Günalp I, Dürük K, Gündüz K
Tumor Department, Eye Clinic, Faculty of Medicine, Ankara University, Turkey.
Jpn J Ophthalmol. 1993;37(4):499-504.
Orbital rhabdomyosarcoma is the most frequently encountered primary malignant orbital tumor in children. Between 1970 and 1991, 68 primary orbital rhabdomyosarcoma cases were diagnosed and treated in our clinic. Mean age at the time of diagnosis was 8.8 years. Seventy-two percent of the cases were of the embryonal type while 14% had alveolar and 14% had botryoid tumors. Following tissue diagnosis by anterior orbitotomy, radiotherapy and multimodal chemotherapy were instituted promptly. Subtotal exenteration was used in massive orbital involvement and total exenteration in recurrences. Using the Kaplan-Meier method of analysis, the survival rate was 84% at three years and 80% at five years. While morbidity due to the disease itself and treatment complications may still be a problem, the survival rates achieved in the long term are promising.
眼眶横纹肌肉瘤是儿童中最常见的原发性眼眶恶性肿瘤。1970年至1991年期间，我院共诊断并治疗了68例原发性眼眶横纹肌肉瘤病例。诊断时的平均年龄为8.8岁。其中72%为胚胎型，14%为肺泡型，14%为葡萄状肿瘤。经前路眼眶切开术进行组织诊断后，立即开始放疗和多模式化疗。对于广泛的眼眶受累采用次全眶内容剜除术，复发病例采用全眶内容剜除术。采用Kaplan-Meier分析方法，三年生存率为84%，五年生存率为80%。虽然疾病本身和治疗并发症导致的发病率可能仍然是一个问题，但长期取得的生存率很有希望。
