Tracheal Neoplasms

Author Credentials 

Tiffany A. Glazer, M.D.
Assistant Professor
Otolaryngology-Head & Neck Surgery
University of Wisconsin 
600 Highland Avenue
K4/723
Madison, WI 53792

 

Module Summary

Most tracheal neoplasms in adults are malignant, with the vast majority of those tumors showing squamous cell or adenoid cystic pathology. Most adults present with subtle clinical symptoms including worsening dyspnea and wheezing. CT chest is helpful in characterizing a tracheal neoplasm, but the mainstay of diagnosis is bronchoscopy. Tracheal neoplasms are best managed with surgery, either bronchoscopic resection (for certain benign tumors only) or tracheal resection and anastomosis. Complications of tracheal resection and reconstruction can include anastomotic ischemia which can be avoided by limiting circumferential dissection, preserving tracheal blood supply, and limiting mediastinal nodal dissection. Adjuvant radiation therapy is recommended after resection of malignant tracheal tumors. Primary radiation therapy is reserved for palliative treatment.

Module Learning Objectives 
  1. Be able to describe the anatomy of the trachea and understand how this correlates to different etiologies of tracheal neoplasms.
  2. Discuss the most common tracheal neoplasms in an adult. 
  3. Explain how to evaluate a patient with a suspected tracheal neoplasm in the clinic, and what studies you would order to augment your examination. 
  4. Describe how to treat benign and malignant tracheal neoplasms in an adult.
  5. Summarize the complications of tracheal resection.  

 

Embryology

Learning Objectives 
  1. Recognize that the respiratory primordium begins to form at the fourth week of gestation with the formation of the laryngotracheal groove, which evaginates to form a pouch-like laryngotracheal diverticulum.
  • Gradual elongation of this diverticulum forms the primitive laryngotracheal tube.
  • The distal end of the tube eventually develops into the lung buds, and the proximal end forms the primitive laryngeal aditus.

 

References 
  1. Moore KL, Persaud TVN: Before We Are Born. Essentials of Embryology and Birth Defects. Philadelphia: WB Saunders, 1998.

Anatomy

Learning Objectives 
  1. Review the dimensions of the trachea in the average adult.
  • Length 10-13 cm; AP & lateral dimensions roughly 2 x 2 cm.
  1. Describe the differences between the posterior and anterior tracheal walls.
  • The posterior tracheal wall or membranous trachea comprises fibroelastic tissue between the ends of the tracheal rings and abuts the anterior-lateral portion of the esophagus. 
  • The anterior tracheal wall is made of 18-22 cartilaginous rings.
  • Know that the membranous trachea is more commonly affected than the cartilaginous trachea. 
  1. Explain that there are multiple layers of the tracheal wall, and tracheal neoplasms can arise from any layer. 
  • As such, tracheal neoplasms are classified histologically as either epithelial or mesenchymal tumors.
  1. Recognize that the proximal and distal thirds of the trachea are most frequently affected. 
  2. Discuss nearby structures that can serve as a source of secondary tracheal neoplasms including the lungs, esophagus, thyroid, and mediastinum.

 

References 
  1. Semaan R, Feller-Kopman D. Tracheostomy, Part One. In: Cameron J, Cameron A, editors. Current Surgical Therapy. 12th ed. Philadelphia (PA): Elsevier, 2017. p. 1390-95. 
  2. Brown LM, Patterson GA. Diagnosis and Management of Tracheal Neoplasms. In: Flint et al, editors. Cummings Otolaryngology. 6th ed. Philadelphia (PA): Elsevier, 2015. p. 1751-66.

