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Natural history of undifferentiated pleomorphic sarcoma: Experience from the US Sarcoma Collaborative
Journal of Surgical Oncology ( IF 2.5 ) Pub Date : 2024-04-02 , DOI: 10.1002/jso.27620
Eleftherios A. Makris 1 , Thuy B. Tran 1 , Daniel J. Delitto 1 , Byrne Lee 1 , Cecilia G. Ethun 2 , Valerie Grignol 3 , J. Harrison Howard 3 , Meena Bedi 4 , T. Clark Gamblin 4 , Jennifer Tseng 5 , Kevin K. Roggin 5 , Konstantinos Chouliaras 6 , Konstantinos Votanopoulos 6 , Darren Cullinan 7 , Ryan C. Fields 7 , Kenneth Cardona 2 , George Poultsides 1 , Amanda Kirane 1
Affiliation  

BackgroundUndifferentiated pleomorphic sarcoma (UPS) is a relatively rare but aggressive neoplasm. We sought to utilize a multi‐institutional US cohort of sarcoma patients to examine predictors of survival and recurrence patterns after resection of UPS.MethodsFrom 2000 to 2016, patients with primary UPS undergoing curative‐intent surgical resection at seven academic institutions were retrospectively reviewed. Epidemiologic and clinicopathologic factors were reviewed by site of origin. Overall survival (OS), recurrence‐free survival (RFS), time‐to‐locoregional (TTLR), time‐to‐distant recurrence (TTDR), and patterns of recurrence were analyzed.ResultsOf the 534 UPS patients identified, 53% were female, with a median age of 60 and median tumor size of 8.5 cm. The median OS, RFS, TTLR, and TTDR for the entire cohort were 109, 49, 86, and 46 months, respectively. There were no differences in these survival outcomes between extremity and truncal UPS. Compared with truncal, extremity UPS were more commonly amenable to R0 resection (87% vs. 75%, p = 0.017) and less commonly associated with lymph node metastasis (1% vs. 6%, p = 0.031). R0 resection and radiation treatment, but not site of origin (extremity vs. trunk) were independent predictors of OS and RFS. TTLR recurrence was shorter for UPS resected with a positive margin and for tumors not treated with radiation.ConclusionFor patients with resected extremity and truncal UPS, tumor size >5 cm and positive resection margin are associated with worse survival OS and RFS, irrespectively the site of origin. R0 surgical resection and radiation treatment may help improve these survival outcomes.

中文翻译:

未分化多形性肉瘤的自然史:美国肉瘤合作组织的经验

背景未分化多形性肉瘤(UPS)是一种相对罕见但具有侵袭性的肿瘤。我们试图利用美国多机构肉瘤患者队列来检查 UPS 切除后生存和复发模式的预测因素。方法对 2000 年至 2016 年在七个学术机构接受根治性手术切除的原发性 UPS 患者进行回顾性分析。按起源地审查流行病学和临床病理学因素。分析了总生存期 (OS)、无复发生存期 (RFS)、局部区域时间 (TTLR)、远期复发时间 (TTDR) 和复发模式。 结果 在确定的 534 名 UPS 患者中,53%女性,中位年龄 60 岁,中位肿瘤大小 8.5 cm。整个队列的中位 OS、RFS、TTLR 和 TTDR 分别为 109、49、86 和 46 个月。四肢 UPS 和躯干 UPS 的生存结果没有差异。与躯干相比,四肢 UPS 更容易接受 R0 切除(87% vs. 75%,p= 0.017),且与淋巴结转移不太常见(1% vs. 6%,p= 0.031)。 R0 切除和放射治疗,但不是原发部位(四肢与躯干),是 OS 和 RFS 的独立预测因素。对于切缘阳性的 UPS 和未经放射治疗的肿瘤,TTLR 复发时间较短。 结论 对于切除四肢和躯干 UPS 的患者,肿瘤大小 > 5 cm 和切缘阳性与较差的生存 OS 和 RFS 相关,无论肿瘤部位如何起源。 R0 手术切除和放射治疗可能有助于改善这些生存结果。
更新日期:2024-04-02
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