Diagnosis and multimodality treatment of primary malignant tracheal tumors.

Primary malignant tracheal tumors (PMTTs) are rare, representing less than 0.5% of all cancers. The predominant histological variants are adenoid cystic carcinoma and squamous cell carcinoma. Because of the trachea's functional reserve, symptoms often appear only after significant airway obstruction, delaying diagnosis. Surgical resection with primary reconstruction is the preferred treatment when feasible. Resections longer than 40 mm are associated with higher anastomotic risk, and the decision between complete (R0) or microscopically incomplete (R1) resections must consider patient-specific anatomy and the potential for adjuvant radiotherapy. In selected cases, R1 resection followed by radiation achieves outcomes comparable to R0 resection. Endoscopic interventions can restore airway patency and are indicated in patients who are unresectable, inoperable, or require bridging to definitive treatment. However, they are palliative and not curative. Imaging and bronchoscopic evaluation are essential for assessing resectability and planning the surgical approach. Management of PMTT requires a multidisciplinary strategy that integrates surgical technique, endoscopic interventions, and adjuvant therapies. Prognosis is influenced by tumor histology, resection margins, and patient-related factors. Given the rarity of PMTT, current evidence is limited, and most clinical decisions rely on retrospective data and surgical experience. This review aims to synthesize existing knowledge and highlight ongoing gaps in the literature to support more informed clinical practice and future research directions.