Endobronchial Adenoid Cystic Carcinoma*

12 Shin MS, Liendo C, Ho KJ. Pneumocystis carinii pneumonia in an AIDS patient: unusual manifestation as multiple cavitary and non cavitary peripheral pulmonary nodules and spontaneous pneumothorax. Clin Imag 1989; 13:225-27 13 Milligan SA, Stulbarg MS, Gamsu G, Golden JA. Pneumocystis carinii pneumonia radiographically simulating tuberculosis. Am Rev Respir Dis 1985; 132:1124-26 14 Conte JE, Hollander H, Golden JA. Inhaled or reduced-dose intravenous pentamidine for Pneumocystis carinii pneumonia. Ann Intern Med 1987; 107:495-98

Endobronchial Adenoid Cystic Carcinoma* Hong W Chin, M.D., Ph.D.;t Tom DeMeester; M.D., F.C.C.F.;:j: Richard Y. Chin, B.A.;§ and Bruce Boman, M.D., Ph.D.1I

A case of endobronchial adenoid cystic carcinoma of the lung is presented. This is probably the first report of endobronchial brachytherapy being applied for recurrent adenoid cystic carcinoma of the lung with a good response. We found that endobronchial implantation therapy is very useful in the control of endobronchial adenoid cystic carcinoma. In addition, a clinical history and options for treatment are brieRy discussed. (Cheat 1991; 100:1464-65)

A

denoid cystic carcinoma is an uncommon histologic variant of adenocarcinoma which usually arises from the salivary. laCrimal, or other exocrine glands.' Occasionally, this uncommon tumor is seen in the breast, larynx, or lung. The extreme rarity of primary adenoid cystic carcinoma of the lung prompted this report of a case which we encountered and treated with endobronchial implantation therapy. CASE REPORT

This 82-year-old white male patient was admitted to St. Joseph Hospital on March 7,1990. In June of 1983, he had been diagnosed as having adenoid cystic carcinoma on the left main-stem bronchus, and laser surgery was /irst performed in August 1983. Since then, the laser surgery had been repeated at intervals of approximately six months because of recurren(:es of the tumor. Lately, the intervals between recurrences have been getting shorter, and his last procedure was done in January 1990. A chest x-ray examination at admission in March 1990 revealed atelectasis of the left lung, with shift of the mediastinum and trachea toward the left (Fig 1). Following admission, the patient underwent endobronchial laser surgery to relieve a bronchial stenosis caused by his tumor. The patient tolerated the procedure well; however, he developed bronchial edema after surgery. Over the next two days the lungs spontaneously reinHated, with good recovery of the patient. Because of the frequent recurrences of the tumor, endobronchial radiation treatment was instituted (Fig 2). The patient responded well to endobronchial radiation therapy and is doing well, with no symptoms as of this report (six months after therapy). ·From Overton Brooks VA Medical Center, Shreveport, La, and Creighton University School of Medicine, Omaha. tProfessor and Director, Radiation Oncology, Overton Brooks VA Medical Center. :j:Professor and Chairman, Department of Surgery. §Medical student, Harvard University, Cambridge, Mass. IIDirector, Cancer Center, Creighton University. Reprint requests: Dr. Chin, LSU-Overton brooks VA Medical Center, 510 East Stoner Avenue, Shreveport, LA 71101

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FIGURE 1. Chest roentgenogram taken at admission reveals atelectasis of left lung, with minimal shift of mediastinum and trachea toward left.

DISCUSSION

Adenoid cystic carcinoma of the lung is extremely uncommon. According to Sweeney and Thomas! only three of 1,500 lung cancers that they reviewed were adenoid cystic carcinomas. Among 5,500 patients with primary lung cancer at Memorial Sloan Kettering Hospital in New York, only five had a histologic diagnosis of adenoid cystic carcinoma. J Because of its extreme rarity, only limited information is available in the literature in regard to its clinical course and the best therapy. Adenoid cystic carcinomas of the lung develop more often in larger bronchi and the trachea than in peripheral locations. They also tend to locally invade the

FIGURE 2. Anteroposterior view of chest con/irms ~()od placement of temporary endobronchial'92Ir implant seeds in left lun~.

Endobronchial Adenoid Cystic Carcinoma (Chin

at a/)

surrounding tissues. Generally recognized characteristics of adenoid cystic carcinoma are its infiltrative nature, a tendency toward local recurrence, and usually a prolonged natural course of the disease. Adenoid cystic carcinoma of the lung does not appear to be associated with smoking. The common clinical manifestations are, of course, related to bronchial symptoms such as cough, shortness of breath, wheezing, and hemoptysis. The chest roentgenogram is often normal or shows only subtle abnormalities. Although the tumor is limited to the site of its origin for a relatively long time and although proper local therapy can often be adequate for local control, adenoid cystic carcinomas often metastasize to distant organs. Resection of the tumor is the treatment of choice, but the rate of complete resectability has been lo~ According to the Mayo experience,· only 3 of 13 patients with a diagnosis of adenoid cystic carcinoma of the bronchus were able to undergo a complete resection because of extension of the tumor into the surrounding tissues. Therefore, in addition to surgical therapy, radiation treatment should often play an important role as an adjuvant therapy, as well as a means of controlling inoperable and residual tumors. Because local recurrence and pulmonary metastases are frequently seen even after curative resection of the primary tumor, surgery alone may not be adequate, and adjuvant radiotherapy is recommended in patients with adenoid cystic carcinoma. 5 In fact, one large series reported a mean survival of8.3 years after curative resection.· As the tumors frequently persist or recur over as long as 30 years, the prognosis is guarded. The survival rates reported are 75 percent at five years and 55 percent at ten years. 5 CONCLUSION

