Baude-Brogniez A, Baranzelli M C, Ferri J, Piot B, Demaille M C, Lecomte-Houcke M, Mercier J, Donazzan M
Service de Chirurgie Maxillo-faciale et Stomatologie, Hôpital R.-Salengro, CHRU, Lille.
Rev Stomatol Chir Maxillofac. 1999 Apr;100(1):34-7.
Ewing's sarcoma is the second most common malignant bone tumor of childhood, yet it is a rare tumor. Primary maxillary localization is unusual and occur in only 1-2% of cases, mostly in the mandible. The prognosis of Ewing's sarcoma has been improving considerably since the introduction of combined modality treatment. The estimated overall survival at 4 years is 75%. It is widely accepted that prompt chemotherapy is necessary to treat occult micrometastasis, present in over 80% of cases at time of diagnosis, and to reduce the tumor size. Prognostic factors correlated with a poor overall survival are large tumor size and poor histologic response to initial chemotherapy. Adequate local control of Ewing's sarcoma can be achieved after chemotherapy, with radical or conservative surgery and radiotherapy. Surgery was recommended whenever possible. Radiation dosage and fields are based upon the quality of surgery and histological response to chemotherapy. Concern has been raised, however, regarding deleterious late effects of radiation in this young population. Conservative surgery and reconstruction are often used to improve functional outcome. We report four cases of Ewing's sarcoma localized to jaw bone and mandible, successfully treated by combined modality treatment.
尤因肉瘤是儿童期第二常见的恶性骨肿瘤，但它是一种罕见肿瘤。原发性上颌骨定位不常见，仅在1%-2%的病例中出现，大多发生在下颌骨。自采用综合治疗模式以来，尤因肉瘤的预后有了显著改善。估计4年总生存率为75%。人们普遍认为，及时进行化疗对于治疗隐匿性微转移（在诊断时超过80%的病例中存在）和缩小肿瘤大小是必要的。与总生存率差相关的预后因素是肿瘤体积大以及对初始化疗的组织学反应差。化疗后，通过根治性或保守性手术及放疗可实现对尤因肉瘤的充分局部控制。只要有可能，就建议进行手术。放射剂量和照射野基于手术质量和对化疗的组织学反应。然而，对于该年轻人群中放疗的有害晚期效应已引起关注。保守性手术和重建常常用于改善功能结局。我们报告4例局限于颌骨和下颌骨的尤因肉瘤病例，通过综合治疗模式成功治愈。
