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Malignant tracheal tumors

James R Jett, MD
Section Editors
Bruce E Brockstein, MD
Steven E Schild, MD
Eric Vallières, MD, FRCSC
Deputy Editor
Sadhna R Vora, MD


The epidemiology, pathology, clinical presentation, treatment, and prognosis of malignant primary tracheal tumors will be reviewed here.

Depending upon histology and location, tracheal tumors may overlap with primary lung cancers or head and neck lesions; these are discussed separately.

(See "Overview of the risk factors, pathology, and clinical manifestations of lung cancer".)

(See "Overview of treatment for head and neck cancer".)


Primary tracheal tumors are rare. Population-based studies from tumor registries in Finland, Denmark, Netherlands, and England report annual incidence rates of tracheal cancer of approximately 0.1 per 100,000 per year, with tracheal cancers accounting for less than one-half of 1 percent of all malignant tumors [1-4]. The largest reported series comes from a Surveillance, Epidemiology, and End Results (SEER) analysis of 578 cases of primary tracheal carcinoma identified over a 31-year period [5]. In that series, the median age was 63 years, and 56 percent of patients were male. In other series, the mean age at presentation was approximately 60 to 65 years, and 60 to 70 percent of cases occurred in males [1-4].


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Literature review current through: Sep 2016. | This topic last updated: Aug 25, 2016.
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  1. Nouraei SM, Middleton SE, Nouraei SA, et al. Management and prognosis of primary tracheal cancer: a national analysis. Laryngoscope 2014; 124:145.
  2. Manninen MP, Antila PJ, Pukander JS, Karma PH. Occurrence of tracheal carcinoma in Finland. Acta Otolaryngol 1991; 111:1162.
  3. Licht PB, Friis S, Pettersson G. Tracheal cancer in Denmark: a nationwide study. Eur J Cardiothorac Surg 2001; 19:339.
  4. Honings J, van Dijck JA, Verhagen AF, et al. Incidence and treatment of tracheal cancer: a nationwide study in the Netherlands. Ann Surg Oncol 2007; 14:968.
  5. Urdaneta AI, Yu JB, Wilson LD. Population based cancer registry analysis of primary tracheal carcinoma. Am J Clin Oncol 2011; 34:32.
  6. Desai DP, Holinger LD, Gonzalez-Crussi F. Tracheal neoplasms in children. Ann Otol Rhinol Laryngol 1998; 107:790.
  7. Macchiarini P. Primary tracheal tumours. Lancet Oncol 2006; 7:83.
  8. Ahn Y, Chang H, Lim YS, et al. Primary tracheal tumors: review of 37 cases. J Thorac Oncol 2009; 4:635.
  9. Gaissert HA, Grillo HC, Shadmehr MB, et al. Uncommon primary tracheal tumors. Ann Thorac Surg 2006; 82:268.
  10. Maziak DE, Todd TR, Keshavjee SH, et al. Adenoid cystic carcinoma of the airway: thirty-two-year experience. J Thorac Cardiovasc Surg 1996; 112:1522.
  11. Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC. of the Lung, Pleura, Thymoma, and Heart. In: World Health Organization Classification of Tumors: Pathology and Genetics, IARC Presee, Lyon, France 2004.
  12. Regnard JF, Fourquier P, Levasseur P. Results and prognostic factors in resections of primary tracheal tumors: a multicenter retrospective study. The French Society of Cardiovascular Surgery. J Thorac Cardiovasc Surg 1996; 111:808.
  13. Dean CW, Speckman JM, Russo JJ. AIRP best cases in radiologic-pathologic correlation: adenoid cystic carcinoma of the trachea. Radiographics 2011; 31:1443.
  14. Heitmiller RF, Mathisen DJ, Ferry JA, et al. Mucoepidermoid lung tumors. Ann Thorac Surg 1989; 47:394.
  15. Molina JR, Aubry MC, Lewis JE, et al. Primary salivary gland-type lung cancer: spectrum of clinical presentation, histopathologic and prognostic factors. Cancer 2007; 110:2253.
