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Endobronchial Hamartoma
Endobronchial Hamartoma Frank T. Lansden. M.D., and Jay I,. Ankeney, M.D.
E
ndobronchial hamartomas are rare tumors since only 53 cases have been reported in the English literature to date [Z]. T h e relative rarity of the lesion and the interesting clinical sequelae of these lesions have prompted the following report of three additional cases. CASE
1
R. M., a 60-year-old white woman, presented to the emergency ward with
severe dyspnea. Seven years prior to admission she had undergone removal of the left breast for adenomyxosarcoma. She remained well until two months prior to admission when she noted the onset of progressive dyspnea. An x-ray was taken, and she was started on antibiotics for pneumonia involving the left lung. Symptoms during that illness included fever, nonproductive cough, wheezing, and intermittent substernal pain. Hemoptysis was noted two weeks prior to admission. Her symptoms abated, only to recur suddenly on the day of admission.
FIG. 1 . T h e surgical specimen from Case 1 (R. M J . The tumor completely filled the main stem bronchus and occluded the major bronchi by finger-like projections. Physical examination revealed an anxious, thin woman in moderate respiratory distress. The left breast was surgically absent. Little or no excursion of the left hemithorax could be detected. Examination of the chest revealed dullness to percussion, diminished tactile fremitus, and absence of breath sounds over the lower one-third of the left chest. The right lung was normal to examination. The trachea was shifted to the left. X-rays taken on admission revealed atelectasis of the left lower lobe which From the Thoracic Surgical Service, University Hospitals of Cleveland, and the Cleveland Veterans Administration Hospital, Cleveland, Ohio. Accepted for publication Apr. 25, 1966.
VOL.
2, NO.
6,
NOV.,
1966
845
LANSDEN AND ANKENEY progressed to total atelectasis of the left lung over the next 24 hours. Complete laboratory work-up was otherwise normal. Bronchoscopy revealed a large tumor mass obstructing the left main-stem bronchus. Biopsies taken were inconclusive for diagnosis. After complete pulmonary work-up and surgical assessment, a left pneumonectomy was performed. At surgery, a ball-valve obstruction of the left mainstem bronchus was encountered. Gross pathological examination of the specimen (Fig. 1) revealed complete obstruction of the main stem bronchus by a large endobronchial tumor with finger-like projections into the adjacent major bronchi. Microscopic examination revealed a tumor composed chiefly of cellular fibrous connective tissue with focal myxomatous change. I t contained blood vessels, fat, and clefts lined by squamous and columnar epithelium. The remaining lung was destroyed and contained tan, mucoid material in the larger bronchi. The final pathology report was returned as benign endobronchial hamartoma with resultant fibrosis, atelectasis, and bronchiectasis distal to the tumor. The patient was discharged on the twelfth postoperative day. CASE
2
P. T., a 60-year-old white male bricklayer, was admitted for evaluation of recurrent pneumonia involving the right lung. The patient stated that he had been well until two years prior to admission, when he developed chills, fever, and cough. X-rays taken at that time revealed pneumonia of the right upper lobe. He responded to treatment and remained well until one month prior to admission, when he experienced similar symptoms and had similar x-ray findings. Symptoms abated with antibiotics but recurred one week later. X-rays taken on admission revealed atelectasis of the anterior segment of the right upper lobe. Bronchoscopy revealed a polypoid tumor partially obstructing the right upper lobe bronchus. The lesion, which was inaccessible for biopsy, appeared smooth and pale yellow in color. Two days later a right upper lobectomy was performed. Upon sectioning the bronchus to the right upper lobe, a polypoid, smooth, tan endobronchial neoplasm was encountered (Fig. 2). The lung distal to the lesion was atelectatic and showed evidence of bronchiectasis and chronic inflammation. Microscopically the tumor contained fat, cartilage, fibrous tissue, and respiratory epithelium-lined clefts. The diagnosis of benign endobronchial hamartoma was thus confirmed. CASE
3
