32,33,34]. erefore, our study was aimed to fill in the gap in investigating clinical presentations and treatment outcomes of retinoblastoma in relation to the advent of recent treatment modalities in ailand

  • We evaluated patients diagnosed with retinoblastoma between January 1, 2007, and December 31, 2018, at Ramathibodi Hospital, Bangkok, ailand. ose with inadequate data on clinical presentation, tumor staging, and treatment modality were excluded

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Summary

Introduction

Retinoblastoma is the most common primary intraocular malignant tumor in children [1], with a global incidence of approximately 7,202–8,102 annually [2]. Of these patients, approximately 40% are in Asia-Pacific countries [3]. The nation-wide multicenter population-based prospective study of the incidence and survival rate of childhood cancer from ai Pediatric Oncology Group ( aiPOG) reported 97 cases of retinoblastoma diagnosed. Previous report from 3 cancer centers in northern, northeastern, and southern ailand during 1990 and 2009, which included 75 retinoblastoma patients, showed the survival rate of 40%, 50%, and 75%, respectively [5]. Numerous studies worldwide, including Asia [7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30] and ailand [4,5,6, 31,32,33,34], have described the clinical manifestations and treatment outcomes of retinoblastoma, but these studies did not use IAC, brachytherapy, or intravitreal chemotherapy as part of retinoblastoma treatment [5,6,7,8, 11,12,13, 17,18,19, 23,24,25, 30,31,32,33,34]. erefore, our study was aimed to fill in the gap in investigating clinical presentations and treatment outcomes of retinoblastoma in relation to the advent of recent treatment modalities in ailand

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