  16. Briselli M, Mark GJ, Grillo HC. Tracheal carcinoids. Cancer 1978; 42:2870.
  17. Marom EM, Goodman PC, McAdams HP. Focal abnormalities of the trachea and main bronchi. AJR Am J Roentgenol 2001; 176:707.
  18. Brand-Saberi BE, Schäfer T. Trachea: anatomy and physiology. Thorac Surg Clin 2014; 24:1.
  19. Sherani K, Vakil A, Dodhia C, Fein A. Malignant tracheal tumors: a review of current diagnostic and management strategies. Curr Opin Pulm Med 2015; 21:322.
  20. Wu CC, Shepard JA. Tracheal and airway neoplasms. Semin Roentgenol 2013; 48:354.
  21. Park CM, Goo JM, Lee HJ, et al. Tumors in the tracheobronchial tree: CT and FDG PET features. Radiographics 2009; 29:55.
  22. Bhattacharyya N. Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis. Otolaryngol Head Neck Surg 2004; 131:639.
  23. Luo M, Duan C, Qiu J, et al. Diagnostic Value of Multidetector CT and Its Multiplanar Reformation, Volume Rendering and Virtual Bronchoscopy Postprocessing Techniques for Primary Trachea and Main Bronchus Tumors. PLoS One 2015; 10:e0137329.
  24. Cavaliere S, Venuta F, Foccoli P, et al. Endoscopic treatment of malignant airway obstructions in 2,008 patients. Chest 1996; 110:1536.
  25. Colt HG, Harrell JH. Therapeutic rigid bronchoscopy allows level of care changes in patients with acute respiratory failure from central airways obstruction. Chest 1997; 112:202.
  26. Jeon K, Kim H, Yu CM, et al. Rigid bronchoscopic intervention in patients with respiratory failure caused by malignant central airway obstruction. J Thorac Oncol 2006; 1:319.
  27. Honings J, Gaissert HA, Verhagen AF, et al. Undertreatment of tracheal carcinoma: multidisciplinary audit of epidemiologic data. Ann Surg Oncol 2009; 16:246.
  28. Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann Thorac Surg 1990; 49:69.
  29. Mendenhall WM, Morris CG, Amdur RJ, et al. Radiotherapy alone or combined with surgery for adenoid cystic carcinoma of the head and neck. Head Neck 2004; 26:154.
  30. Gaissert HA, Grillo HC, Shadmehr MB, et al. Long-term survival after resection of primary adenoid cystic and squamous cell carcinoma of the trachea and carina. Ann Thorac Surg 2004; 78:1889.
  31. Honings J, Gaissert HA, Weinberg AC, et al. Prognostic value of pathologic characteristics and resection margins in tracheal adenoid cystic carcinoma. Eur J Cardiothorac Surg 2010; 37:1438.
  32. Xie L, Fan M, Sheets NC, et al. The use of radiation therapy appears to improve outcome in patients with malignant primary tracheal tumors: a SEER-based analysis. Int J Radiat Oncol Biol Phys 2012; 84:464.
  33. Cooper JS, Pajak TF, Forastiere AA, et al. Postoperative concurrent radiotherapy and chemotherapy for high-risk squamous-cell carcinoma of the head and neck. N Engl J Med 2004; 350:1937.
  34. Bernier J, Domenge C, Ozsahin M, et al. Postoperative irradiation with or without concomitant chemotherapy for locally advanced head and neck cancer. N Engl J Med 2004; 350:1945.
  35. Misiukiewicz KJ, Camille N, Tishler R, et al. Organ preservation for adenoid cystic carcinoma of the larynx. Oncologist 2013; 18:579.
  36. Laurie SA, Ho AL, Fury MG, et al. Systemic therapy in the management of metastatic or locally recurrent adenoid cystic carcinoma of the salivary glands: a systematic review. Lancet Oncol 2011; 12:815.
  37. Lloyd S, Yu JB, Wilson LD, Decker RH. Determinants and patterns of survival in adenoid cystic carcinoma of the head and neck, including an analysis of adjuvant radiation therapy. Am J Clin Oncol 2011; 34:76.
  38. Webb BD, Walsh GL, Roberts DB, Sturgis EM. Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 2006; 202:237.
  39. Zhu F, Liu Z, Hou Y, et al. Primary salivary gland-type lung cancer: clinicopathological analysis of 88 cases from China. J Thorac Oncol 2013; 8:1578.