Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Tracheal adenoid cystic carcinoma
- Radiologic Findings
- Fig. 1. Chest posteroanterior radiograph demonstrating a small round soft-tissue-density nodule within the trachea at the level of the thoracic inlet.
Figs. 2–5. Pre- and post-contrast computed tomography (CT) scans showing a 1.8-cm intraluminal polypoid nodule arising from the lateral wall with partial obstruction of the tracheal lumen.
- Brief Review
- Primary tracheal tumors are uncommon, accounting for < 0.1% of all cancer-related deaths. Most primary tracheal tumors in adults (≥ 50%) are malignant. Squamous cell carcinoma (54.5%) is the most common primary tumor of the trachea, followed by adenoid cystic carcinoma (18%).
Adenoid cystic carcinomas seem to be unrelated to smoking. There is no sex predilection, and the average age of patients is in the fifth decade. The symptoms are usually related to airway obstruction. Dyspnea and hemoptysis may also occur. Wheezing is a common symptom. Patients may be incorrectly diagnosed and treated for asthma.
The radiologic appearance of tumors can be divided into intraluminal wall thickening and exophytic forms. Intraluminal tumors are intraluminal hemispheric masses whose spread is limited to the tracheal wall. The wall thickening type manifests as diffuse wall thickening due to tumor infiltration, and the thickened part presents as either a flat or spindle-shaped mass along the tracheal wall. The exophytic form is radiologically characterized by an extratracheal tumor.
Bronchoscopy is a complementary diagnostic procedure. The chief advantage of imaging is the demonstration of tracheal wall thickening and extraluminal changes. Computed tomography (CT) is valuable for demonstrating the primary tumor and its extent. Extraluminal growth in the transverse plane, a common feature of adenoid cystic carcinomas, is observed. CT seems to be most useful for the detection of distant metastases. CT cannot predict the invasion of local structures. The use of contrast material is not useful for improving the visualization of fat planes between mediastinal tumors and the aorta. CT underestimates the extent of the tumor in the longitudinal plane. Adenoid cystic carcinomas may grow submucosally without producing a distinct mass.
Pathologically, adenoid cystic carcinomas may infiltrate through the fibrous membrane between adjacent cartilage plates into the peritracheal tissue. Perineural or intraneural infiltration, as well as pericardial, great vessel, or pulmonary hilar involvement may occur. Metastasis to regional lymph nodes may be present in 10% of cases at the initial diagnosis of the tumor. Distant metastases may develop in the lungs, liver, abdomen, lymph nodes, or bones.
The treatment of adenoid cystic carcinomas consists of surgical resection and anastomosis. The criteria for inoperability include excessive longitudinal extent, macroscopic mediastinal nodal metastases, distant metastatic deposits, and direct invasion of contiguous mediastinal structures such as the aorta and esophagus. When surgery is not indicated, usually because of extensive local disease or metastases, radiation therapy is recommended. The eventual outcome of "curative" surgery is difficult to predict because of the recurrent tendency of this tumor.
The 5- and 10-year survival rates are excellent (79% and 57%, respectively). However, the long-term outcome is poor owing to late local recurrences (27%) and late distant metastatic spread (55%) occurring after a median time interval of 24–180 months after surgery.
- References
- 1. Spizarny DL, Shepard JO, Mcloud TC, et al. CT of adenoid cystic carcinoma of the trachea. AJR 1986 ; 146 : 1129-1132
2. Li W, Ellerbroek NA, Libshitz HI. Primary malignant tumors of the trachea : a radiologic and clinical study. Cancer 1990 ; 66 : 894-899
3. Prommegger R, Salzer GM, Long-term results of surgery for adenoid cystic carcinoma of the trachea and bronchi. Eur J Surg Oncol 1998 ; 24(5) ; 440-444
4. Azar T, Abdul-Karim FW, Tucker HM. Adenoid cystic carcinoma of the trachea. Laryngoscope 1998 ; 108(9) ; 1297-1300
- Please refer to
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- Keywords