Yarovoy Andrey A, Golanov Andrey V, Yarovaya Vera A, Kostjuchenko Valery V, Volodin Denis P
Ocular Oncology and Radiology, S. Fyodorov Eye Microsurgery Federal State Institution, Moscow, RUS.
Department of Radiation Oncology, N.N. Burdenko National Medical Research Center for Neurosurgery, Moscow, RUS.
Cureus. 2022 Sep 3;14(9):e28751. doi: 10.7759/cureus.28751. eCollection 2022 Sep.
Background External beam radiotherapy for resistant retinoblastoma is now seen as a last resort to saving the eye because of the risk of severe side effects: secondary cancers and cosmetic problems of orbital bone growth retardation. To reduce such complications, treatment modalities have shifted towards new radiation therapy techniques. No information on single fraction Gamma Knife® radiosurgery (GKRS) for intraocular retinoblastoma exists. Materials and methods Eighteen children (19 eyes) with retinoblastoma were treated with GKRS. The mean age at the time of treatment was 35 months (from 12 to 114 months). Before GKRS, all routes of chemotherapy delivery were held in all cases. The eligibility criteria for GKRS were retinoblastomas not amenable either to systemic or local chemotherapy and local ophthalmological treatment, retinoblastomas too large for conventional local methods, and inability to perform intraarterial chemotherapy. Conventional external beam radiotherapy was excluded in the presented cases, given the possible complications mentioned above. In every case, eye removal was suggested to the child's parents, but they flatly refused. GKRS was proposed as the last chance to save the eye (in four cases, it was performed on the only eye). The median prescribed dose was 22 Gy (interquartile range [IQR]: 18-35 Gy), and the median prescribed isodose was 50% (IQR: 36-90%). Results Local control was achieved in 79% of cases (complete tumor regression in 69%, incomplete regression in 10%). Two eyes (10.5%) could not be preserved and had to be enucleated due to the tumor recurrence. Two eyes (10.5%) developed secondary complications (total vitreous hemorrhage, retinal detachment, and iris neovascularization), making adequate tumor control nearly impossible. Overall, 15 eyes (79%) were preserved, and four eyes (21%) were enucleated after GKRS with no signs of tumor recurrence and metastasis in the mean follow-up of 41 months. No acute radiation side effects occurred in any patient after GKRS. Ten children (10 eyes, 53%) were diagnosed with vitreous hemorrhage from mild to severe. Three eyes presented with optic neuropathy one year after GKRS, and four eyes developed retinopathy. Radiation-induced cataract occurred in two eyes. There were no cases of secondary glaucoma or keratopathy in our study. All patients and eyes treated by GKRS were stable within 41 months (from seven to 74 months). Conclusions Single fraction Gamma Knife® radiosurgery may be a reasonable salvage treatment for resistant and recurrent retinoblastoma as an alternative approach to enucleation in selected cases. GKRS should be considered in retinoblastoma management.
背景 由于存在严重副作用风险：继发性癌症以及眼眶骨生长发育迟缓的美容问题，对于耐药性视网膜母细胞瘤，现在外照射放疗被视为挽救眼球的最后手段。为减少此类并发症，治疗方式已转向新的放射治疗技术。目前尚无关于单剂量伽玛刀放射外科手术（GKRS）治疗眼内视网膜母细胞瘤的相关信息。
材料与方法 18例患有视网膜母细胞瘤的儿童（19只眼）接受了GKRS治疗。治疗时的平均年龄为35个月（12至114个月）。在进行GKRS之前，所有病例均暂停了所有化疗给药途径。GKRS的入选标准为既不适合全身化疗也不适合局部化疗以及局部眼科治疗的视网膜母细胞瘤、对于传统局部方法来说太大的视网膜母细胞瘤、无法进行动脉内化疗的情况。鉴于上述可能的并发症，本研究病例排除了传统外照射放疗。在每个病例中，都向患儿家长建议进行眼球摘除，但他们坚决拒绝。GKRS被提议作为挽救眼球的最后机会（4例中，是对仅有的一只眼进行手术）。处方剂量中位数为22 Gy（四分位数间距[IQR]：18 - 35 Gy），处方等剂量中位数为50%（IQR：36 - 90%）。
结果 79%的病例实现了局部控制（69%完全肿瘤消退，10%不完全消退）。2只眼（10.5%）因肿瘤复发无法保留，不得不进行眼球摘除。2只眼（10.5%）出现了继发性并发症（全玻璃体出血、视网膜脱离和虹膜新生血管形成），使得充分控制肿瘤几乎不可能。总体而言，15只眼（79%）得以保留，4只眼（21%）在GKRS后进行了眼球摘除，在平均41个月的随访中无肿瘤复发和转移迹象。GKRS后所有患者均未出现急性放射副作用。10名儿童（10只眼，53%）被诊断为有轻度至重度的玻璃体出血。3只眼在GKRS后1年出现视神经病变，4只眼出现视网膜病变。2只眼发生了放射性白内障。本研究中未出现继发性青光眼或角膜病变病例。所有接受GKRS治疗的患者和眼睛在41个月内（7至74个月）病情稳定。
结论 单剂量伽玛刀放射外科手术对于耐药性和复发性视网膜母细胞瘤可能是一种合理的挽救治疗方法，可作为特定病例中眼球摘除的替代方法。在视网膜母细胞瘤的治疗中应考虑GKRS。
