대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Adenoid Cystic Carcinoma of the Trachea

Radiologic Findings

Chest radiograph shows multiple lung nodules and narrowing of the cervical trachea. CT scan at the level of clavicle shows markedly thickened tracheal wall with narrowing of the lumen. The thyroid glands are involved. Another CT scan at the level of innominate vein and artery shows thickened tracheal wall and osteolytic metastasis at the posterior arc of the left 4th rib. CT scan of lung window setting shows multiple nodules suggesting hematogenous metastasis in both lungs.

Bronchoscopic biopsy shows adenocarcinoma of tubular shape in the stroma. Fine needle aspiration biopsy(H&E and Popanicolou smear) shows adenoid cystic carcinoma of cribriform pattern, characterized by hyaline globules and surrounding small monotonous cells.

Brief Review

Primary tracheal tumors are rare, occurring in 0.2 per 100,000 persons per year. Adenoid cystic carcinoma (ACC) is the second most common histologic type of tracheal malignancy (most common: squamous cell carcinoma, 3rd common: mucoepidermoid, and 10% of sarcoma, lymphoma, adenocarcinoma, chondrosarcoma, plasmacytoma, small cell carcinoma, and metastases). Tracheal ACC is an indolent tumor that affects people at any age but has a peak incidence in the fifth decade. There is a nearly equal male-to-female ratio. Almost half of tracheal ACCs occur in the proximal trachea, accounting for the most common presenting symptoms: dyspnea, cough, and hoarseness.[1]
Until recently, adenoid cystic carcinoma (ACC) has been considered to be a borderline disease between benign and malignant because of its low level of malignancy, slow growth and scarce aggressiveness. ACC is now a histologically well defined tumor and is currently classified as malignant; it has a prolonged natural history, is infiltrative, and tends to recur locally and give rise to local-regional metastases or, more rarely, remote metastases.[2]
Takahashi[3] report a 33-year-old woman with adenoid cystic carcinoma of the trachea was treated by resection of the upper and mid-trachea, plus total laryngectomy, subtotal thyroidectomy, partial resection of the cervical esophageal muscle layer and mediastinal tracheostomy. This tumor invaded directly into the thyroid gland, the larynx, the recurrent laryngeal nerve and the cervical esophagus. Three years after the operation, metastases developed in the right lung (S10, S2) and partial resection of the lung was performed. This patient is now free from disease. Aggressive surgical resection is important for the improvement of survival in this condition, although this tumor shows low grade malignancy and is slowly growing.

References

1. Schneider P, Schirren J, Muley T, Vogt-Moykopf I. Primary tracheal tumors: experience with 14 resected patients. Eur J Cardiothorac Surg. 2001 Jul;20(1):12-8.
2. Blanco Orozco AI, Ginel Canamaque A, Sanchez Navarro JM, Torres Cansino M. Adenoid cystic carcinoma of respiratory airways: course and treatment. Arch Bronconeumol. 1999 Jun;35(6):257-60. Spanish.
3. Takahashi H, Kubota M, Nakata T, Nagai I, Kimura S, Noguchi S..A case of pulmonary metastasis from adenoid cystic carcinoma of the trachea]. Nippon Kyobu Geka Gakkai Zasshi. 1990 Jun;38(6):1063-7. Review. Japanese.

Keywords

Airway, Malignant tumor,