Humpl T, Brühl K, Pitz S, de Mooy P, Bartels U, Gutjahr P
Kinderklinik, Klinikum der Johannes Gutenberg-Universität Mainz.
Ophthalmologe. 1997 Dec;94(12):914-9. doi: 10.1007/s003470050221.
Rhabdomyosarcoma is the most common malignant orbital tumor in children. Treatment modalities (individualized therapy or study protocol) have been changed radically. Surgery was supplemented by radiation therapy and chemotherapy. The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution.
Between 1954 and 1995, 18 patients (age at presentation 1 month to 17 years, 11 male and 7 females) with orbital rhabdomyosarcoma were diagnosed and treated at our institution.
The 5-year survival rate was 76%. Primary exenteration was replaced by tumor resection (microsurgery), radiation therapy and chemotherapy. Favorable outcome in the precytostatic era (with permanent loss of function) was not significantly improved by polychemotherapy. Microsurgery seems to increase the risk of recurrence.
Considering the high risk of recurrence, primary exenteration in selected patients with expanded primary tumor is still a therapeutic option.
横纹肌肉瘤是儿童最常见的眼眶恶性肿瘤。治疗方式（个体化治疗或研究方案）已发生了根本性变化。手术辅以放射治疗和化疗。我们进行回顾性分析的目的是在一个单一机构的未筛选患者群体中确定与治疗变化相关的预后情况。
1954年至1995年间，我们机构诊断并治疗了18例眼眶横纹肌肉瘤患者（就诊时年龄1个月至17岁，男11例，女7例）。
5年生存率为76%。原发灶眼球摘除术已被肿瘤切除术（显微手术）、放射治疗和化疗所取代。在细胞毒性化疗时代之前（伴有永久性功能丧失）的良好预后并未因多药化疗而得到显著改善。显微手术似乎会增加复发风险。
考虑到复发风险高，对于部分原发肿瘤扩大的患者，原发灶眼球摘除术仍是一种治疗选择。
