Department of Pediatric Hematology and Oncology, University Duisburg-Essen, University Hospital Essen, Essen, Germany.
Department of Pediatric Oncology and Hematology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Pediatr Blood Cancer. 2021 Jun;68(6):e28963. doi: 10.1002/pbc.28963. Epub 2021 Mar 15.
Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond the natural limits of the eye is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment.
Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma Group (EURbG) network. Extended information was gathered via personal email communication.
Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy.
Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.
高级眼内视网膜母细胞瘤可以通过眼球摘除术治愈，但视网膜母细胞瘤细胞超出眼睛自然范围的扩散与高死亡率有关。眼球摘除术后的辅助治疗已被证明可以预防有眼外视网膜母细胞瘤危险因素的儿童发生转移。然而，组织学标准和辅助治疗方案存在差异，对于最佳治疗选择尚无统一共识。
通过欧洲视网膜母细胞瘤转诊中心的 EURbG 网络的所有成员进行在线调查，收集有关辅助治疗指南的数据。通过个人电子邮件交流收集扩展信息。
从 17 个国家的 26 个中心收集数据。92.3%的视网膜母细胞瘤中心有辅助治疗指南。超过 80%的中心对辅助治疗的适应证和强度达成共识。大多数中心对于孤立性局限性脉络膜侵犯或前视神经鞘侵犯不采用辅助治疗。对于广泛的脉络膜侵犯或后视神经鞘侵犯的患者，给予辅助化疗，而对于视神经切除缘的微小侵犯或通过巩膜延伸的侵犯，给予化疗和放疗联合治疗。
欧洲视网膜母细胞瘤转诊中心的适应证和辅助治疗方案相似但不统一。除了组织病理学危险因素之外，进一步的生物标志物可以改善治疗分层。欧洲中心的高度共识为具有前瞻性验证新生物标志物的共同欧洲研究奠定了良好的基础。