 

Pathogenesis

Learning Objectives 
  1. Recognize that tracheal neoplasms can be categorized as primary or secondary.
  • Primary tumors arise within the trachea itself; secondary tumors arise from local invasion or hematogenous metastases. 
  1. Indicate that primary tumors can be divided into benign or malignant, and secondary tumors by definition are always malignant. 
  • Remember from above that tracheal neoplasms can also be divided into epithelial or mesenchymal. 
  • Recognize that in adults, 90% of primary tracheal tumors are malignant.
  1. Be able to list tracheal tumor types depending on their classification: 
  • Primary tracheal tumors
    • Epithelial
      • Benign (3)
      • Malignant (6)
    • Mesenchymal
      • Benign (12)
      • Malignant (8)
  • Secondary tracheal tumors
    • Epithelial 
      • Malignant (local invasion or metastasis) 

 

References 
  1. McCarthy MJ, Rosado-de-Christenson ML. Tumors of the trachea.  J Thorac Imag  1995;10:180.
  2. Pearson FG, Todd TR, and Cooper JD: Experience with primary neoplasms of the trachea and carina. J Thorac Cardiovasc Surg 1984; 88: pp. 511-518.
  3. Grillo HC, and Mathisen DJ: Primary tracheal tumors: treatment and results. Ann Thorac Surg 1990; 49: pp. 69-77.
  4. Perelman MI, Koroleva N, Birjukov J, et al: Primary tracheal tumors. Semin Thorac Cardiovasc Surg 1996; 8: pp. 400-402.
  5. Regnard JF, Fourquier P, and 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: pp. 808-813.
  6. Refaely Y, and Weissberg D: Surgical management of tracheal tumors. Ann Thorac Surg 1997; 64: pp. 1429-1432.
  7. Webb BD, Walsh GL, Roberts DB, et al: Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 2006; 202: pp. 237-246.
  8. Ahn Y, Chang H, and Lim YS: Primary tracheal tumors: review of 37 cases. J Thorac Oncol 2009; 4: pp. 635-638.
  9. Shadmehr MB, Farzanegan R, and Graili P: Primary major airway tumors; management and results. Eur J Cardiothoracic Surg 2011; 39: pp. 749-754.

 

Incidence

Learning Objectives 
  1. Recognize that tracheal neoplasms are rare, and the annual incidence is 2 cases per 1 million patients. 
  • Because of their rarity, most tracheal neoplasms are studied as small case series from large tertiary referral institutions.
  1. Cite that the annual incidence of primary tracheal cancer is 0.1 cases per 100,000 patients.
  • Tracheal malignancies account for 0.2% of all cancers of the respiratory tract.
  • Laryngeal and bronchial cancer occur 40 and 400 times more frequently (respectively).
  1. State the disease-specific and all-cause mortality for primary malignant tracheal tumors. 

 

References 
  1. Macchiarini P: Primary tracheal tumours. Lancet Oncol 2006; 7: pp. 83-91.
  2. Meyers BF, and Mathisen DJ: Management of Tracheal Neoplasms. Oncologist 1997; 2: pp. 245-253.
  3. Webb BD, Walsh GL, Roberts DB, et al: Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 2006; 202: pp. 237-246.

 

Patient Evaluation

Learning Objectives 
  1. Remember that because the incidence of these tumors is low, so is the index of suspicion. The diagnosis and definitive treatment are often delayed.
  • At the time of diagnosis, about 1/3 of patients have locally advanced disease. Only 24.2% of patients come to medical attention with carcinoma localized to the trachea, and 18.7% have distant disease.
  1. Indicate that tracheal neoplasms do not produce symptoms until the lumen of the airway is narrowed by approximately 75%.
  • Dyspnea on exertion develops if the tracheal lumen is about 8 mm, and at rest if the lumen is about 5 mm. 
  1. Recognize the most common symptoms patients with a tracheal neoplasm present with adult-onset “asthma,” cough, and dyspnea: 
  • Patients with tracheal neoplasms are treated for asthma or COPD for years until they no longer respond. 
  • Explain that at this point, the patient would need a CT chest +/- bronchoscopy in order to diagnose a tracheal neoplasm. 
  1. Acknowledge that dysphagia and hoarseness are poor prognostic signs and understand why.
  2. Recognize that physical exam may initially be underwhelming. 
  • Patients may present with subtle stridor, wheezing, bronchial breath sounds, increased accessory muscle use, or alteration in the tone of voice.
  • Be able to further characterize the patient’s stridor. 
    • Tracheal neoplasms often produce inspiratory stridor that worsens when lying down.  
  1. Remember that past medical history, particularly focusing on previous cancers, is important to consider as some tumors may cause direct tracheal invasion.