W. C., a 54-year-old white man, was seen in consultation for symptoms of progressive cough and sputum production. He stated that he had been treated for multiple bouts of pneumonia and had been hospitalized on five occasions. His cough was productive of two to three cupfuls of greenish mucoid sputum daily and was associated with moderate dyspnea. He had experienced a weight loss of 20 pounds over the past year and had been a heavy smoker for years. Physical examination on admission revealed a mild kyphoscoliosis with increased anteroposterior diameter of the chest. There were fine rales present over the lower right chest posteriorly with dullness noted in the same area. X-rays taken on admission revealed atelectasis of the right lower and middle lobes. Pulmonary function studies revealed a diminished maximum breathing capacity with predominantly left-sided function. Bronchoscopy and a bronchogram (Fig. 3) revealed a large polypoid tumor in the right main-stem bronchus. The tumor completely occluded the lower and middle lobe orifices and partially blocked the upper lobe orifice. A biopsy of the lesion was thought to represent benign polyp. One week later a right pneumonectomy was performed. 846
THE ANNALS OF THORACIC SURGERY
A
B FIG. 2. Gross ( A ) and microscopic (B) appearance of the tumor in Case 2 (P. T.). T h e tumor arose from the wall of the upper lobe bronchus, partially obstructing its lumen. Microscopic examination revealed adipose tissue, cartilage, myxomatous strorna, and respiratory epithelium. VOL. 2, NO.
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NOV.,
1966
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LANSDEN AND ANKENEY
F I G . 3. Bronchogram of Case 3 (W. C). A large polypoid mass is seen filling the intermediate bronchus and artially occluding the upper lobe orifice. T h e right lower and middle lobes cou d not be filled.
P
A
B
F I G . 4 . Gross ( A ) and microscopic ( B ) appearance of the t u m o r in Case 3 ( W . C.). T h e tumor arose from the right lower lobe bronchus by a long stalk. Microscopically it consisted of cartilage, fat, and epithelium-lined clefts surrounded by myxomatous and fibrocellular elements.
Upon sectioning the right main-stem bronchus (Fig. 4), a large, smooth, yellow endobronchial lesion was noted. It arose from a long stalk attached firmly to the right lower-lobe bronchus. All bronchi of the middle and lower lobes were filled with a mucoid, tan material which was very tenacious. The involved lobes were completely destroyed with severe bronchiectasis, atelectasis, and pneumonitis. The upper lobe was severely emphysematous and contained large bullae. The tumor consisted of a polypoid mass of adipose tissue, cartilage, and loose, fibrous connective tissue. Some myxomatous elements were present associated with epithelium-lined spaces. The pathological diagnosis was endobronchial hamartoma. The postoperative course was uncomplicated, and the patient was discharged improved without cough or sputum production.
848
THE ANNALS OE THORACIC SURGERY
CASE REPORT:
Endobronchial Hamartoma
DISCUSSION
T h e term hamartoma was introduced by Albrecht in 1904 to describe a tumor containing an abnormal mixture of tissues, all of which are present normally in the organ in which it arises. Hamartomas of the lung are solid tumors containing benign elements of fat, cartilage, and epithelium-lined spaces. They are frequently calcified and at times ossified. Fibrous connective tissue and myxomatous stroma are present in the majority of cases. These tumors differ from teratomas in that no epithelial elements foreign to the parent organ are present. Although cases of malignant degeneration have been reported [5], the tumor is considered benign. A hamartoma may arise as a faulty development of a bronchial anlage, a neoplasia of normal tissue, or hyperplasia of tissues in response to inflammation. T h e exact pathogenesis is unknown; however, most authors believe this tumor to be congenital in origin [7]. Recently Bateson [11 has suggested that both intrapulmonary and endobronchial hamartomas arise in a similar manner by metaplasia of a bronchus and that the two neoplasms differ only in their anatomical situation. In keeping with the obscure etiology and varying histological structure of the tumor, it has also been called chondroma, osteochondroma, lipochondroma, lipo-osteochondroadenoma, and bronchioma. Hamartoma of the lung is reported to occur once in every 400 patients [3]. As is true with bronchogenic carcinoma, hamartomas are found most frequently in male subjects in the fifth and sixth decades of life. In a recent Veterans Administration Cooperative Study on solitary nodules, Steele [6] reported that 10% of the pulmonary nodules resected in males above the age of 50 were hamartomas. Since hamartomas are impossible to distinguish from malignant neoplasms by nonsurgical techniques, surgical excision is indicated. Endobronchial hamartomas occur much less frequently, accounting for approximately 1% of all pulmonary hamartomas [4]. When present they often cause bronchial obstruction resulting in atelectasis, bronchiectasis, and recurrent pneumonitis. SUMMARY
Endobronchial hamartoma is a relatively rare lesion, with only 53 cases previously reported in the English literature. Its recognition is important in order to prevent the inevitable pulmonary sepsis and destruction of distal lung segments. Three additional cases are reported. REFERENCES 1. Bateson, E. M.
