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未分化多形性软组织肉瘤的预后预测:266例患者的20年经验

Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20-year experience of 266 cases.

作者信息

Vodanovich Domagoj A, Spelman Tim, May Deborah, Slavin John, Choong Peter F M

机构信息

Department of Orthopaedics, St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.

Department of Surgery, St Vincent's Hospital Melbourne, The University of Melbourne, Melbourne, Victoria, Australia.

出版信息

ANZ J Surg. 2019 Sep;89(9):1045-1050. doi: 10.1111/ans.15348. Epub 2019 Jul 30.

DOI:10.1111/ans.15348
PMID:31364245
Abstract

BACKGROUND

Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment.

METHODS

Data of UPS tumours from 1996 to 2016 were collected, totalling 266 unique UPS patients. Median follow-up was 7.8 years. All tumours were retrospectively analysed for prognostic factors of the disease, including local recurrence (LR) and metastatic disease (MD) at diagnosis, tumour size, grade, location and depth, patient age, adjuvant therapy and surgical margin. Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes.

RESULTS

The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P < 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P < 0.001; 4.42), ≥10 cm (P < 0.001; 6.80)) and MD at diagnosis (P < 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P < 0.001; 0.34).

CONCLUSIONS

UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.

摘要

背景

未分化多形性肉瘤(UPS)是一种罕见的间叶源性恶性肿瘤,它是在恶性纤维组织细胞瘤(MFH)重新分类后产生的。本研究的目的是确定多模式治疗后UPS预后的相关因素。

方法

收集1996年至2016年期间UPS肿瘤的数据,共有266例不同的UPS患者。中位随访时间为7.8年。对所有肿瘤进行回顾性分析,以确定疾病的预后因素,包括诊断时的局部复发(LR)和转移性疾病(MD)、肿瘤大小、分级、位置和深度、患者年龄、辅助治疗和手术切缘。将总生存期(OS)、治疗后LR和无转移生存期作为观察指标。

结果

所有年龄段的5年和10年总生存率分别为60%和48%,中位生存时间为10.1年。多变量分析显示,与总生存期降低相关的不良预后因素为年龄较大(P < 0.001;风险比1.03)和诊断时存在MD(P = 0.001;2.89),上肢肿瘤预后较好(P = 0.043;2.30)。术后LR预后不良与年龄较大(P = 0.046;1.03)和手术切缘阳性(P = 0.028;2.68)有关。肿瘤较大(5 - 9厘米(P < 0.001;4.42),≥10厘米(P < 0.001;6.80))以及诊断时存在MD(P < 0.001;3.99)的患者治疗后MD增加,辅助治疗预后较好,可降低MD(P < 0.001;0.34)。

结论

UPS是一种高级别软组织肉瘤,争取切缘阴性的手术联合放疗是其首选治疗方法。年龄较大、下肢部位、就诊时存在MD、肿瘤较大和手术切缘阳性均为不利因素。

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