Uncommon Primary Tracheal Tumors

Background Primary tracheal tumors other than adenoid cystic or squamous cell carcinoma are uncommon and have a heterogeneous histologic appearance. The experience regarding their treatment and long-term outcome is limited, and alternatives to segmental tracheal resection, including endoscopic treatment or radiation, continue to be explored. Methods A retrospective analysis was performed of uncommon tracheal tumors among 360 primary tracheal tumors seen over 40 years, excluding adenoid cystic and squamous cell carcinoma. Results Of 90 patients, 34 (38%) had benign tumors and 56 malignant: 11 carcinoid tumors, 14 mucoepidermoid carcinomas, 13 sarcomas, 15 nonsquamous bronchogenic carcinomas, 2 lymphomas, and 1 melanoma. Three patients had a second tracheal malignancy. Dyspnea was the most common symptom in benign tumors and hemoptysis in malignant tumors. Twelve patients did not undergo tracheal resection (13.3%) and 1 died before resection. Surgical therapy in 77 patients (85%) consisted of laryngectomy in 3, laryngotracheal resection in 9, tracheal resection in 46, and carinal resection in 19. Hospital mortality was 2.6% (2 of 77 patients) and major complications occurred in 16% (12 of 77 patients). Mean follow-up was 9.7 years. After resection, survival at 10 years was 94% for benign and 83% for carcinoid tumors, and at 5 years survival was 60% for bronchogenic carcinoma, 100% for mucoepidermoid tumors, and 78% for sarcomas. Patients with lymphomas and melanoma are alive more than 8 years after resection. Ten patients experienced recurrence (14%). Conclusions Surgical resection of uncommon primary tracheal tumors alleviates airway obstruction, is curative in patients with benign or slow-growing malignant lesions, and prolongs survival in highly malignant lesions.