Relationship between intrapulmonary and endobronchial cartilage-containing tumors (so-called hemartomata). Thorax 20:447, 1965. VOL. 2, NO.
6,
NOV.,
1966
849
LANSDEN AND ANKENEY 2. Kurrus, F. D., and Conn, H. J. Endobronchial hamartoma. J. Thorac. Cardiov. Surg. 50: 138, 1965. 3. Liebow, A. A. Atlas of Tumor Pathology. Sect. 5, fasc. 17, Tumors of the Lower Respiratory Tract. Washington, D.C.: Armed Forces Institute of Pathology, 1952. 4. Shields, T. W., and Lynn, T. E. Endobronchial hamartoma (report of a case). Arch. Surg. 76:358, 1958. 5. Simon, M., and Ballon, H. An unusual hamartoma (so-called chondroma of the lung). J . Thorac. Surg. 16:379, 1947. 6. Steele, J. D. The solitary pulmonary nodule: Report of a cooperative study of resected asymptomatic solitary pulmonary nodules in males. J. Thorac. Cardiov. Surg. 46:21, 1963. 7. Womack, N. A., and Graham, E. A. Mixed tumors of the lung. Arch. Path. 26:165, 1938.
850
THE ANNALS OF THORACIC SURGERY
E
ndobronchial hamartomas are rare tumors since only 53 cases have been reported in the English literature to date [Z]. T h e relative rarity of the lesion and the interesting clinical sequelae of these lesions have prompted the following report of three additional cases. CASE
1
R. M., a 60-year-old white woman, presented to the emergency ward with
severe dyspnea. Seven years prior to admission she had undergone removal of the left breast for adenomyxosarcoma. She remained well until two months prior to admission when she noted the onset of progressive dyspnea. An x-ray was taken, and she was started on antibiotics for pneumonia involving the left lung. Symptoms during that illness included fever, nonproductive cough, wheezing, and intermittent substernal pain. Hemoptysis was noted two weeks prior to admission. Her symptoms abated, only to recur suddenly on the day of admission.
FIG. 1 . T h e surgical specimen from Case 1 (R. M J . The tumor completely filled the main stem bronchus and occluded the major bronchi by finger-like projections. Physical examination revealed an anxious, thin woman in moderate respiratory distress. The left breast was surgically absent. Little or no excursion of the left hemithorax could be detected. Examination of the chest revealed dullness to percussion, diminished tactile fremitus, and absence of breath sounds over the lower one-third of the left chest. The right lung was normal to examination. The trachea was shifted to the left. X-rays taken on admission revealed atelectasis of the left lower lobe which From the Thoracic Surgical Service, University Hospitals of Cleveland, and the Cleveland Veterans Administration Hospital, Cleveland, Ohio. Accepted for publication Apr. 25, 1966.
VOL.
2, NO.
