- Email: [email protected]
Endobronchial Hamartoma
Endobronchial Hamartoma* Borja G. Cosı´o, MD; Victoria Villena, MD; Jose Echave-Sustaeta, MD; Eduardo de Miguel, MD; Jose Alfaro, MD; Luis Hernandez, MD; and Teresa Sotelo, MD
Objectives: To describe clinical, endoscopic, radiographic, and follow-up characteristics of a series of patients in whom endobronchial hamartoma (EH) had been diagnosed. Methods: Retrospective study of all cases of hamartoma diagnosed by bronchial biopsy between 1974 and 1997 in a tertiary referral hospital in Madrid, Spain. Results: EH was diagnosed 47 patients during the study period. Four patients were excluded from the study because no clinical history was available. We analyzed the cases of 43 patients (37 men and 6 women), with a mean (ⴞ SD) age of 62 ⴞ 12 years. Seven patients had a concurrent lung neoplasm, and the EH was an incidental endoscopic finding. Among the other 36 patients, 31 had a new onset of respiratory symptoms, most commonly, recurrent respiratory infections in 16 patients (44%) and hemoptysis in a further 12 patients (33.4%). Chest radiograph findings were abnormal in 38 of 43 patients. At bronchoscopy, the lesions were equally distributed throughout the right and left lungs with no clear lobar predilection. Endobronchial obstruction was evident in 26 patients (72.2%) without concurrent neoplasm, 17 of whom underwent resection with a rigid bronchoscope and laser, with total resolution in 13 patients. Partial resolution was achieved in four patients, two of whom needed a second endoscopic procedure. Five patients were treated with open lung surgery. Clinical and endoscopic follow-up was performed in 23 patients at 1 to 73 months (mean, 17 months), and recurrence was found in 4 patients. Conclusion: EH frequently produces respiratory complaints and radiographic abnormalities. Patients with endobronchial obstructions had satisfactory responses to endoscopic therapy. (CHEST 2002; 122:202–205) Key words: benign lung neoplasm; endobronchial; hamartoma Abbreviation: EH ⫽ endobronchial hamartoma
hamartomas was coined by Albrecht in T he1904termto describe tumor-like malformations result1
ing from a presumptive developmental abnormality. In 1934, Goldsworthy2 applied this term to benign tumors located in the lung that were composed predominantly of a combination of fat and cartilage. Other authors have proposed that the hamartoma represents a true neoplasm instead of a developmental abnormality.3 Cytogenetic studies have identified chromosomal bands of recombination located at positions 6p21 and 14q24, supporting the idea that hamartomas represent mesenchymal clonal neoplasms.4 Lung hamartomas are the most common form of *From the Respiratory Department (Drs. Cosı´o, Villena, EchaveSustaeta, de Miguel, Alfaro, and Hernandez) and the Pathology Department (Dr. Sotelo), Hospital 12 de Octubre, Madrid, Spain. Presented at the European Respiratory Society Congress in Madrid, October 1999. Manuscript received November 14, 2000; revision accepted December 24, 2001. Correspondence to: B. G. Cosı´o, MD, Servicio de Neumologı´a, Hospital 12 de Octubre, Cra De Andalucı´a Km 5,400, 28041 Madrid, Spain; e-mail: [email protected] 202
benign lung tumors, with an incidence between 0.025% and 0.32%5,6 according to different necropsy studies. In large clinical series of patients with lung hamartomas, most patients are asymptomatic at the time of diagnosis, and the hamartoma is a radiographic finding. Endobronchial hamartomas (EHs) have a low frequency in all these studies, and their characteristics are poorly described. In the largest review series that has been published (n ⫽ 215),7 only 1.4% of hamartomas had an endobronchial location, the remainder being located within the parenchyma. Other series estimate the incidence of EHs between 10% and 20% of all lung hamartomas.8 –10 This is the first review specifically addressing the characteristics of EHs. The aim of this study is to analyze the clinical, radiographic, and endoscopic characteristics of the EHs, and to discuss the outcome of treated cases in this the largest series described. Materials and Methods The Bronchoscopy Unit of the 12 de Octubre Hospital, a tertiary hospital with 1,800 beds, performed about 31,000 bronClinical Investigations
choscopy procedures between 1974 and 1997. Since 1980, this hospital has served as a referral center for endoscopic therapy of endobronchial lesions by rigid bronchoscope and laser therapy. In this study, we retrospectively analyzed the cases of all patients with bronchial biopsy-proven EH during a period of 23 years. The Bronchoscopy Unit database was analyzed in order to identify the patients in whom EHs had been diagnosed by bronchial biopsy. Pathology department files were reviewed to confirm the diagnosis. Once the patients were identified and the diagnosis was confirmed, the clinical history, radiology findings, and endoscopic follow-up procedures were reviewed. The following variables were studied: age at diagnosis; gender; symptoms at diagnosis; indication for bronchoscopy; medical history with special emphasis on previous or concurrent neoplasm; radiographic abnormalities; endobronchial location of the hamartomas; endoscopic appearance; pathologic characteristics; grade of bronchial obstruction; therapy, if any; clinical and endoscopic follow-up; and recurrence. The indication for therapy was based on the presence of severe symptoms such as hemoptysis or recurrent obstructive pneumonia. The selection of therapy depended on the size and site of the obstruction and the technical applicability of each technique. Surgery has not been performed in the management of EH in this center since 1980 due to the highly successful outcomes achieved with bronchoscopy. The rigid bronchoscopy procedures included mechanical resection of the hamartoma and the application of an Nd-YAG laser to its borders when necessary due to insufficient resection or excessive bleeding. In the evaluation of the endoscopic therapy, the reestablishment of the bronchial lumen to ⬎ 80% of the original lumen diameter with radiologic resolution was considered to be successful therapy. In all cases, successful endobronchial therapy was accompanied by radiologic resolution as well. Partial success was considered when the bronchial lumen was reestablished between 50% and 80% without complete radiologic resolution. Recurrence was considered to have occurred when growth of the lesion was observed with progression of the obstruction in ⬎ 20% of the previously reported lumen diameter.