 

References 
  1. Honings J, Gaissert HA, van der Heijden HF, et al: Clinical aspects and treatment of primary tracheal malignancies. Acta Otolaryngol 2010; 130: pp. 763-772.
  2. Bhattacharyya N: Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis. Otolaryngol Head Neck Surg 2004; 131: pp. 639-642.
  3. Yang KY, Chen YM, Huang MH, et al: Revisit of primary malignant neoplasms of the trachea: clinical characteristics and survival analysis. Jpn J Clin Oncol 1997; 27: pp. 305-309.
  4. Webb BD, Walsh GL, Roberts DB, et al: Primary tracheal malignant neoplasms: the University of Texas MD Anderson Cancer Center experience. J Am Coll Surg 2006; 202: pp. 237-246.

 

Measurement of Functional Status

Learning Objectives 
  1. Remember that pulmonary function tests (PFTs) can help lead to a diagnosis of tracheal neoplasm. 
  • PFT results depend upon the location of the tumor (extra- or intrathoracic, type of obstruction (fixed or variable), and extent of the tumor.
  • Be able to draw or recognize a PFT showing a tracheal (intrathoracic) tumor.
    • Impairment of the mean inspiratory flow is minimal and peak expiratory flow is decreased.
  1. Acknowledge that a normal PFT does not rule out a tracheal neoplasm. 

 

References 
  1. Honings J, Gaissert HA, van der Heijden HF, et al: Clinical aspects and treatment of primary tracheal malignancies. Acta Otolaryngol 2010; 130: pp. 763-772.
  2. Mussi RK, Toro IF, and Pereira MC: Mucoepidermoid carcinoma of the trachea mimicking asthma. J Bras Pneumol 2009; 35: pp. 280-284.

 

Imaging

Learning Objectives 
  1. Remember from above that diagnosis of a tracheal tumor requires radiography +/- bronchoscopy. 
  • Understand that a PA and lateral chest radiograph will identify an obstructive tracheal neoplasm in ¼ - ½ of the cases. 
    • Be able to describe the findings on an Xray that are suggestive of a tracheal tumor.
  • Understand the benefits of a CT chest scan.
    • CT chest scans are critical for diagnosing the degree of luminal compromise, mediastinal tumor extension, nodal, or metastatic disease.
    • Identify different CT chest protocols that may help further characterize the neoplasm (i.e., inspiratory/expiratory scans, spiral CT with 3D recon).
  1. Be able to recognize radiographic features that may help differentiate between benign and malignant tracheal neoplasms. 
  • Benign: intraluminal, spreads along tracheal wall, well-circumscribed, smooth, lobulated, size <2cm.
  • Malignant: extramural extension, irregular, extension over variable lengths of trachea, size >2cm, circumferential tracheal involvement, enlarged mediastinal nodes.
  1. Recognize the limits of CT chest scans.
  • Unreliable at determining submucosal spread of tumor. Identify a method to determine submucosal spread (particularly dangerous for adenoid cystic carcinoma). 
  1. Explain that bronchoscopy is the mainstay of diagnosis for tracheal neoplasms and understand what information this would give that a CT chest scan wouldn’t.
  • Bronchoscopy allows for biopsy of the tumor, precise measurement of tumor length, identification of tumor extension, determination of the proximity of the tumor to the larynx or carina, and mucosal extension of the tumor. 
References 
  1. McCarthy MJ, and Rosado-de-Christenson ML: Tumors of the trachea. J Thorac Imaging 1995; 10: pp. 180-198.
  2. Maehara M, Ikeda K, Ohmura N, et al: Leiomyoma of the trachea: CT and MRI findings. Radiat Med 2006; 24: pp. 643-645.
  3. Weber AL, and Grillo H: Tracheal lesions—assessment by conventional films, computed tomography and magnetic resonance imaging. Isr J Med Sci 1992; 28: pp. 233-240.

