Siebenrock K A, Nascimento A G, Rock M G
Department of Orthopaedics, University of Bern, Switzerland.
Clin Orthop Relat Res. 1996 Aug(329):288-99. doi: 10.1097/00003086-199608000-00036.
Thirty-four cases with either extraskeletal Ewing's sarcoma (21 patients) or malignant peripheral neuroectodermal tumor of the soft tissues (13 patients) were reviewed retrospectively. The patients were treated between 1964 and 1991. Followup periods averaged 7.2 years for patients with peripheral neuroectodermal tumors and 10.4 years for patients with Ewing's sarcoma (minimal followup of 2 years). There were no significant differences in patient's age, gender, tumor location, and stages on presentation between patients with Ewing's sarcoma and those with malignant peripheral neuroectodermal tumor of the soft tissue. All but 2 patients underwent surgery for tumor resection. Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. The 5-year overall survival was 50% for Ewing's sarcoma and 44% for peripheral neuroectodermal tumor. The 5-year disease free survival was 33% for both types of tumors. Survival rates were higher for Stage III compared with Stage IV disease for both types of tumors. There was a tendency for better outcome after complete surgical tumor resection. Survivors tended to be of a younger age at the time of tumor diagnosis. The results suggest that from a clinical perspective, these 2 tumors are quite similar.
对34例骨外尤因肉瘤患者（21例）和软组织恶性外周神经外胚层肿瘤患者（13例）进行了回顾性研究。这些患者于1964年至1991年期间接受治疗。外周神经外胚层肿瘤患者的随访期平均为7.2年，尤因肉瘤患者的随访期平均为10.4年（最短随访期为2年）。尤因肉瘤患者和软组织恶性外周神经外胚层肿瘤患者在患者年龄、性别、肿瘤位置以及就诊时的分期方面均无显著差异。除2例患者外，所有患者均接受了肿瘤切除术。95%的尤因肉瘤患者和85%的外周神经外胚层肿瘤患者接受了辅助治疗（放疗或化疗或两者皆用）。尤因肉瘤的5年总生存率为50%，外周神经外胚层肿瘤为44%。两种类型肿瘤的5年无病生存率均为33%。两种类型肿瘤的III期患者生存率均高于IV期。肿瘤完全手术切除后的预后有更好的趋势。幸存者在肿瘤诊断时往往年龄较小。结果表明，从临床角度来看，这两种肿瘤非常相似。
