Tracheal tumor are neoplasms originating from the tissues of the tracheal wall. They are manifested by difficulty breathing, stridor, paroxysmal painful cough (dry at first, then with purulent sputum), hemoptysis and impaired vocalization. When the esophagus sprouts, difficulties arise when eating. With the collapse of a malignant tumor of the trachea, bleeding, hyperthermia, increased ESR and symptoms of general intoxication develop. The diagnosis is established on the basis of symptoms, laryngoscopy, tracheoscopy, bronchoscopy, radiography, CT, MRI and biopsy. Treatment is surgical removal of the tumor followed by radiotherapy.
General information
Tracheal tumor are rare neoplasms originating from epithelial or connective tissue. They make up 0.1-0.2% of the total number of malignant tumors. They are included in the group of oncological diseases of the respiratory tract. They are more often diagnosed in men. They usually develop at the age of 40-60 years, they are extremely rare in children. Most tracheal tumor are localized in the upper third of the organ. Clinical manifestations, the probability of developing certain complications and the features of metastasis are determined depending on the type of tumor, the localization of the primary focus and the characteristics of the growth of the neoplasm (infiltrating, exotracheal, endotracheal, peritracheal, mixed). Treatment is carried out by specialists in the field of oncology, otolaryngology and pulmonology.
Etiology and pathogenesis
The causes of the development are not exactly clarified. The risk factors include unfavorable heredity, a number of oncoviruses, smoking, alcoholism, chronic inflammatory processes of the oral cavity and respiratory tract (gingivitis, stomatitis, caries, laryngitis, tracheitis, bronchitis), increased radiation levels, unfavorable environmental conditions, prolonged contact with certain chemicals (for example, inhalation of dust during the production of asbestos, inhalation of vapors of varnishes, solvents and dyes during repair work or furniture manufacturing, etc.).
Tracheal tumor can be primary or secondary. The primary ones develop from the epithelium or connective tissue of the trachea. Secondary are localized in neighboring organs (esophagus, mediastinum, thyroid gland, bronchi, larynx), affect the trachea as a result of aggressive infiltrating growth or aspiration metastasis. The most common primary tracheal tumor are adenocystic and squamous cell carcinoma.
Adenocystic cancer (cylindroma) originates from the glandular epithelium, is characterized by relatively slow progression, aggressive local growth and a tendency to relapse. Aspiration, lymphogenic and hematogenic metastasis is possible. Squamous cell carcinoma develops from the squamous epithelium, usually occurs on the posterior or lateral wall of the trachea. This malignant tumor of the trachea is diagnosed in middle-aged and elderly men, in most cases it is formed in the thoracic region or at the bifurcation level. It grows relatively slowly, is prone to germination of surrounding tissues.
Tracheal sarcoma is rarely diagnosed, it originates from connective tissue, and is usually located at the bifurcation level. It may be primarily malignant or arise as a result of the degeneration of a benign neoplasm. Local infiltrating growth prevails, metastases occur only in the later stages of the disease. Rare tracheal tumor also include mucoepidermoid adenomas, carcinoids, reticuloendotheliomas, reticulosarcomas, malignant neurofibromas, hemangiopericytomas and some other neoplasms.
Most often, tracheal tumor spread by local aggressive growth. Aspiration metastasis has a certain value. Metastases to the lymph nodes are often detected. Distant metastasis is observed infrequently, since patients do not have time to live up to this stage of the disease. Distant metastases can be found in the pleura, kidneys, liver, pancreas, skin, adrenal glands and other organs. The cause of death is usually suffocation and other complications associated with local tumor growth.
Symptoms
For some time, the disease is asymptomatic. The first manifestations are usually cough and shortness of breath. Cough is unstable, dry in the early stages, paroxysmal. Subsequently, sputum appears, which is then separated with difficulty, then simultaneously coughed up in large portions. Episodic or regular hemoptysis is possible. Shortness of breath first occurs only with physical exertion, and then persists at rest. The nature of shortness of breath depends on the level of location of the neoplasm. When the upper parts are affected, inspiratory dyspnea develops mainly, when the lower parts are affected, expiratory.
The malignant tumor of the trachea continues to grow and covers a significant part of the tracheal lumen. In the later stages, the patient assumes a forced position (usually sitting) to reduce suffocation and ensure oxygen supply to the lungs. Breathing becomes noisy. While maintaining the elasticity of the trachea, noise appears on exhalation. Over time, the rigidity of the trachea increases more and more, the noise on exhalation is joined by the noise on inhalation. There are disorders of voice formation: hoarseness, hoarseness or aphonia.
When a malignant tumor of the trachea spreads to the esophagus, dysphagia and pain occur when swallowing food. Over time, esophageal obstruction may develop. When the neoplasm disintegrates, sputum with an unpleasant odor appears, bleeding is possible. There is an increase in temperature, symptoms of general intoxication, erythropenia, an increase in ESR and a shift of the leukocyte formula to the left. The collapse of the tumor may be accompanied by a slight decrease in shortness of breath due to partial restoration of the tracheal lumen.
Diagnostics
The diagnosis is established on the basis of anamnesis, clinical symptoms, data from instrumental and laboratory studies. When the neoplasm is located in the upper trachea, it can be seen during a mirror laryngoscopy. With low-lying tracheal tumor, direct laryngoscopy, upper tracheoscopy or bronchoscopy is required. During the procedure, the doctor takes tissue or mucus for subsequent histological and cytological examination.
Radiography, CT and MRI are used to determine the size and location of the tumor, the degree of involvement of surrounding tissues and nearby anatomical formations. In the process of diagnosing a malignant tumor of the trachea, the possibility of a false negative result of histological examination is taken into account. Crucial importance is attached to clinical data and the cumulative results of instrumental studies. Sometimes differential diagnosis with tuberculous or syphilitic granulomas is required.
Treatment and prognosis
Combined treatment – surgical removal of the neoplasm in combination with pre- and postoperative radiation therapy. The possibility of radical surgical intervention is determined by the prevalence and localization of a tracheal tumor. The higher the neoplasm is located, the easier it is to remove it. The tumor is excised within healthy tissues. The tracheal rings are brought together and sewn together as much as possible. With small defects, the trachea is plasticized using a free skin-muscle flap. With extensive defects, muscle-periosteal or cartilage grafts or skin flaps reinforced with tantalum mesh are used.
In case of inoperable tracheal tumor, palliative surgical interventions are performed – tracheostomy or tracheofissure. When the lower part of the trachea is compressed, a tracheotomy cannula is carried out through the stenosed area. Radiation therapy and chemotherapy are prescribed for both operable and inoperable tracheal tumor, however, in the case of inoperable cancer, the effect of its use is unstable. Some time after the end of the course of treatment, the tumor recurs. To increase the effectiveness, intra-cavity brachytherapy and remote radiation therapy are used.
The prognosis for tracheal tumor in most cases is unfavorable. With squamous cell carcinoma, the five–year survival rate is about 40%, with adenocystic cancer – from 65 to 85%. In case of late initiation of treatment, the prognosis worsens. Preventive measures include quitting smoking, stopping contact with harmful chemicals and early access to a pulmonologist when dry cough, increasing shortness of breath and other symptoms characteristic of tracheal tumor appear.