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骨盆尤因肉瘤:临床特征与总生存期

Ewing sarcoma of the pelvis: Clinical features and overall survival.

作者信息

Brown Jeffrey Mark, Rakoczy Kyla, Tokson Jacqueline Hart, Jones Kevin B, Groundland John S

机构信息

University of Miami Miller School of Medicine, Orthopaedics, Miami, FL, United States of America; Department of Orthopaedics, Musculoskeletal Surgical Oncology, University of Utah, Huntsman Cancer Institute, Salt Lake City, UT, United States of America.

University of Miami Miller School of Medicine, Orthopaedics, Miami, FL, United States of America.

出版信息

Cancer Treat Res Commun. 2022;33:100634. doi: 10.1016/j.ctarc.2022.100634. Epub 2022 Sep 13.

DOI:10.1016/j.ctarc.2022.100634
PMID:36126512
Abstract

INTRODUCTION

Primary Ewing Sarcoma of Bone is a malignancy whose treatment requires both systemic chemotherapy and local control through surgical resection and/or radiation. Ewing Sarcoma of the pelvis has been noted to confer a worse prognosis relative to other anatomic sites of Ewing Sarcoma. This study explores the presenting features, treatment modalities for local control, and overall survival of primary Ewing sarcoma of the pelvis in comparison to other commonly affected anatomic sites.

METHODS

The National Cancer Institute Surveillance, Epidemiology, and End-Results (SEER) database was used to identify cases of pelvic Ewing sarcoma diagnosed between years 2004 and 2015. Demographic variables including sex, race, and age at diagnosis were described for each case, as well as therapeutic modalities including surgery and radiation. Bone-specific Collaborative Staging variables, including tumor size, tumor extension, and metastasis at diagnosis, were described for the same cohort. Univariate and multivariate assessments were performed for statistical comparison between presenting factors, treatment modalities, and between anatomic locations of presentation.

RESULTS

Within the database, 296 patients with Ewing sarcoma of the pelvic bones were available for review, which represented 25.7% of the 1152 cases surveyed across all anatomic sites. In the subset of patients with Ewing Sarcoma of the pelvis, 63.5% were male; the median age of diagnosis was 17 years; extra-compartmental tumor extension was noted in 82.1%; average tumor size was 9.7 cm; and metastasis at diagnosis was noted in 46.1% of the cohort. Only 28.6% of the pelvis sarcoma patients received surgical resection as part or all of their local control treatment, while 67.6% received some form of radiation therapy. When compared to the presenting features of Ewing Sarcoma from other anatomic sites, patients with pelvic tumors had larger tumors at time of diagnosis, higher rates of metastatic disease, and were less likely to undergo surgical intervention. The 2-, 5-, and 10-year overall survival rates for the patients presenting with Ewing Sarcoma of the pelvis was 70.3%, 49.7%, and 41.9%, respectively, which were significantly lower across all time-points than any other anatomic site.

DISCUSSION AND CONCLUSION

Ewing Sarcoma of the pelvis is an aggressive malignancy that presents with relatively large tumors and a high rate of metastatic dissemination. Surgical intervention is less frequent when Ewing Sarcoma presents in the pelvis than when it presents in other anatomic locations. These factors may contribute to the worse overall survival of Ewing Sarcoma when compared to the same diagnosis originating in other anatomic sites. Prospective, randomized study is required to determine the true causal effects of these factors on survival.

摘要

引言

原发性骨尤文肉瘤是一种恶性肿瘤,其治疗需要全身化疗以及通过手术切除和/或放疗进行局部控制。与尤文肉瘤的其他解剖部位相比,骨盆尤文肉瘤的预后较差。本研究探讨了骨盆原发性尤文肉瘤与其他常见受累解剖部位相比的临床表现、局部控制的治疗方式以及总生存率。

方法

利用美国国立癌症研究所监测、流行病学和最终结果(SEER)数据库,确定2004年至2015年间诊断为骨盆尤文肉瘤的病例。描述了每个病例的人口统计学变量,包括性别、种族和诊断时的年龄,以及治疗方式,包括手术和放疗。对同一队列描述了骨特异性协作分期变量,包括肿瘤大小、肿瘤扩展和诊断时的转移情况。对临床表现因素、治疗方式以及临床表现的解剖部位之间进行单因素和多因素评估,以进行统计比较。

结果

在数据库中,有296例骨盆骨尤文肉瘤患者可供审查,占所有解剖部位调查的1152例病例的25.7%。在骨盆尤文肉瘤患者亚组中,63.5%为男性;诊断的中位年龄为17岁;82.1%的患者出现隔室外肿瘤扩展;平均肿瘤大小为9.7厘米;46.1%的队列在诊断时出现转移。只有28.6%的骨盆肉瘤患者接受了手术切除作为其局部控制治疗的一部分或全部,而67.6%的患者接受了某种形式的放射治疗。与其他解剖部位的尤文肉瘤临床表现相比,骨盆肿瘤患者在诊断时肿瘤更大,转移疾病发生率更高,且接受手术干预的可能性更小。骨盆尤文肉瘤患者的2年、5年和10年总生存率分别为70.3%、49.7%和41.9%,在所有时间点均显著低于任何其他解剖部位。

讨论与结论

骨盆尤文肉瘤是一种侵袭性恶性肿瘤,表现为相对较大的肿瘤和较高的转移扩散率。尤文肉瘤出现在骨盆时,手术干预比出现在其他解剖部位时更少。与起源于其他解剖部位的相同诊断相比,这些因素可能导致尤文肉瘤的总生存率更差。需要进行前瞻性随机研究来确定这些因素对生存的真正因果效应。

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