Adenoid cystic carcinoma of the tracheobronchial tree: clinicopathologic and immunohistochemical studies of 21 cases.

AIMS: To review retrospectively 21 cases adenoid cystic carcinoma of the tracheobronchial tree (TACC) with emphasis on their clinical and pathologic features, treatment and the possible prognostic factors.

METHODS AND RESULTS: 21 cases TACC diagnosed by surgical biopsy or resection at the Peking Union Medical College Hospital (PUMCH) over 10 years. Patients aged 24-69 years (median, 49 years), 6 men/15 women. Cough (18/21), dyspnea (14/21) and hemoptysis (10/21) were the most frequent manifestations. 15 patients had tumors in trachea. Ten patients had pathologically positive margin (n = 11). Immunohistochemically, BCL-2, CD117, P16, type IV collagen, SMA and P63 were positive (20/20); GFAP was focally positive (4/20); TTF-1 and P53 were negative (0/20). Ki-67 index ranged from 2% to 35%. Fifteen patients had followed up, 13 of which received postoperative radiotherapy. The median relapse-free survival (RFS) was 56.9 months and the 5-year RFS was 48.6%. By univariate analysis, postoperative radiotherapy had favorable prognostic significance (P < 0.05).

CONCLUSIONS: TACC, which is mainly located in primary trachea or bronchus, is difficult to be detected at early stage. The tumors are not likely to be completely removed by surgery, and postoperative radiotherapy is helpful for reducing the likelihood of recurrence and metastasis.