Tracheal Tumors Treatment & Management

Updated: Sep 23, 2019
  • Author: Brian J Daley, MD, MBA, FACS, FCCP, CNSC; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
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Treatment

Approach Considerations

Surgery is usually indicated once diagnosis of a primary tracheal neoplasm is made. This is because these patients tend to progress rapidly once symptomatic because of the near-total tracheal luminal obstruction that is frequently present. Tracheal tumors are likely to be found during evaluation for more common pulmonary conditions (eg, pneumonia, cancer, and adult-onset asthma).

Because of the imminent threat to an adequate airway, urgent surgical intervention for primary tracheal tumors is not usually contraindicated, especially when patients are symptomatic. Bronchoscopic biopsy is contraindicated in the presence of highly vascular tumors (eg, hemangiomas).

Radiotherapy can be offered if the patient cannot tolerate surgical treatment. Chemotherapy can also be given after initial treatment with surgery, radiotherapy, or both. Laser removal of the intratracheal tumor is usually performed for palliation.

As newer modalities such as laser ablation and cryoablation undergo further development, treatment options may change. Furthermore, there is much promise for newer procedures in the field of tracheal reconstruction that could improve the quality of life for those affected by tracheal malignancy and other tracheal disorders. Treatment options that are being investigated include the following:

  • Composite autografts
  • Allografts
  • Chimeric autografts and allografts
  • Tissue-engineered grafts
  • Prosthetic scaffolds
  • Use of free-tissue vascularized carriers
  • Tracheal allotransplantation

Many of these techniques require further research for validation. [9, 10]

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Medical Therapy

In general, medical therapy has not been useful in the treatment of tracheal tumors. Successful treatment of squamous papillomatosis with interferon has been reported. Steroids once were used in tracheal hemangiomas, most of which now are treated by observation only because spontaneous regression is common.

Some case studies have reported that combination chemotherapy with concurrent carboplatin and paclitaxel, or concurrent nedaplatin and 5-fluorouracil together with radiation therapy, might be an effective treatment option for unresectable adenoid cystic carcinoma (ACC). [11, 12]

Reports of high-dose brachytherapy to treat primary tracheal tumors can be found in the literature [13] ; however, these cases generally involved tumors that either were recurrences or were unresectable because of the patient's condition. In isolated cases, local control was achieved with brachytherapy alone.

In a study of patients with tracheal tumors who received palliative treatment with endotracheal brachytherapy alone, Nguyen et al found that this approach yielded effective palliation and symptomatic improvement while giving rise to minimal toxicity. [14]  In a small series, Doggett et al reported good short-term results with the use of percutaneous computed tomography (CT)-fluoroscopy-guided radioisotope seed placement to manage ACC of the trachea. [15]

There is some evidence to suggest that immunotherapy (eg, nivolumab) may eventually prove useful in the treatment of patients for whom conventional therapies have failed. [16]

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Surgical Therapy

Surgical resection is the mode of treatment with the best hope for cure. In the series of 198 patients reported by Grillo and Mathisen, 70 (35%) had squamous cell carcinoma (SCC). [17] Of these, 44 (63%) were resected, with an operative mortality of 5%. The overall survival rate was 27% at 3 years and 13% at 5 years.

Laser resection as definitive treatment is appropriate for the following patients:

  • Patients with metastatic disease
  • Patients unable to tolerate primary resection
  • Patients with tumors that are too locally invasive to allow excision

In such patients, a laser procedure with stent placement may improve airway patency and allow for other definitive treatments.

The rapid expansion of technology has given rise to less invasive therapeutic options (eg, stenting, allografts). [18] Tracheobronchial stenting offers a minimally invasive palliative therapy for patients with unresectable malignant central airway obstruction that appears to be mostly beneficial in the short term. [19] This procedure provides symptomatic relief but seems to be less beneficial after 30 days as a result of tumor and tissue ingrowth. Indications for stenting include the following:

  • Endoluminal tumor
  • Malignant stricture
  • Extrinsic compression, except for extrinsic compression that is secondary to vascular compression, because this places patients at too high a risk for stent erosion and hemorrhage

In 2011, Seifalian et al, from the University College of London, produced the first completely synthetic trachea. Made of nanocomposite material, the synthetic trachea was transplanted into a patient whose own windpipe was damaged by cancer. [20] The operation was performed in Sweden at the Karolinska University Hospital in conjunction with the Karolinska Institute.

The synthetic windpipe’s wide and porous surface area allowed the stem cells taken from the patient's bone marrow and lining cells from the nose to be seeded with the patient's tissue. Within days, a synthetic windpipe, which essentially was the patient’s own, was created in a revolving bioreactor and then transplanted into the patient. This technique allows the patient to provide the stem cells to create the new trachea, avoiding a long waiting period to find a donor and eliminating the need for the antisuppressant drugs that other transplant patients must take.

