Tiwari Akshay, Gupta Himesh, Jain Sandeep, Kapoor Gauri
Department of Surgical Oncology, Rajiv Gandhi Cancer Institute and Research Center, Rohini, Delhi, India.
Indian J Orthop. 2010 Oct;44(4):378-83. doi: 10.4103/0019-5413.69307.
The management of Ewing's sarcoma family of tumors (ESFT, Ewing's sarcoma/primitive neuroectodermal tumor) has been established as a multimodality treatment. Advances in imaging and diagnostics, chemotherapy, surgical techniques, radiotherapy and prosthetic technology have resulted in drastic changes in the outcome of this disease, with most of the recent studies having 5-year survival rates of more than 60%. The Indian patients present at a more advanced stage and the compliance of treatment is suboptimal. While there is plenty of data in the world literature on the outcome of Ewing's sarcoma, there is paucity of data in Indian patients. Therefore, we conducted the present study to analyze the outcome of multimodality treatment of ESFT of the extremities at a tertiary nonprofit institute over a decade.
34 patients who had histopathologically proven diagnosis of Ewing's sarcoma of the extremities and had received treatment at our institute from 1997 through 2007 were included for analysis. The majority of patients had involvement of the femur (35%), followed by tibia (17%), fibula and foot (15% each), humerus (12%) and soft tissue of thigh (6%). Twenty-nine patients presented with localized disease (Enneking stage II B) while five patients presented with metastases (Enneking stage III). All patients received Vincristine, Actinomycin D, Cyclofosfamide + Ifosfamide and Etoposide (VAC+IE)-based chemotherapy and local treatment was offered to all but three patients having multicentric disease. The local treatment offered were, radiation (n= 15), surgery (n= 12) both surgery and radiation (n=4). All patients were analyzed for oncological outcome (event-free and overall survival, local and systemic relapses) by clinical and imaging evaluation and functional outcome by using the musculoskeletal tumor society (MSTS) score. These outcomes were correlated with age, sex, size of tumor, stage at presentation, modality of local treatment and site of relapse.
At the final follow-up (mean, 26 months; median, 17 months; range, 3-97 months), the overall and event-free survivals were 47 ± 12% and 34 ± 9%, respectively. Sixty-two percent of the patients presented with a tumor size more than 8 cm. On correlation with age, sex, size of tumor, stage at presentation, modality of local treatment and site of relapse, no correlation of survival was seen with any of the variables except event-free survival with size of the tumor. The functional outcome of all the patients was satisfactory (MSTS score >16 out of 30). No patient underwent amputation.
Although the demographic profile, stage at presentation and the local and systemic treatment regimen followed in our study was similar to the world literature, the outcome of Ewing's sarcoma in Indian patients were found to be inferior to that reported in the western literature. Larger multicentric studies with longer follow-up are required to exactly determine the key areas crucial in improving this outcome.
尤因肉瘤家族性肿瘤（ESFT，尤因肉瘤/原始神经外胚层肿瘤）的治疗已确立为多模式治疗。影像学和诊断学、化疗、手术技术、放射治疗和假体技术的进步导致了这种疾病治疗结果的巨大变化，最近的大多数研究5年生存率超过60%。印度患者就诊时疾病分期更晚，治疗依从性也欠佳。虽然世界文献中有大量关于尤因肉瘤治疗结果的数据，但印度患者的数据却很少。因此，我们开展了本研究，以分析一家非营利性三级机构在十年间对四肢ESFT进行多模式治疗的结果。
纳入34例经组织病理学确诊为四肢尤因肉瘤且于1997年至2007年在我院接受治疗的患者进行分析。大多数患者的股骨受累（35%），其次是胫骨（17%）、腓骨和足部（各15%）、肱骨（12%）以及大腿软组织（6%）。29例患者为局限性疾病（Enneking II B期），5例患者有转移（Enneking III期）。所有患者均接受了基于长春新碱、放线菌素D、环磷酰胺+异环磷酰胺和依托泊苷（VAC+IE）的化疗，除3例有多中心疾病的患者外，所有患者均接受了局部治疗。提供的局部治疗方法有：放疗（n=15）、手术（n=12）、手术加放疗（n=4）。通过临床和影像学评估分析所有患者的肿瘤学结局（无事件生存和总生存、局部和全身复发），并使用肌肉骨骼肿瘤学会（MSTS）评分评估功能结局。这些结局与年龄、性别、肿瘤大小、就诊时分期、局部治疗方式和复发部位相关。
在最后一次随访时（平均26个月；中位数17个月；范围3 - 97个月），总生存率和无事件生存率分别为4
