A 43-year-old white female was referred to pulmonary clinic for the evaluation of hemoptysis. Her primary care physician had followed her for the past year for chronic cough. When questioned further she reported that this cough had been increasing productive of thick material that was “strange tasting” and seemed almost tissue like as opposed to sputum she had experienced in the past. She reported streaky hemoptysis over two weeks time, a 9 lb. weight loss over 2 months, but denied fever, denied chills, denied dyspnea on exertion. Pt had been treated off and on with inhalers over the past year with no benefit.

Past history included a negative mammogram and pap and pelvic exam within 3 months. The patient has never smoked and drinks alcohol rarely. She currently takes no medications and has no allergies.

Family history is significant for colon carcinoma in her mother and coronary artery and mitral valvular disease in her father.

Physical exam significant for markedly reduced breath sounds posteriorly on the left.

CXR showed tracheal deviation to the left and left lower lobe collapse as well as an opacification in the subcarinal area. Chest CT performed subsequently showed a approx. 4 cm mass in the subcarinal area extending around the left mainstem bronchus to the hilar region with probable impingement and growth into the left mainstem bronchus. There is subsequent LLL collapse. No effusions or lymphadenopathy are appreciated.

Bronchoscopy was performed and a mass arising in the left mainstem bronchus 3 cm from the carina was noted. The mass nearly occluded the LLL bronchus. The carina was minimally widened. Biopsies of the mass were adenoid cystic carcinoma. Carinal biopsies were also consistent with adenoid cystic despite its near normal appearance. Tracheal biopsies were negative.

CT Surgery was consulted and the mass considered inoperable due to the carinal involvement. She was referred to Radiation Oncology, but would further obstruct her left mainstem in the interim and require emergent radiation therapy. She would reinflate the lung partially (the LLL did not reinflate). She is currently receiving an experimental protocol at the University of Washington for neutron beam therapy.

Adenoid cystic carcinoma is the most common subtype of tracheobronchial gland tumor. Approximately 80% arise with equal frequency in the trachea and main bronchi. 10-15% develop in the lung periphery. Although a rare neoplasm, with squamous cell carcinoma, it comprises the vast majority of primary tumors of the trachea. The ages are reported between 18-65 but the average is between 45 and 50. Cigarette smoking is not associated.

The tumor forms a firm nodule that is centrally located in approximately 90% of the cases; generally it is between 1 and 5 cm in diameter at the time it is resected. Adenoid cystic has a tendency to spread along and encircle the bronchus from which it arise as well as neighboring bronchi and vessels.

Histologically the tumor is identical to that of salivary gland tumors, consisting most commonly of rather uniform cells with relatively little cytoplasm arranged in well-defined nests of variable size. The nests frequently have a cribiform pattern in which the cells are separated by well-defined cystic spaces containing a mucinous substance that stains strongly with alcian blue and weakly with periodic acid-Schiff. The microscopic appearance is identical to that of adenoid cystic in other locations and a metastatic adenoid cystic in the lung cannot be distinguished from a lung primary lesion. It is postulated that adenoid cystic carcinoma arises in the collecting duct acinar region of the bronchial glands and lining (secretory) cells are present in this location.

Obstructive symptoms and hemoptysis are consequently common clinical findings. Recurrences are a problem and may occur many years after primary tumor resection.