Operative approach

Because of potential airway compromise, surgical intervention generally proceeds rapidly from the time of diagnosis.

Surgical treatment of proximal airway tumors presents some technical challenges specifically related to the maintenance of acceptable ventilation beyond the area of obstruction. Techniques have been developed for distal intubation during the resection of the tumor. Percutaneous transtracheal ventilation has been successfully used for laser endoscopic treatment of subglottic tumors. A study by Ding et al found that therapeutic bronchoscopy could significantly alleviate central airway obstruction, reduce shortness of breath, and improve quality of life for patients with tracheal neoplasms. [21]

Tumors of the upper third of the trachea can be approached transcervically via a standard collar incision. Tumors in the middle third of the trachea may require a partial or complete median sternotomy in addition to a cervical incision. Distal-third tumors are resected easily via a right thoracotomy to avoid the aortic arch.

Intraoperative bronchoscopy is used for accurate tumor localization. Lesions are resected with attempts to preserve as much trachea and lung tissue as possible. However, lobectomy may be necessary to ensure negative margins and node assessment. Using sleeve resections of the tracheal or bronchial tissue can preserve lung tissue.

Conventional wisdom has been that, at most, only 2 cm could be removed in order for the trachea to be dependably reconstructed in an end-to-end manner. Longer lesions are managed by means of lateral resection, with as wide a bridge of tracheal tissue as possible left to maintain the rigidity and patency of the airway.

Because the defects are usually too large to be closed by suturing, various materials are used as patches. Prosthetic materials usually fail because the bed of mesenchymal tissue in which the foreign body lies becomes, in effect, a chronic ulcer and responds characteristically because it is adjacent to a contaminated epithelial surface. Granulation tissue then proliferates in an attempt to heal the area, producing obstruction or stricture. Migration of the prosthesis may lead to erosion of major vessels.

Complex reconstructions that use the patient's own tissues generally have been successful only in the neck, where delayed healing can be accepted and multistaged procedures are possible. Reconstruction in the mediastinum requires that a fully-fashioned rigid tube with an epithelial lining be present at the conclusion of the initial operation.

Prosthetics such as nitinol mesh stents with overlapping cervical myocutaneous flaps to protect the neotrachea have been described. [22] The pectoralis major and latissimus dorsi myocutaneous flaps have also been successfully used in complex anterior mediastinal tracheostomy reconstruction techniques. [23]

Studies indicate that as much as half of the trachea can be removed and primary anastomosis achieved if extensive mobilization techniques are used. These techniques include the following:

  • Division of the inferior pulmonary ligament
  • Mobilization of the right mainstem bronchus from the pulmonary artery and vein and from the pericardium
  • Release of the larynx by separation of its thyrohyoid attachments

Grillo recommended using absorbable polyglactin for all tracheal anastomoses to minimize granuloma formation. [24] Usually, greater lengths of trachea may be removed in younger patients because of the greater elasticity of the trachea.

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Complications

Less-than-complete tracheal resection may lead to local recurrence. This may be acceptable if morbidity risks limit operative choice. In a retrospective study by Chen et al that involved 52 patients with primary ACC of the trachea, the investigators suggested that postoperative radiotherapy should be recommended for patients with incomplete resections. [25]

Experience is growing with the use of stents to temporize before definitive resection or to treat patients who are not surgical candidates. [26, 27] Complications from bronchial or tracheal stenting include the following:

  • Rupture
  • Granulation tissue
  • Residual scarring
  • Stenosis and bleeding

If diagnosis is delayed, tracheoesophageal fistula and tracheoinnominate fistula can occur.

Complications of tracheal surgery include the following:

  • Restenosis
  • Anastomotic and sternal dehiscence
  • Anterior spinal cord ischemia
  • Acquired respiratory distress syndrome
  • Fistula formation

Reported rates are low and increases with increasingly higher levels of resection. [28, 29] In one series of primary resection and anastomosis, the ability to definitively remove the airway after surgery approached 100%. [30]

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Long-Term Monitoring

For patients with benign lesions, serial follow-up examination is recommended, especially if tracheal resection is not performed.

For those with malignant lesions, follow-up examination similar to that for lung cancer is appropriate, with serial computed tomography (CT) scans over the following 5 years. Preoperative radiation therapy is administered given for ACC and adjuvant radiation for mucoepidermoid carcinoma. Combined-modality therapy may be considered for carcinoid or other neuroendocrine tumors exhibiting more aggressive characteristics than the typical carcinoid lesions. Because of the infrequency of these tumors, most data are retrospective, and series of outcomes are small.

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