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多学科团队(MDT)管理在原发性胸内滑膜肉瘤诊治中的价值:单中心经验

The value of multidisciplinary team (MDT) management in the diagnosis and treatment of primary intrathoracic synovial sarcomas: a single-center experience.

作者信息

He Huayu, Yang Lin, Peng Yue, Liu Li, Liu Lei, Xue Qi, Gao Shugeng

机构信息

Department of Thoracic Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

J Thorac Dis. 2021 Feb;13(2):600-612. doi: 10.21037/jtd-20-2887.

Abstract

BACKGROUND

Synovial sarcoma (SS) is a rare malignant soft tissue tumor. Primary intrathoracic SS is extremely rare, with limited diagnosis and treatment experiences. The aim of our study was to retrospectively study the clinicopathological characteristics, treatment and prognosis of primary intrathoracic SS and the impact of multidisciplinary team (MDT) management in diagnosis and treatment on patient prognosis.

METHODS

The clinical and pathological characteristics, treatment, survival and prognosis of patients with primary intrathoracic SS admitted to the National Cancer Center from January 1999 to December 2018, as well as MDT intervention during diagnosis and treatment, were retrospectively analyzed.

RESULTS

Thirteen patients were enrolled, including 7 (53.8%) males and 6 (46.3%) females, with primary intrathoracic SS in the lung (8/13, 61.5%), mediastinum (4/13, 30.8%) and pleura (1/13, 7.7%) as confirmed by morphological observation, immunohistochemical (IHC) staining and fluorescence in situ hybridization (FISH). Overall, 10/13 (76.9%) patients underwent surgery, and 6/10 (60.0%) received postoperative adjuvant therapy. Only 23.1% of patients received nonsurgical therapy. The MDT discussed and managed seven patients before and/or after surgery and one patient who did not undergo surgery. The estimated 3- and 5-year overall survival (OS) rates were 50.0% and 30.0%, respectively. Patients who were managed by an MDT had a longer median OS time than those who were not (46.0 18.0 months). Age (P=0.018), tumor location (P=0.029), and Ki-67 (P=0.020) were found to be significantly related to OS.

CONCLUSIONS

Monophasic morphology and fusion gene characteristics are the main features for the diagnosis of primary intrathoracic SS. MDT management can help obtain accurate diagnoses and provide reasonable therapeutic options.

摘要

背景

滑膜肉瘤(SS)是一种罕见的恶性软组织肿瘤。原发性胸内滑膜肉瘤极为罕见,诊断和治疗经验有限。本研究的目的是回顾性研究原发性胸内滑膜肉瘤的临床病理特征、治疗及预后,以及多学科团队(MDT)管理在诊断和治疗中对患者预后的影响。

方法

回顾性分析1999年1月至2018年12月在国家癌症中心收治的原发性胸内滑膜肉瘤患者的临床病理特征、治疗、生存及预后情况,以及诊断和治疗期间的MDT干预情况。

结果

共纳入13例患者,其中男性7例(53.8%),女性6例(46.3%),经形态学观察、免疫组化(IHC)染色及荧光原位杂交(FISH)证实,原发性胸内滑膜肉瘤位于肺(8/13,61.5%)、纵隔(4/13,30.8%)和胸膜(1/13,7.7%)。总体而言,10/13(76.9%)例患者接受了手术,6/10(60.0%)例患者接受了术后辅助治疗。仅23.1%的患者接受了非手术治疗。MDT在手术前和/或手术后讨论并管理了7例患者,以及1例未接受手术的患者。估计3年和5年总生存率(OS)分别为50.0%和30.0%。接受MDT管理的患者中位OS时间比未接受MDT管理的患者更长(46.0比18.0个月)。发现年龄(P=0.018)、肿瘤位置(P=0.029)和Ki-67(P=0.020)与OS显著相关。

结论

单相形态和融合基因特征是诊断原发性胸内滑膜肉瘤的主要特征。MDT管理有助于获得准确诊断并提供合理的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/677c/7947479/2488dcaac397/jtd-13-02-600-f1.jpg

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