(1) Background: Orbital rhabdomyosarcoma is a rare and aggressive soft tissue tumor that primarily occurs in the eye socket (orbit) of children. Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy, aiming to remove the tumor and prevent metastasis. (2) Methods: An institutional retrospective study was conducted with data from 39 patients with primary orbital RMS treated between 1995 and 2016 at the Amsterdam University Medical Centers/Emma Children Hospital. (3) Results: The median age at presentation was 7 years (range, 9 months to 16 years). The median follow-up period was 9.4 years (range, 3 to 25 years). Ten underwent chemotherapy and excision without additional radiotherapy. A total of 29 patients received additional local treatment: Ablative surgery MOld technique with after loading brachytherapy and surgical REconstruction (AMORE) (N = 21), proton (N = 4) or external beam radiation treatment (EBRT; N = 4). We found 14 cases with recurrences, 9 of which underwent exenteration and two of which died. The 10-year overall survival rate was 95% and the EFS was 63%. (4) Conclusions: long-term follow-up with 10-year survival rate of orbital RMS in this series was 95% achieved by local tumor control and eye preservation in 77% of our study population.
（1）背景：眼眶横纹肌肉瘤是一种罕见且具有侵袭性的软组织肿瘤，主要发生于儿童的眼眶区域。治疗通常采用手术、化疗和放疗相结合的综合方案，旨在切除肿瘤并防止转移。（2）方法：本研究为机构回顾性分析，纳入1995年至2016年间在阿姆斯特丹大学医学中心/艾玛儿童医院收治的39例原发性眼眶横纹肌肉瘤患者数据。（3）结果：患者就诊中位年龄为7岁（范围：9个月至16岁），中位随访时间为9.4年（范围：3至25年）。其中10例患者接受化疗联合切除术而未行额外放疗；29例患者接受额外局部治疗：包括AMORE方案（消融手术联合后装近距离放疗及手术重建，N=21）、质子治疗（N=4）或外照射放疗（N=4）。共发现14例复发患者，其中9例接受眶内容物剜除术，2例死亡。10年总生存率为95%，无事件生存率为63%。（4）结论：本系列研究通过局部肿瘤控制实现了95%的10年长期生存率，其中77%的患者成功保留了眼球。
Long-Term Outcomes After Multidisciplinary Treatment for Pediatric Orbital Rhabdomyosarcoma
肿瘤(癌症)患者之家