6,
NOV.,
1966
845
LANSDEN AND ANKENEY progressed to total atelectasis of the left lung over the next 24 hours. Complete laboratory work-up was otherwise normal. Bronchoscopy revealed a large tumor mass obstructing the left main-stem bronchus. Biopsies taken were inconclusive for diagnosis. After complete pulmonary work-up and surgical assessment, a left pneumonectomy was performed. At surgery, a ball-valve obstruction of the left mainstem bronchus was encountered. Gross pathological examination of the specimen (Fig. 1) revealed complete obstruction of the main stem bronchus by a large endobronchial tumor with finger-like projections into the adjacent major bronchi. Microscopic examination revealed a tumor composed chiefly of cellular fibrous connective tissue with focal myxomatous change. I t contained blood vessels, fat, and clefts lined by squamous and columnar epithelium. The remaining lung was destroyed and contained tan, mucoid material in the larger bronchi. The final pathology report was returned as benign endobronchial hamartoma with resultant fibrosis, atelectasis, and bronchiectasis distal to the tumor. The patient was discharged on the twelfth postoperative day. CASE
2
P. T., a 60-year-old white male bricklayer, was admitted for evaluation of recurrent pneumonia involving the right lung. The patient stated that he had been well until two years prior to admission, when he developed chills, fever, and cough. X-rays taken at that time revealed pneumonia of the right upper lobe. He responded to treatment and remained well until one month prior to admission, when he experienced similar symptoms and had similar x-ray findings. Symptoms abated with antibiotics but recurred one week later. X-rays taken on admission revealed atelectasis of the anterior segment of the right upper lobe. Bronchoscopy revealed a polypoid tumor partially obstructing the right upper lobe bronchus. The lesion, which was inaccessible for biopsy, appeared smooth and pale yellow in color. Two days later a right upper lobectomy was performed. Upon sectioning the bronchus to the right upper lobe, a polypoid, smooth, tan endobronchial neoplasm was encountered (Fig. 2). The lung distal to the lesion was atelectatic and showed evidence of bronchiectasis and chronic inflammation. Microscopically the tumor contained fat, cartilage, fibrous tissue, and respiratory epithelium-lined clefts. The diagnosis of benign endobronchial hamartoma was thus confirmed. CASE
3
W. C., a 54-year-old white man, was seen in consultation for symptoms of progressive cough and sputum production. He stated that he had been treated for multiple bouts of pneumonia and had been hospitalized on five occasions. His cough was productive of two to three cupfuls of greenish mucoid sputum daily and was associated with moderate dyspnea. He had experienced a weight loss of 20 pounds over the past year and had been a heavy smoker for years. Physical examination on admission revealed a mild kyphoscoliosis with increased anteroposterior diameter of the chest. There were fine rales present over the lower right chest posteriorly with dullness noted in the same area. X-rays taken on admission revealed atelectasis of the right lower and middle lobes. Pulmonary function studies revealed a diminished maximum breathing capacity with predominantly left-sided function. Bronchoscopy and a bronchogram (Fig. 3) revealed a large polypoid tumor in the right main-stem bronchus. The tumor completely occluded the lower and middle lobe orifices and partially blocked the upper lobe orifice. A biopsy of the lesion was thought to represent benign polyp. One week later a right pneumonectomy was performed. 846
THE ANNALS OF THORACIC SURGERY
A
B FIG. 2. Gross ( A ) and microscopic (B) appearance of the tumor in Case 2 (P. T.). T h e tumor arose from the wall of the upper lobe bronchus, partially obstructing its lumen. Microscopic examination revealed adipose tissue, cartilage, myxomatous strorna, and respiratory epithelium. VOL. 2, NO.
6,
NOV.,
1966
847
LANSDEN AND ANKENEY
F I G . 3. Bronchogram of Case 3 (W. C). A large polypoid mass is seen filling the intermediate bronchus and artially occluding the upper lobe orifice. T h e right lower and middle lobes cou d not be filled.
P
A
B
F I G . 4 . Gross ( A ) and microscopic ( B ) appearance of the t u m o r in Case 3 ( W . C.). T h e tumor arose from the right lower lobe bronchus by a long stalk. Microscopically it consisted of cartilage, fat, and epithelium-lined clefts surrounded by myxomatous and fibrocellular elements.