 

Pathology

Learning Objectives 
  1. Be able to list tracheal tumor types depending on their classification: 
  • Primary tracheal tumors
    • Epithelial
      • Benign 
        • Squamous cell papilloma 
        • Papillomatosis 
        • Pleomorphic adenoma
      • Malignant
        • Squamous cell carcinoma
        • Adenoid cystic carcinoma
        • Carcinoid
        • Mucoepidermoid carcinoma
        • Adenocarcinoma
        • Small-cell undifferentiated carcinoma
    • Mesenchymal
      • Benign
        • Fibroma
        • Hemangioma
        • Granular cell tumor
        • Schwannoma
        • Neurofibroma
        • Fibrous histiocytoma
        • Pseduosarcoma
        • Hemangioendothelioma
        • Leiomyoma
        • Chondroma
        • Chrondroblastoma
        • Lipoma
      • Malignant
        • Leiomyosarcoma
        • Chrondrosarcoma
        • Paraganglioma
        • Spindle-cell sarcoma
        • Lymphoma
        • Malignant fibrous histiocytoma
        • Rhabdomyosarcoma
        • Melanoma
  • Secondary tracheal tumors
    • Epithelial 
      • Malignant
        • Invasion by adjacent malignancy
        • Metastasis  
  1. Recognize the HPV strains most commonly associated with tracheal papillomatosis. 
  2. Remember that 90% of primary tracheal neoplasms are malignant. 
  • Approximately 75% of all malignant primary tracheal neoplasms are squamous cell carcinoma (SCC) or adenoid cystic carcinoma (ACC). Be able to recognize characteristics of these tumors in a patient:
    • SCC of the trachea tends to be aggressive, grows rapidly, and metastasizes early.
    • ACC tends to push structures rather than invade them and have a predilection for perineural, submucosal, and distant metastatic spread. 

 

References 
  1. McCarthy MJ, Rosado-de-Christenson ML. Tumors of the trachea.  J Thorac Imag  1995;10:180.
  2. Harris K, and Chalhoub M: Tracheal papillomatosis: what do we know so far? Chron Respir Dis 2011; 8: pp. 233-235
  3. Brown LM, Patterson GA. Diagnosis and Management of Tracheal Neoplasms. In: Flint et al, editors. Cummings Otolaryngology. 6th ed. Philadelphia (PA): Elsevier, 2015. p. 1751-66.

 

Treatment

Learning Objectives 
  1. Describe how to surgically treat benign tracheal neoplasms.
  • Segmental tracheal resection and primary anastomosis.
  • If patient has pedunculated or exophytic tumor, can remove via bronchoscopic resection with rigid bronchoscope and biopsy forceps, cautery excision, cryoablation, or laser photocoagulation.
  1. Explain that tracheal resection with primary anastomosis is the therapy of choice for malignant adult tracheal tumors.
  • Name the limitations of surgical resection (involvement of nearby structures, length of trachea, mediastinal nodal involvement).
    • Know how much tracheal length can be resected and anastomosed without undue tension.
    • Note that positive mediastinal nodes in ACC does not preclude resection as patients with metastatic ACC remain asymptomatic for years.
  1. Communicate the anesthetic and airway management of a patient with a tracheal neoplasm who requires surgical resection. 
  • Describe how to intubate the patient.
  • Describe how to ventilate the lungs during tracheal division and reconstruction. 
  1. Describe mobilization and release techniques to provide additional length if the tracheal resection will place the anastomosis under tension. 
  • Know in which situations suprahyoid release will not provide much help.
  1. Calculate when to apply radiation therapy.
  • Adjuvant: 60 Gy recommended for almost all patients with malignant tracheal tumors due to high local recurrence rates.
  • Palliative: for unresectable tumors with severe symptoms.
  1. Report when palliative, therapeutic bronchoscopy is indicated. 

 

References 
  1. Pearson FG, Todd TR, and Cooper JD: Experience with primary neoplasms of the trachea and carina. J Thorac Cardiovasc Surg 1984; 88: pp. 511-518.
  2. Grillo HC, Dignan EF, and Miura T: Extensive Resection and Reconstruction of Mediastinal Trachea Without Prosthesis or Graft: An Anatomical Study in Man. J Thorac Cardiovasc Surg 1964; 48: pp. 741-749.
  3. Montgomery WW: Suprahyoid release for tracheal anastomosis. Arch Otolaryngol 1974; 99: pp. 255-260.
  4. Stephens KE, and Wood DE: Bronchoscopic management of central airway obstruction. J Thorac Cardiovasc Surg 2000; 119: pp. 289-296.

