Mannor G E, Rose G E, Plowman P N, Kingston J, Wright J E, Vardy S J
Orbital Clinic, Moorfields Eye Hospital, London, England.
Ophthalmology. 1997 Jul;104(7):1198-201. doi: 10.1016/s0161-6420(97)30162-6.
Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recurrence or persistence of the local orbital tumor still occurs. There are no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma.
From clinical databases, 67 children with orbital rhabdomyosarcoma were identified. Seven (10%) of the 67 children had tumors refractory to combined chemotherapy and radiation therapy and underwent exenteration or eye-sparing tumor excision. Their clinical course and outcome were reviewed retrospectively.
No patient was lost to follow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven children with refractory tumor are still alive at more than 3 years after surgery (3.2-11 years; mean, 6.9). In one of the two children who died, tumor extended beyond the operative margins at exenteration, and the other child died with regional metastasis within a month of exenteration.
Although more than 90% of children with orbital rhabdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. All of the five surviving children appear to be disease free.
联合化疗和放疗提高了原发性眼眶横纹肌肉瘤患儿的生存率，但眼眶局部肿瘤仍会复发或持续存在。目前尚无针对这些特殊患儿的既定治疗指南，本文作者对难治性原发性眼眶横纹肌肉瘤患儿的联合治疗方法及疗效进行了综述。
从临床数据库中筛选出67例眼眶横纹肌肉瘤患儿。其中7例（10%）对联合化疗和放疗无效，接受了眶内容摘除术或保留眼球的肿瘤切除术。对他们的临床病程及治疗结果进行回顾性分析。
所有患者均获随访，随访时间为3.2至11年。7例难治性肿瘤患儿中，5例（71%）术后3年以上仍存活（3.2至11年；平均6.9年）。死亡的2例患儿中，1例在眶内容摘除术时肿瘤超出手术切缘，另1例在眶内容摘除术后1个月内死于区域转移。
尽管超过90%的眼眶横纹肌肉瘤患儿对儿科肿瘤学家和放疗师的联合治疗有反应，但眼科医生进行的眼眶局部（挽救性）手术对少数难治性肿瘤患儿可能有价值。5例存活患儿均无疾病迹象。