Upon sectioning the right main-stem bronchus (Fig. 4), a large, smooth, yellow endobronchial lesion was noted. It arose from a long stalk attached firmly to the right lower-lobe bronchus. All bronchi of the middle and lower lobes were filled with a mucoid, tan material which was very tenacious. The involved lobes were completely destroyed with severe bronchiectasis, atelectasis, and pneumonitis. The upper lobe was severely emphysematous and contained large bullae. The tumor consisted of a polypoid mass of adipose tissue, cartilage, and loose, fibrous connective tissue. Some myxomatous elements were present associated with epithelium-lined spaces. The pathological diagnosis was endobronchial hamartoma. The postoperative course was uncomplicated, and the patient was discharged improved without cough or sputum production.
848
THE ANNALS OE THORACIC SURGERY
CASE REPORT:
Endobronchial Hamartoma
DISCUSSION
T h e term hamartoma was introduced by Albrecht in 1904 to describe a tumor containing an abnormal mixture of tissues, all of which are present normally in the organ in which it arises. Hamartomas of the lung are solid tumors containing benign elements of fat, cartilage, and epithelium-lined spaces. They are frequently calcified and at times ossified. Fibrous connective tissue and myxomatous stroma are present in the majority of cases. These tumors differ from teratomas in that no epithelial elements foreign to the parent organ are present. Although cases of malignant degeneration have been reported [5], the tumor is considered benign. A hamartoma may arise as a faulty development of a bronchial anlage, a neoplasia of normal tissue, or hyperplasia of tissues in response to inflammation. T h e exact pathogenesis is unknown; however, most authors believe this tumor to be congenital in origin [7]. Recently Bateson [11 has suggested that both intrapulmonary and endobronchial hamartomas arise in a similar manner by metaplasia of a bronchus and that the two neoplasms differ only in their anatomical situation. In keeping with the obscure etiology and varying histological structure of the tumor, it has also been called chondroma, osteochondroma, lipochondroma, lipo-osteochondroadenoma, and bronchioma. Hamartoma of the lung is reported to occur once in every 400 patients [3]. As is true with bronchogenic carcinoma, hamartomas are found most frequently in male subjects in the fifth and sixth decades of life. In a recent Veterans Administration Cooperative Study on solitary nodules, Steele [6] reported that 10% of the pulmonary nodules resected in males above the age of 50 were hamartomas. Since hamartomas are impossible to distinguish from malignant neoplasms by nonsurgical techniques, surgical excision is indicated. Endobronchial hamartomas occur much less frequently, accounting for approximately 1% of all pulmonary hamartomas [4]. When present they often cause bronchial obstruction resulting in atelectasis, bronchiectasis, and recurrent pneumonitis. SUMMARY
Endobronchial hamartoma is a relatively rare lesion, with only 53 cases previously reported in the English literature. Its recognition is important in order to prevent the inevitable pulmonary sepsis and destruction of distal lung segments. Three additional cases are reported. REFERENCES 1. Bateson, E. M.
Relationship between intrapulmonary and endobronchial cartilage-containing tumors (so-called hemartomata). Thorax 20:447, 1965. VOL. 2, NO.
6,
NOV.,
1966
849
LANSDEN AND ANKENEY 2. Kurrus, F. D., and Conn, H. J. Endobronchial hamartoma. J. Thorac. Cardiov. Surg. 50: 138, 1965. 3. Liebow, A. A. Atlas of Tumor Pathology. Sect. 5, fasc. 17, Tumors of the Lower Respiratory Tract. Washington, D.C.: Armed Forces Institute of Pathology, 1952. 4. Shields, T. W., and Lynn, T. E. Endobronchial hamartoma (report of a case). Arch. Surg. 76:358, 1958. 5. Simon, M., and Ballon, H. An unusual hamartoma (so-called chondroma of the lung). J . Thorac. Surg. 16:379, 1947. 6. Steele, J. D. The solitary pulmonary nodule: Report of a cooperative study of resected asymptomatic solitary pulmonary nodules in males. J. Thorac. Cardiov. Surg. 46:21, 1963. 7. Womack, N. A., and Graham, E. A. Mixed tumors of the lung. Arch. Path. 26:165, 1938.
850
THE ANNALS OF THORACIC SURGERY