Department of Medical Oncology, Chris O'Brien Lifehouse, Sydney, New South Wales, Australia.
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia.
Cancer Rep (Hoboken). 2024 Apr;7(4):e2065. doi: 10.1002/cnr2.2065.
Extrapleural pneumonectomy (EPP) is a complex surgical procedure involving en-bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural-based sarcoma of predominantly pediatric patients suggest EPP may be a life-prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center.
Clinicopathologic variables, surgical details, and follow-up information were extracted for patients undergoing EPP for pleural-based sarcoma between August 2017 and December 2020. Primary outcomes were event-free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease-free interval (DFI) prior to EPP, and early and late postoperative complications.
Eight patients were identified, seven with soft tissue sarcoma and one with bone sarcoma. Patients had either localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. All patients underwent resection of their pleural-based sarcoma by an experienced cardiothoracic surgeon, and some patients had pre or postoperative treatment. The perioperative morbidity was comparable with previously published reports of EPP performed in mesothelioma patients. At median follow-up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients (75%) had disease recurrence; five (62.5%) died of progressive disease. Two patients (25%) had not recurred: one died of a radiation-related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low-grade histology and achieving a metabolic response to preoperative chemotherapy.
In adults with pleural-based sarcoma, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes. In the absence of a confirmed response to neoadjuvant treatment, long term survival is poor and EPP should not be recommended.
胸膜外全肺切除术（EPP）是一种复杂的外科手术，涉及壁层和脏层胸膜、肺、心包和同侧膈肌的整块切除。主要为儿科患者的胸膜肉瘤小病例系列研究表明，EPP 可能是一种延长生命的手术选择。我们旨在描述在专门的肉瘤中心接受 EPP 的成人的特征和结果。
提取 2017 年 8 月至 2020 年 12 月期间因胸膜肉瘤接受 EPP 的患者的临床病理变量、手术细节和随访信息。主要结局是从 EPP 之日起的无事件生存（EFS）和总生存（OS）。次要结局是 EPP 前的无病间隔（DFI）以及早期和晚期术后并发症。
确定了 8 名患者，其中 7 名为软组织肉瘤，1 名为骨肉瘤。患者患有原发性胸部长肉瘤、肉瘤复发性胸部长肉瘤或初发性转移性疾病。所有患者均由经验丰富的心胸外科医生切除了胸膜肉瘤，一些患者接受了术前或术后治疗。围手术期发病率与先前发表的在间皮瘤患者中进行的 EPP 报告相当。在 22.5 个月的中位随访中，中位 EFS 为 6.0 个月，OS 为 20.7 个月。6 名患者（75%）出现疾病复发；5 名（62.5%）死于进行性疾病。2 名患者（25%）未复发：1 名死于放射性相关食管破裂，1 名在 37.0 个月时无疾病证据且存活。EFS 最长的患者特征包括低级别组织学和对术前化疗的代谢反应。
在患有胸膜肉瘤的成年人中，EPP 很少能治愈，但在专门中心进行时，它似乎是一种可行的挽救性手术。患者选择至关重要，强烈考虑采用多模式治疗来优化患者结局。在没有新辅助治疗反应得到证实的情况下，长期生存情况较差，不应推荐进行 EPP。