To our knowledge, this is the first report of an endobronchial adenoid cystic carcinoma that was treated with endobronchial brachytherapy with a good response. This patient has shown a typical slow-growing course of the disease in spite of multiple local recurrences. As he has now survived more than seven years since the first diagnosis, the surgical treatments he received must have been excellent and thorough; however, such successful surgical management was insufficient to eradicate the disease completely. Postoperative radiation treatment might be useful, particularly when the surgical margins are not ample. REFERENCES

1 Cleveland RH, Nice CM Jr, Zisldnd J. Primary adenoid cystic carcinoma (cylindroma) of the trachea. Radiology 1977; 122:597600

2 Sweeney \VB, Thomas JM. Adenoid cystic carcinoma of the lung. Contemp Sur~ 1986; 28:97-100 3 Turnbull AD, Huvos AG, Goodner fr. Beattie EF Jr. The malignant potential ofbronchial adenoma. Ann Thorac Surg 1972; 14:453-62 4 Conlan AA, Payne WS, Woolner LB, Sanderson DR. Adenoid cystic carcinoma (cylindroma) and mucoepidermoid carcinoma of the bronchus. J Thorac Cardiovasc Surg 1978; 76:369-77 5 Pearson FG, Todd TRJ, Cooper JD. Experience with primary neoplasms of the trachea and carina. J Thorac Cardiovasc Surg 1984; 88:511-18

Functional Laryngeal Obstruction Relieved by Panting* Arthur E. Pitchenik, M.D., F.C.C.R

A 49-year-old man presented with a two-day history of severe recurrent dyspnea and inspiratory stridor. A chest roentgenogram, computed tomographic scan of the neck, direct laryngoscopy, and bronchoscopy excluded organic upper airway obstruction. Laryngospasm occurred during the bronchoscopy. Although flow volume loops revealed severe upper airway obstruction (inspiratory and expiratory), airway resistance measured plethysmographically (during panting) was normal. Because of this observation, panting was recommended for relief of the patient's recurrent attacks offunctional laryngeal obstruction. The panting maneuver immediately and completely relieved all 25 to 30 subsequent attacks. After the patient recovered clinically, a flow volume loop was repeated and was found to be normal. The marked discrepancy between severe Row limitation (as detected by Row volume loops) and normal airway resistance (measured plethysmographically) may be a diagnostic test for functional laryngeal obstruction, and panting may be an effective emergency measure for its relief. Relief by panting may also suggest the diagnosis. A second patient with an almost identical symptom complex is described, in whom the panting maneuver was also dramatically successful in promptly aborting recurrent severe attacks of airway obstruction and stridor. (Chest 1991; 100:1465-67)

T aryngeal dysfunction causing acute airway obstruction is .1..J probably much more common than appreciated. It loll

is often misdiagnosed and treated as acute asthma or acute organic upper airway obstruction. Some patients have received unnecessary emergency endotracheal intubation or tracheotom~3-5.7·10 A patient with paroxysmal functional larryngeal obstruction is described in whom 25 to 30 attacks were immediately and completely relieved by panting. The panting maneuver was suggested as a mode of treatment because airway resistance (measured plethysmographically during panting) was normal despite the fact that flow volume loops (measured during forced inspiratory and expiratory capacity maneuvers) revealed severe upper airways obstruction. The marked discrepancy between inspiratory flow limitation and airway resistance has previously been described in two patients with nonorganic acute upper airway (inspiratory) obstruction, and on this basis, it was suggested as a diagnostic test for this condition.· This is supported by similar pulmonary function findings in our patient. Although there is a physiologic basis for it, a literature review reveals no previous reports that document that panting symptomatically relieves functional laryngeal obstruction and thereby suggests the diagnosis at the same time. CASE REPORT

A 49-yeaMlld businessman presented with a two-day history of severe recurrent attacks of inspiratory "crowing" and inability to breathe. He stated that the attacks felt "life threatening" and were Division, University of Miami and Veterans AdmInIstration Medical Centers, Miami. Reprint requests: Dr. Pitchenik, Pulmonary Section (lllF), Miami VA Medical Center; 1201 NW 16th Street, Miami 33125 *Fro~ t~e Pt,Ilmonar~

CHEST I 100 I 5 I NOVEMBER. 1991

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