Abstract
BackgroundAdult Ewing sarcoma (ES) is a rare disease, the optimal treatment model is unknown. This study aimed to retrospectively analyze treatment-related prognostic factors of nonspinal ES in Chinese adults.MethodsEighty-one patients treated between January 2005 and December 2017 were included in the present study. Thirty-three (40.7%) presented with metastatic disease at diagnosis. Eight patients were submitted to primary surgery followed by chemotherapy, while 73 patients received chemotherapy before and after surgery and/or local radiotherapy. The chemotherapy regimen included 8–17 cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) alternating with ifosfamide and etoposide (IE) every 3 weeks. Clinical outcomes and safety were analyzed.ResultsVDC/IE chemotherapy was well tolerated in adult patients with ES. Multivariate Cox regression analyses revealed that chemotherapy of at least 12 cycles was a favorable independent prognostic factor of event-free survival (hazard ratio, 0.558; 95% confidence interval, 0.323–0.965; P = 0.037) and overall survival (hazard ratio, 0.424; 95% confidence interval, 0.240–0.748; P = 0.003). Similarly, a low frequency of chemotherapy delays was an independent prognostic factor of improved OS (hazard ratio, 0.438; 95% confidence interval, 0.217–0.887; P = 0.022).ConclusionOur study suggests that adults with ES should be treated with an aggressive multidisciplinary approach, intensive chemotherapy with adequate cycles and appropriate intervals can be recommended in this group.
Highlights
Adult Ewing sarcoma (ES) is a rare disease, the optimal treatment model is unknown
Among all 81 patients, 48 (59.3%) patients presented with localized disease, 33 (40.7%) patients presented with metastatic disease (14 with lung metastases, 10 with bone metastases, 1 with lymphonode metastases, 7 with both lung and bone metastases, 1 with simultaneous bone and bone marrow metastases) at diagnosis (Table 1)
We followed the pediatric VDC/ifosfamide and etoposide (IE) protocol, 50 patients didn’t completed the planned 17 chemotherapy cycles due to various reasons: the most common reason was lack of money (21 patients, 42%), followed by the belief that adults inevitably fare worse than children and increasing chemotherapy duration may not lead to improvement in survival (16 patients, 32%), toxicity-related treatment abandonment occurred in eight patients (16%), and other reasons accounted for five patients (10%)
Summary
Adult Ewing sarcoma (ES) is a rare disease, the optimal treatment model is unknown. Ewing sarcoma (ES) is the second most common primary bone malignancy in children, but is extremely rare in adults [1,2,3]. The treatment of ES relies on a multidisciplinary approach that couples risk-adapted chemotherapy and local therapy (surgery, radiation therapy, or both). Chemotherapy plays a pivotal role in the treatment of ES. The regimen including vincristine, doxorubicin, and cyclophosphamide (VDC) alternating with ifosfamide and etoposide (IE) is a category 1 recommendation for ES in NCCN guideline [4]. There is no consensus on the optimal chemotherapy cycles and intervals. It was reported that VDC/IE every 3 weeks for 14 cycles contributed to similar survival compared with
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