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Endobronchial hamartoma
Journal of
1 38
Thoracic and Cardiovascular
Surgery
Endobronchial hamartoma Fred D. Kurrus, M.D., and J. Harold Conn, M.D., Jackson, Miss.
Xulmonary hamartomas occurring in the lung periphery are commonly found in asymptomatic patients or as an incidental finding at autopsy. Endobronchial hamar tomas, however, are quite different in that they may cause respiratory symptoms due to obstruction of the bronchus with subse quent destruction of the distal lung. There have been 52 cases of endobronchial hamartoma documented in the English literature. The following report is of the fifty-third case. Case report A 68-year-old white man was admitted to the Veterans Administration Hospital on Aug. 13, 1964, with a chief complaint of having had a "chest cold" and "asthma" for the past 2Vi years. He had had intermittent episodes of wheezing and coughing and had lost 10 pounds. He stated that he had quit smoking 20 years prior to this time. Four days before admission he had had a severe coughing episode associated with hemoptysis. Physical examination was unremarkable ex cept for minimal dullness at the base of the right lung. Pulmonary work-up was initiated and chest x-ray films showed an atelectasis of the right lower and right middle lobes (Fig. 1). Bronchoscopy revealed a smooth tumor filling the right intermediate bronchus, and biopsy was reported as a suspected chondroma, since only degenerated cartilage tissue was present. The remainder of the pulmonary work-up was normal and on Sept, 2, 1964, a right thoracotomy was performed. At operation, the patient was found to have a markedly emphysematous right upper lobe, which filled almost the entire right hemithorax. The right lower and middle lobes were contracted into a From the Surgical Service, Veterans Administration Hos pital, University of Mississippi Medical Center, Jack son, Miss. Received for publication Feb. 18, 1965.
small cystic mass surrounded by dense fibrotic ad hesions. A right middle and lower bilobectomy was performed and, when the main bronchus was opened just below the right upper lobe orifice, a 2 by 2.5 cm., round, smooth tumor was found and removed (Fig. 2). It had been attached distally by a small (2 mm.) pedicle. The pathologist's report was a hamartoma. The right middle and lower lobes were completely destroyed and were filled with a brown, gelatinous, purulent ma terial. Since the occlusion had occurred over a considerable length of time, there was no problem with expansion of the right upper lobe, and it completely filled the right chest due to compensa tory emphysema. The postoperative course was uneventful, and he was discharged 2 weeks postoperatively. Comment The hamartoma is a tumor containing an abnormal mixture of normal components of the organ in which it arises. This is in contradistinction to the teratoma in which tissues foreign to the organ are present. Although the peripheral pulmonary hamar-
Fig. 1. Preoperative roentgenogram shows atelec tasis of right middle and lower lobes.
Volume 50 Number 1 July, 1965
toma occurs about once in every 400 per sons,1 the endobronchial hamartoma is quite a rare finding. In a recent extensive review of the English literature, Stengel2 found 50 cases of endobronchial hamartoma and added the fifty-first case. Aletras 3 reported an additional case to make a reported total of 52. Donoghue4 found only six endobron
Endobronchial hamartoma
13 9
chial hamartomas in 11,626 patients who underwent bronchoscopy at the Mayo Clinic. The most generally accepted theory as to the etiology of the hamartoma is, as stated by Womack,5 that these tumors represent failures of embryonic bronchial buds to develop normally. Microscopically
Fig. 2. Gross specimen shows large amount of cartilage and small pedicle.
Fig. 3. Microscopic section demonstrates mixture of components usually found in hamartomas (x50; reduced %).
