Elsevier

The Lancet Oncology

Volume 7, Issue 1, January 2006, Pages 83-91
The Lancet Oncology

Review
Primary tracheal tumours

https://doi.org/10.1016/S1470-2045(05)70541-6Get rights and content

Summary

Primary tumours of the trachea can be benign or malignant and account for fewer than 0·1% of tumours. However, they are a diagnostic and therapeutic challenge. Benign tumours are usually misdiagnosed as asthma or chronic lung disease, and can delay diagnosis for months or years. Because of their rapid growth and onset of haemoptysis, malignant tumours are often diagnosed earlier than benign tumours and patients thus often present with locally advanced disease. Inappropriate treatment is an equally frustrating issue. Modern techniques for tracheal surgery—laryngotracheal, tracheal, or carinal resection—combined with radiotherapy, can be offered curatively with low perioperative risks. Nevertheless, the low numbers of patients undergoing resection and the associated poor survival in epidemiological studies over the past two decades have shown that surgery is rarely considered outside referral centres, with radiotherapy or another form of local treatment (eg, endotracheal stents, debridement, brachytherapy) generally preferred. The liberal use of these other techniques should be avoided because surgery has the potential to cure all patients with benign and low-grade tumours and most patients with malignant primary tracheal tumours, and other techniques are usually palliative at best.

Introduction

Primary tumours of the trachea (figure 1) are rare and are usually malignant in adults and benign in children. Although patients present with signs and symptoms of central-airway obstruction, the definitive diagnosis of these tumours is commonly delayed, and most patients therefore present with advanced disease.1 However surgery should still be considered in all patients with benign tumours and in most with malignant disease, even in advanced disease; only rarely is another local procedure—such as endotracheal stents, debridement, or brachytherapy—appropriate.2 Selection criteria and treatment indications are not consistent and even patients with resectable disease are often managed with palliative treatments. This approach is probably due to lack of awareness of the curative effect of surgery rather than the unavailability of prospective studies that assess and compare treatments, which are almost impossible to do because of the rarity of the tumours. Here, we summarise the evidence available to guide clinicians in treatment of patients with primary tracheal tumours.

Section snippets

Anatomy

The adult trachea is about 12 cm in length, 1·5–2·5 cm wide, and connects the larynx to the carina; the distal two-thirds is located within the thorax. The trachea is composed of c-shaped cartilage rings and is spanned by the trachealis muscle, which provides support anteriorly and laterally and is made up of longitudinal and transverse layers of smooth muscle cells. It also has a posterior membrane and fibrous tissue with a thick layer that covers the outer surface of the cartilaginous ring

Epidemiology

Primary cancer of the upper respiratory tract accounts for more than 1% of malignant diseases, but the frequency of tumours is not consistent throughout the upper respiratory tract. Although the supraglottis is affected in 1·3 per 100 000 people, and the glottis in 2·3 per 100 000 people, fewer than 0·04 per 100 000 population have tumours in the subglottis or trachea.9, 10 Primary tracheal tumours account for 0·1–0·4% of malignant diseases, with 2·6 new cases arising per million people every

Pathology

Primary tracheal tumours can arise from the respiratory epithelium, salivary glands, and mesenchymal structures of the trachea (panel).19, 20 In adults, 90% of primary tumours are malignant,7 compared with 10–30% in children.15, 16 Squamous-cell carcinoma and adenoid cystic carcinoma, which occur in about the same proportions, account for about two-thirds of adult primary tracheal tumours. The remaining third are distributed widely in a heterogeneous group of malignant or benign tumours,19, 20

Clinical presentation

Primary tracheal tumours can cause signs and symptoms of upper-airway obstruction (dyspnoea, wheezing, and stridor), mucosal irritation and ulceration (cough and haemoptysis), or direct invasion and involvement of continuous structures (recurrent nerve palsy and dysphagia). Distant metastases develop in fewer than 10% of patients. Diagnosis is often not made until several months after first presentation in adults, possibly because the tracheal lumen has a large functional reserve and tumours do

Diagnosis

Shortness of breath and wheezing that is unresponsive to bronchodilators should arouse suspicion of a tracheal tumour. Conventional chest radiographs are rarely diagnostic, yet are commonly obtained as the initial radiological test, and tumours can be easily overlooked (figure 3). CT is the most useful method to assess tracheal tumours radiologically, and is regarded as the standard imaging technique for diagnosis and to assess the extent of the tumour and the delineation of the relation of the

Management

Primary benign and malignant tumours are usually treated with surgery, endoscopic resection by various techniques, and radiotherapy. However, only surgery can cure all benign and low-grade malignant tumours, achieve long-term survival in tracheal carcinomas, provide pathological confirmation of complete tumour removal, and relieve airway obstruction permanently.7, 28 The decision to resect or irradiate a tracheal tumour will depend on many factors, including the health of the patient, tumour

Guidelines for management

Epidemiological studies in Europe,12 the USA,14 and Japan,8 have shown that most patients with primary malignant tracheal tumours present with advanced local disease, have squamous-cell tumours, and are treated non-surgically. The low rates of resection in these studies are consistent with those reported by Gelder and Hetzel, UK.13 Furthermore, 5-year survival was 39% in patients with squamous-cell tumours who received resection, but only 7% in those who did not. In patients with adenoid cystic

Conclusion

Primary tracheal tumours are the least common neoplastic lesions of the airway, and this subsite specificity could be related directly to the local mucosal capacity for immunoediting, with immunosurveillance being higher in the subglottis and trachea than in the glottis and bronchi.18 Most patients with primary tracheal benign and malignant tumours present with limited locoregional disease once symptoms occur. Although non-specific symptoms commonly delay diagnosis, resection with primary

Search strategy and selection criteria

Data for this review were identified by searches of Medline, Current Contents, PubMed, and references from relevant articles using, the search terms “primary tracheal tumours”, including all subheadings. Abstracts and reports from meetings were not included. I also reviewed the references of all relevant papers identified, review articles and textbooks. Only papers published in English between June, 1950 and Aug, 2005 were included.

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