Extraskeletal Ewing's Sarcoma Family of Tumours in Adults: Analysis of 57 Patients from a Single Institution
Extraskeletal Ewing's Sarcoma Family of Tumours in Adults: Analysis of 57 Patients from a Single Institution
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DOI：
10.1016/j.clon.2010.02.010
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发表时间：
2010-06-01
影响因子：
3.4
通讯作者：
Memon, M.
中科院分区：
文献类型：
--
作者：
El Weshi, A.;Allam, A.;Memon, M.
目的皮肤外尤文氏肉瘤（EES）是一种罕见的软组织肉瘤，本研究的目的是评估结果和预后的成年患者提出的EES治疗的多模态治疗材料和方法所有大于15岁的EES患者转介到我们的机构在1995年1月至2004年12月。57名患者被确定，他们诊断时的中位年龄为20岁。结果原发肿瘤的中位直径为11 cm（范围4-30 cm）18例患者（31%）在初始表现时有转移性疾病，23例（40%）实现了边缘阴性的广泛手术切除化疗包括长春新碱、阿霉素、异环磷酰胺，50例（88%）给予放线菌素D治疗，37例（65%）给予放射治疗，41例（72%）达到完全缓解，16例（28%）在治疗中进展，21例（51%）复发，15例（36%）局部复发。长达46个月（范围6-143个月），5年无事件生存率和总生存率分别为35%和47%。肿瘤大小和手术切缘与总生存率和事件显著相关，结论EES是一种侵袭性肿瘤，局部复发和远处转移的发生率高。在对多模式治疗的反应和影响治疗结果的预后因素方面，成人EES与骨骼尤文肉瘤没有什么不同。适当的手术切除、积极的化疗和辅助局部放疗，如有指征，构成了在这种罕见疾病中实现最佳结果的最佳治疗（C）2010由Elsevier Ltd出版的皇家放射科医师学院版权所有
Aims Extraskeletal Ewing's sarcoma (EES) is a rare form of soft tissue sarcoma the aim of the present study was to assess the outcome and the prognosis of adult patients presenting with EES treated with multi-modality therapyMaterials and methods All EES patients older than 15 years referred to our institution between January 1995 and December 2004 were reviewed In total, 57 patients were identified Their median age at diagnosis was 20 years (range 15-57)Results The median size of the primary tumour was 11 cm (range 4-30 cm) Eighteen patients (31%) had metastatic disease at initial presentation Wide surgical resection with negative margins was achieved in 23 cases (40%) Chemotherapy consisting of vincristine, adriamycin, ifosfamide, actinomycin D was given in 50 patients (88%) Radiotherapy was delivered in 37 patients (65%) Forty-one patients (72%) achieved complete remission and 16 (28%) progressed on therapy Twenty-one patients (51%) relapsed Local recurrence was encountered in 15 patients (36%) At a median follow-up of 46 months (range 6-143 months), the 5-year event-free survival and overall survival rates were 35 and 47%, respectively Metastases at presentation, tumour size and surgical resection margin associated significantly with overall survival and event-free survivalConclusion EES is an aggressive type of tumour with a high incidence of local recurrence and distant metastasis This series showed that the outcome of adult EES is not unlike that of skeletal Ewing's sarcoma in terms of response to multi-modality treatment and the prognostic factors influencing treatment outcome Adequate surgical resection, aggressive chemotherapy and adjuvant local radiation therapy, when indicated, constitute the optimal treatment to achieve the best results in this rare type of disease (C) 2010 The Royal College of Radiologists Published by Elsevier Ltd All rights reserved