 

Medical Therapies

Learning Objectives 
  1. Name medications that can help treat a patient in respiratory distress secondary to tracheal stenosis.
  • Nebulized bronchodilators, racemic epinephrine, and heliox. 

 

References 
  1. Hackeling T, Triana R, Ma OJ, et al: Emergency care of patients with tracheostomies: a 7-year review. Am J Emerg Med 1998; 16: pp. 681.

Staging

Learning Objectives 
  1. Acknowledge that an AJCC staging system does not exist for tracheal tumors. 
  2. However, a tumor/node/metastasis system has been proposed on the basis of a SEER database review of 92 cases of primary tracheal tumors. Be able to describe this staging system. 
  • T stage
    • T1: primary tumor confined to trachea; size <2cm
    • T2: primary tumor confined to trachea; size >2cm
    • T3: spread outside the trachea but not to adjacent structures
    • T4: Spread to adjacent structures
  • N stage
    • N0: no evidence of regional nodal metastasis
    • N1: positive regional nodal disease
    • Nx: unknown or cannot be assessed
  • M stage
    • M0: no evidence of distant metastasis
    • M1: distant metastasis
    • Mx: unknown or cannot be assessed

 

References 
  1. Bhattacharyya N: Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis. Otolaryngol Head Neck Surg 2004; 131: pp. 639-642.

Case Studies

  1. A 28-year-old man with long standing history of laryngeal recurrent respiratory papillomatosis comes to your clinic with worsening wheezing despite maximal medical management by his pulmonologist. His pulmonologist performed pulmonary function tests last week which showed normal inspiratory volumes, but a plateau pattern on forced expiration. Your in-office flexible laryngoscopic exam shows good control of his laryngeal RRP. What are your next steps in diagnosis for this patient? Assuming this is distal extension of his RRP, what are your treatment options?
  2. A 72-year-old male, lifelong smoker, presents with history of COPD which has acutely worsened over the past month. His pulmonologist ordered a CXR which shows focal tracheal narrowing. Flexible laryngoscopic exam is normal. Are there any other images you want to obtain? Would you take this patient to the OR? If the tracheal lumen is 8mm, how will you intubate? If there is an isolated tracheal tumor, what is the most likely diagnosis and how will you treat it?

Complications

Learning Objectives 
  1. Name four factors placing adult patients at significant risk of complications following tracheal resection and reconstruction. 
  • (1) An increased length of resection, (2) the need for laryngeal release, (3) laryngotracheal or carinal resection as opposed to standard tracheal resection, and (4) squamous cell histology.
  1. Remember that the most common problems after tracheal surgery include atelectasis, retained secretions, pneumonia, edema, and swallowing dysfunction with aspiration.
  2. Name the two reasons anastomotic dehiscence usually occurs.
  • Excessive tension & devascularization of the trachea, which can occur with circumferential dissection and/or mediastinal dissection. 
  1. Acknowledge a lethal complication of tracheal resection.
  • Tracheal-innominate or tracheal-pulmonary artery fistula formation.
  1. Explain a long-term complication of tracheal resection.
  • Granulation formation at the anastomotic site. 

 

References 
  1. Regnard JF, Fourquier P, and 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: pp. 808-813.
  2. Lanuti M, and Mathisen DJ: Management of complications of tracheal surgery. Chest Surg Clin N Am 2003; 13: pp. 385-397.

 

Review

Review Questions 
  1. What are the most common benign and malignant tracheal neoplasms in adults?
  2. How do adults with tracheal neoplasms typically present?
  3. How do you diagnose a patient with a suspected tracheal neoplasm?
  4. How would you manage the airway in a patient with a tracheal neoplasm who needs to go to the operating room?
  5. What techniques can you employ for bronchoscopic resection of benign tracheal neoplasms?
  6. What are the indications for tracheal resection and anastomosis?
  7. What are the complications of tracheal resection and anastomosis?
  8. Which patients with malignant tracheal tumors should receive adjuvant radiation therapy? Palliative radiation therapy?