恶性气管狭窄：当前诊断与治疗方法的叙述性综述

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中文摘要

背景： 恶性气管狭窄（MTS）是一种罕见且危及生命的疾病，由原发性气管肿瘤引起，主要为腺样囊性癌（ACC）和鳞状细胞癌（SCC），更罕见地由其他原发性肿瘤如淋巴瘤引起，或由邻近恶性肿瘤（甲状腺、食管、肺或纵隔癌）的局部延伸/转移所致。MTS占呼吸系统肿瘤的<0.1%，表现为进行性呼吸衰竭，需要胸外科、介入肺脏病学、肿瘤学和重症监护等多学科紧急救治。诊断依赖于临床评估、先进影像学和内镜检查。支气管镜（BC）仍是直接观察和活检或实施治疗操作的最佳检查方法。预后取决于组织学、局部范围以及能否实现R0切除。气管切除加一期吻合仍是可切除病例的治愈性标准。介入操作（减瘤、激光、冷冻治疗、支架置入）对于姑息治疗和急性稳定至关重要。方法：使用MeSH/Emtree术语和自由文本关键词，对PubMed/MEDLINE、Embase、Scopus和Web of Science（1965-2025）进行了结构化（非系统性）检索。纳入评估成人恶性中央气道狭窄、原发性气管肿瘤或继发性侵犯并接受手术、支气管镜或多模式干预（包括ECMO支持）的研究。结果：恶性气管狭窄（MTS）——主要为腺样囊性癌和鳞状细胞癌——尽管发病率低，但造成严重的临床负担。切缘阴性的完全手术切除仍是可切除疾病的治愈性治疗。在不可切除病例中，多模式姑息干预（支架置入、激光/电灼消融和机械减瘤）可快速恢复气道并改善生活质量，而ECMO在经验丰富的中心进行的特定危重气道操作中是既定的抢救或辅助支持策略。三维生物打印和组织工程重建仍处于实验或早期临床阶段，生物可吸收支架和基于人工智能（AI）的工具仍处于研究阶段；目前均不属于MTS常规管理的一部分。结论：对适当选择的可切除疾病进行R0切除，以及对不可切除的症状性疾病进行介入气道管理，是支持证据最强的策略，尽管证据主要来自专科中心的回顾性系列。新兴技术需要前瞻性验证才能临床采用。管理应个体化、多学科，并集中在有经验的气道中心进行。

英文摘要

Background: Malignant tracheal stenosis (MTS) is a rare, life-threatening condition caused by primary tracheal tumor, mainly adenoid cystic carcinoma (ACC) and squamous cell carcinoma (SCC), and more rarely other primary neoplasms such as lymphomas, or local extension/metastasis from adjacent malignancies (thyroid, esophageal, lung, or mediastinal cancers). Accounting for <0.1% of respiratory tumors, MTS presents with progressive respiratory failure, requiring urgent multidisciplinary care involving thoracic surgery, interventional pulmonology, oncology, and intensive care. Diagnosis relies on clinical evaluation, advanced imaging and endoscopy. Bronchoscopy (BC) remains the best investigation for direct visualization and biopsy or performing therapeutic maneuvers. Prognosis depends on histology, local extent, and achieving R0 resection. Tracheal resection with primary anastomosis remains the curative standard for resectable cases. Interventional procedures (debulking, laser, cryotherapy, stenting) are essential for palliative management and acute stabilization. Methods: A structured (non-systematic) search of PubMed/MEDLINE, Embase, Scopus, and Web of Science (1965-2025) was performed using MeSH/Emtree terms and free-text keywords. Studies were included if they evaluated adult patients with malignant central airway stenosis, primary tracheal tumors, or secondary invasion undergoing surgical, bronchoscopic, or multimodal interventions, including ECMO support. Results: Malignant tracheal stenosis (MTS)-predominantly adenoid cystic and squamous cell carcinomas-imposes a critical clinical burden despite its low incidence. Complete surgical resection with negative margins remains the curative treatment for resectable disease. In unresectable cases, multimodal palliative interventions (stenting, laser/electrocautery ablation, and mechanical debulking) provide rapid airway restoration and quality-of-life improvements, whereas ECMO is an established rescue or adjunctive support strategy in selected critical airway procedures performed in experienced centers. Three-dimensional bioprinting and tissue-engineered reconstruction remain experimental or early clinical, and bioresorbable stents and artificial intelligence (AI)-based tools remain investigational; none is currently part of routine MTS management. Conclusions: R0 resection for appropriately selected resectable disease and interventional airway management for unresectable symptomatic disease are the best-supported strategies, although the evidence derives mainly from retrospective series from specialized centers. Emerging technologies require prospective validation before clinical adoption. Management should be individualized, multidisciplinary and centralized in experienced airway centers.