Sio Terence T, Vu Charles C, Sohawon Schoeb, Van Houtte Paul, Thariat Juliette, Novotny Paul J, Miller Robert C, Bar-Sela Gil
*Department of Radiation Oncology, Mayo Clinic §Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN †Department of Radiation Oncology, Jules Bordet Institute, Brussels, Belgium ‡Department of Radiation Oncology, Cancer Center Antoine-Lacassagne, Nice, France ∥Department of Oncology, Rambam Health Care Campus, Haifa, Israel.
Am J Clin Oncol. 2016 Feb;39(1):32-6. doi: 10.1097/COC.0000000000000005.
To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS).
Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed.
Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P<0.001), primary size >10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P<0.001). In multivariate analysis for DFS, primary size >10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome.
Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS.
报告国际骨外骨肉瘤（ESOS）患者队列的特征、治疗方法及结果。
通过罕见癌症网络收集ESOS患者的回顾性数据。分析患者特征、多模式治疗信息及生存状态。
在4家医疗机构中识别出37例患者。31例（86%）患者患有3级或4级肿瘤。大多数患者（27例[73%]）处于III期疾病。14例（38%）接受了新辅助化疗或放化疗。在28例（85%）接受手术的患者中，21例（75%）实现了切缘阴性，15例（41%）随后接受了辅助化疗。在45个月的中位随访期，20例（55%）患者存活，其中13例（43%）无疾病。单因素分析显示，总生存期差与IV期（P<0.001）、未手术（P<0.001）、原发肿瘤大小>10 cm（P=0.002）及年龄（P=0.002）有关。多因素分析中，原发肿瘤大小>10 cm（P=0.005）是总生存期的预后因素。对于无转移的患者，单因素分析显示无病生存期（DFS）与原发肿瘤大小>10 cm（P=0.003）、手术（P=0.004）、局部复发（P=0.003）及年龄（P<0.001）有关。在DFS的多因素分析中，原发肿瘤大小>10 cm（P=0.01）及年龄较大（P<0.001）对预后较差有显著意义。
多模式治疗仍是局限性ESOS的标准治疗方法，新辅助治疗的指征尚不清楚。肿瘤较大及年龄较大预示DFS较差。