Journal of
14 0
Kurrus and Conn
Thoracic and Cardiovascular Surgery
there is usually seen a preponderance of cartilaginous tissue which leads to the occa sional diagnosis of chondroma. As in our case, other constituents, such as fat tissue and epithelial derivative, are almost invari ably present (Fig. 3). The usual presenting symptoms of a patient with an endobronchial hamartoma are cough, wheezing, "asthma," hemoptysis, and progressive dyspnea. Physical examina tion usually is unremarkable and x-ray films may or may not show distal atelectasis or pneumonitis. Lemon and Good6 have dis cussed the difficulty in making an accurate preoperative diagnosis and state that occasionally a typical picture of a smooth hilar density with distal atelectasis or pneumonitis may be found. However, in most series the tumor itself has rarely been seen on x-ray film. Donoghue4 has mentioned that frequently endobronchial hamartomas can be com pletely removed by bronchoscopy. However, in our case, as in many, the distal lung was found to be destroyed, which makes re-expansion impossible. Young7 reported the cases of 2 patients similar to ours, both of whom underwent resection of the distal lung in addition to the tumor. We believe that the slow growth of these tumors causes a gradual occlusion of the bronchus, re sulting in a long-standing irreversible cystic dilatation of the distal lung. Thus the treat
ment of choice should be thoracotomy with removal of not only the tumor but also the bronchiectatic distal lung. Summary The endobronchial hamartoma is a rare tumor and, although benign, can cause severe respiratory problems with eventual bronchial occlusion and destruction of the distal lung. A case is reported which brings the total number to 53 in the English litera ture. The recommended treatment of choice is thoracotomy with excision of the tumor and any distal bronchiectatic lung. REFERENCES 1 Liebow, A. A.: Atlas of Tumor Pathology, Section 5, Fascicle 17, Tumors of the Lower Respiratory Tract, Washington, D. C , 1952, Armed Forces Institute of Pathology. 2 Stengel, B. F., Lepley, D., and Weisel, W.: Endobronchial Hamartoma, a Difficult Diag nostic Problem, Am. J. Surg. 107: 637, 1964. 3 Aletras, A., Björk, V. O., Foss, B., Intonti, F., and Madsen, R.: Benign Bronchopulmonary Neoplasm, Dis. Chest 44: 498, 1963. 4 Donoghue, E. F., Andersen, H. A., and Mc Donald, J. R.: Unusual Bronchial Tumors, Ann. Otol., Rhin. & Laryng. 65: 820, 1956. 5 Womack, N. A., and Graham, E. A.: Mixed Tumors of the Lung, Arch. Path. 26: 165, 1938. 6 Lemon, W. R., and Good, C. A.: Hamartoma of the Lung: The Improbability of Preoperative Diagnosis, Radiology 55: 693, 1950. 7 Young, J. M., Jones, E., Hughes, F. A., Foley, F. E., and Fox, J. R.: Endobronchial Hamar toma, J. THORACIC SURG. 27: 300,
1954.
1 38
Thoracic and Cardiovascular
Surgery
Endobronchial hamartoma Fred D. Kurrus, M.D., and J. Harold Conn, M.D., Jackson, Miss.
Xulmonary hamartomas occurring in the lung periphery are commonly found in asymptomatic patients or as an incidental finding at autopsy. Endobronchial hamar tomas, however, are quite different in that they may cause respiratory symptoms due to obstruction of the bronchus with subse quent destruction of the distal lung. There have been 52 cases of endobronchial hamartoma documented in the English literature. The following report is of the fifty-third case. Case report A 68-year-old white man was admitted to the Veterans Administration Hospital on Aug. 13, 1964, with a chief complaint of having had a "chest cold" and "asthma" for the past 2Vi years. He had had intermittent episodes of wheezing and coughing and had lost 10 pounds. He stated that he had quit smoking 20 years prior to this time. Four days before admission he had had a severe coughing episode associated with hemoptysis. Physical examination was unremarkable ex cept for minimal dullness at the base of the right lung. Pulmonary work-up was initiated and chest x-ray films showed an atelectasis of the right lower and right middle lobes (Fig. 1). Bronchoscopy revealed a smooth tumor filling the right intermediate bronchus, and biopsy was reported as a suspected chondroma, since only degenerated cartilage tissue was present. The remainder of the pulmonary work-up was normal and on Sept, 2, 1964, a right thoracotomy was performed. At operation, the patient was found to have a markedly emphysematous right upper lobe, which filled almost the entire right hemithorax. The right lower and middle lobes were contracted into a From the Surgical Service, Veterans Administration Hos pital, University of Mississippi Medical Center, Jack son, Miss. Received for publication Feb. 18, 1965.
small cystic mass surrounded by dense fibrotic ad hesions. A right middle and lower bilobectomy was performed and, when the main bronchus was opened just below the right upper lobe orifice, a 2 by 2.5 cm., round, smooth tumor was found and removed (Fig. 2). It had been attached distally by a small (2 mm.) pedicle. The pathologist's report was a hamartoma. The right middle and lower lobes were completely destroyed and were filled with a brown, gelatinous, purulent ma terial. Since the occlusion had occurred over a considerable length of time, there was no problem with expansion of the right upper lobe, and it completely filled the right chest due to compensa tory emphysema. The postoperative course was uneventful, and he was discharged 2 weeks postoperatively. Comment The hamartoma is a tumor containing an abnormal mixture of normal components of the organ in which it arises. This is in contradistinction to the teratoma in which tissues foreign to the organ are present. Although the peripheral pulmonary hamar-
Fig. 1. Preoperative roentgenogram shows atelec tasis of right middle and lower lobes.
