Abdel Rahman Hany, El-Baradie Tarek, El-Baradie Manal, Bahaa Sherif, Shalan Mohammed
The Departments of Pediatric Oncology, National Cancer Institute, Cairo University, Egypt.
J Egypt Natl Canc Inst. 2010 Mar;22(1):41-7.
Ewing's sarcoma accounts for 4-6% of primary malignant bone tumors and it affects the head and neck in only 1-4% of cases. The purpose of this study was to review the NCI experience with Ewing's sarcoma of the head and neck in children.
A retrospective analysis of patient files with head and neck Ewing's sarcoma treated at the National Cancer Institute, Cairo University, Egypt, during the period from 1997 to 2008 was done. Files were reviewed and data for patients, tumor and treatment profile were extracted.
Twenty patients out of 280 with Ewing's sarcoma were identified during an 11-year period. Patients had a median age of 11.5 years (range 5 months - 22 years) with a male to female ratio of 1:1. The most common tumor site was in the mandible (9/20, 45%) followed by a neck mass (4/20, 20%) and a clavicular mass (3/20, 15%). Six patients (30%) were metastatic at presentation. Most of the patients (19/20, 95%) received chemotherapy. Local therapy was in the form of radical radiotherapy for 8 patients (40%), 2 patients (10%) had surgery alone, while five patients (25%) had surgical resection and postoperative radiotherapy. Overall survival ranged from 1 to 128 months, with a median of 36 months. At the end of the study, 9 patients (45%) were alive in CR, 6 (30%) were lost to FU in disease progression, while 5 patients died from disease progression.
Ewing's sarcoma of the head and neck is a disease of a rare incidence with debate about the optimum local therapy. Small non-metastatic tumors with good response to chemotherapy have abetter outcome.
Ewing's sarcoma - Head and neck - Management.
尤因肉瘤占原发性恶性骨肿瘤的4% - 6%，仅1% - 4%的病例发生于头颈部。本研究旨在回顾美国国立癌症研究所（NCI）治疗儿童头颈部尤因肉瘤的经验。
对1997年至2008年期间在埃及开罗大学国立癌症研究所接受治疗的头颈部尤因肉瘤患者档案进行回顾性分析。查阅档案并提取患者、肿瘤及治疗情况的数据。
在11年期间，280例尤因肉瘤患者中有20例被确诊。患者的中位年龄为11.5岁（范围5个月至22岁），男女比例为1:1。最常见的肿瘤部位是下颌骨（9/20，45%），其次是颈部肿块（4/20，20%）和锁骨肿块（3/20，15%）。6例患者（30%）初诊时已有转移。大多数患者（19/20，95%）接受了化疗。局部治疗方式为8例患者（40%）接受根治性放疗，2例患者（10%）仅接受手术，5例患者（25%）接受手术切除及术后放疗。总生存期为1至128个月，中位生存期为36个月。研究结束时，9例患者（45%）处于完全缓解（CR）状态存活；6例患者（30%）在疾病进展期失访；5例患者死于疾病进展。
头颈部尤因肉瘤发病率罕见，关于最佳局部治疗存在争议。对化疗反应良好的小的非转移性肿瘤预后较好。
尤因肉瘤 - 头颈部 - 治疗
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