Volume 50 Number 1 July, 1965
toma occurs about once in every 400 per sons,1 the endobronchial hamartoma is quite a rare finding. In a recent extensive review of the English literature, Stengel2 found 50 cases of endobronchial hamartoma and added the fifty-first case. Aletras 3 reported an additional case to make a reported total of 52. Donoghue4 found only six endobron
Endobronchial hamartoma
13 9
chial hamartomas in 11,626 patients who underwent bronchoscopy at the Mayo Clinic. The most generally accepted theory as to the etiology of the hamartoma is, as stated by Womack,5 that these tumors represent failures of embryonic bronchial buds to develop normally. Microscopically
Fig. 2. Gross specimen shows large amount of cartilage and small pedicle.
Fig. 3. Microscopic section demonstrates mixture of components usually found in hamartomas (x50; reduced %).
Journal of
14 0
Kurrus and Conn
Thoracic and Cardiovascular Surgery
there is usually seen a preponderance of cartilaginous tissue which leads to the occa sional diagnosis of chondroma. As in our case, other constituents, such as fat tissue and epithelial derivative, are almost invari ably present (Fig. 3). The usual presenting symptoms of a patient with an endobronchial hamartoma are cough, wheezing, "asthma," hemoptysis, and progressive dyspnea. Physical examina tion usually is unremarkable and x-ray films may or may not show distal atelectasis or pneumonitis. Lemon and Good6 have dis cussed the difficulty in making an accurate preoperative diagnosis and state that occasionally a typical picture of a smooth hilar density with distal atelectasis or pneumonitis may be found. However, in most series the tumor itself has rarely been seen on x-ray film. Donoghue4 has mentioned that frequently endobronchial hamartomas can be com pletely removed by bronchoscopy. However, in our case, as in many, the distal lung was found to be destroyed, which makes re-expansion impossible. Young7 reported the cases of 2 patients similar to ours, both of whom underwent resection of the distal lung in addition to the tumor. We believe that the slow growth of these tumors causes a gradual occlusion of the bronchus, re sulting in a long-standing irreversible cystic dilatation of the distal lung. Thus the treat
ment of choice should be thoracotomy with removal of not only the tumor but also the bronchiectatic distal lung. Summary The endobronchial hamartoma is a rare tumor and, although benign, can cause severe respiratory problems with eventual bronchial occlusion and destruction of the distal lung. A case is reported which brings the total number to 53 in the English litera ture. The recommended treatment of choice is thoracotomy with excision of the tumor and any distal bronchiectatic lung. REFERENCES 1 Liebow, A. A.: Atlas of Tumor Pathology, Section 5, Fascicle 17, Tumors of the Lower Respiratory Tract, Washington, D. C , 1952, Armed Forces Institute of Pathology. 2 Stengel, B. F., Lepley, D., and Weisel, W.: Endobronchial Hamartoma, a Difficult Diag nostic Problem, Am. J. Surg. 107: 637, 1964. 3 Aletras, A., Björk, V. O., Foss, B., Intonti, F., and Madsen, R.: Benign Bronchopulmonary Neoplasm, Dis. Chest 44: 498, 1963. 4 Donoghue, E. F., Andersen, H. A., and Mc Donald, J. R.: Unusual Bronchial Tumors, Ann. Otol., Rhin. & Laryng. 65: 820, 1956. 5 Womack, N. A., and Graham, E. A.: Mixed Tumors of the Lung, Arch. Path. 26: 165, 1938. 6 Lemon, W. R., and Good, C. A.: Hamartoma of the Lung: The Improbability of Preoperative Diagnosis, Radiology 55: 693, 1950. 7 Young, J. M., Jones, E., Hughes, F. A., Foley, F. E., and Fox, J. R.: Endobronchial Hamar toma, J. THORACIC SURG. 27: 300,
1954.