Table 1—Indication for Bronchoscopy Indication
No. of Patients (%)
Recurrent pneumonia Hemoptysis Radiographic abnormalities Volume loss Others Persistent cough Endoscopic finding in a review procedure
16 (37.2) 10 (23.3) 5 (11.6) 10 (23.3) 1 (2.3) 1 (2.3)
tree are shown in Figure 1. There was not a predominant location for EHs in our series. Endoscopic features that suggested the presence of a hamartoma were the presence of an exophytic or polypoidal mass with a smooth, well-limited surface, and without signs of submucosal infiltration (Fig 2). At biopsy, spongiform consistency was always described. The endoscopic appearance of the lesion later diagnosed as an hamartoma was described as suggestive of benign neoplasm in all 43 cases. The histologic pattern most frequently described was that of chondroid hamartoma in 16 cases (37.2%) and lipoid hamartoma in 13 cases (30.2%). A lipoid hamartoma is shown in detail in Figure 3. In the other 14 samples, no predominant cellular component was identified.
Results During the study period, 47 EHs were diagnosed in the Hospital 12 de Octubre. Four patients were excluded because their clinical histories were not available. Forty-three patients were studied (37 men and 6 women), with a mean (⫾ SD) age of 62 ⫾ 12 years (range, 29 to 81 years). Only nine patients were asymptomatic at the time of diagnosis. On presentation, the most frequent clinical symptoms were recurrent respiratory infections or obstructive pneumonia in 16 patients (37%) and hemoptysis in 14 patients (32%), with or without other respiratory complaints such as cough or dyspnea. Indications for bronchoscopy (Table 1) in these patients can be classified into the following three groups: (1) bronchial symptoms such as persistent cough or hemoptysis; (2) radiographic abnormalities such as volume loss, solitary pulmonary nodule, interstitial pattern, or recurrent alveolar infiltrates; and (3) recurrent pneumonia. The locations of the EHs in the tracheobronchial www.chestjournal.org
Figure 1. Distribution of the 43 EHs within the tracheobronchial tree as follows: trachea, 4; right main bronchus, 2; left main bronchus, 6; intermediate bronchus, 3; middle lobe bronchus, 3; right upper lobe bronchus, 8; left upper lobe bronchus, 8; left upper lobe bronchus, 3; right lower lobe bronchus, 3; and left lower lobe bronchus, 3. CHEST / 122 / 1 / JULY, 2002
203
Table 2—Symptoms at Diagnosis
Figure 2. Endoscopic appearance of EH.
In seven patients, the EH was an endoscopic finding during the assessment of a concurrent lung neoplasm (squamous carcinoma, three patients; adenocarcinoma, one patient; large cell carcinoma, one patient; oat cell carcinoma, one patient; and lymphoma, one patient). In only one case was the hamartoma in the same lung as the neoplasm, albeit in a different lobe. Of the 36 patients without concurrent lung neoplasms, 31 (86.1%) had the onset of new respiratory complaints at the time of diagnosis. The most common complaint, recurrent respiratory infections, was present in 16 patients (44.4%), while hemoptysis was present in 12 patients (33.4%) [Table 2]. Chest radiography findings for this group were normal in just five patients (13.9%). The radiographic abnormalities most frequently observed were alveolar infiltrates (11 patients; 30.6%), atelectasis or volume loss (9 patients; 25%), or both (8 patients; 22.2%). Other radiographic patterns were observed in three patients (8.3%) in association with non-neoplastic
Clinical Presentation
No. of Patients (%)
Obstructive pneumonia Hemoptysis Cough and hemoptysis Cough Dyspnea and hemoptysis Dyspnea Asymptomatic
16 (44.4) 6 (16.7) 4 (11.1) 2 (5.6) 2 (5.6) 1 (2.8) 5 (13.9)
parenchymal disease (benign solitary pulmonary nodules, two patients; and interstitial disease, one patient). EHs produced a degree of bronchial obstruction in 26 of the 36 patients with no concurrent tumoral lung disease related to a tumor (tracheal obstruction, 1 patient; main bronchus obstruction, 5 patients; lobar bronchus obstruction, 10 patients; and segmental bronchus obstruction, 10 patients). Of the 36 patients with EHs but no concurrent malignant neoplasms, 24 underwent treatment that was aimed at eliminating the EH (Table 3). The therapeutic method most frequently used was rigid bronchoscopy with or without laser application. Five patients were treated surgically, with three undergoing bronchotomies and two undergoing lobectomies. Endoscopic reevaluation was performed in 22 of the 24 patients that were treated. The treatment was considered to be successful in 18 patients (81.8%), and partially successful in 4 patients (18.2%). Of the 17 patients treated with rigid bronchoscopy, the treatment was considered to be successful in 13 and partially successful in 4. There was an endoscopic follow-up in 15 of the 17 patients treated by this method, during a mean period of 16.2 months (range, 1 to 72 months). In this group, recurrence was observed in four patients, and a second endoscopic procedure was performed in three of them. Discussion The endobronchial location of the benign hamartoma is a rare occurrence. In a 1950 study of lung carcinoma, bronchial adenoma, and lung hamartoma, Le Roux11 found 3,000 patients with lung
Table 3—Management of EHs Treatment
Figure 3. Lipoid hamartoma. (hematoxylin-eosin, original ⫻400). Components shown in detail are adipoid and mesenchymal tissue. 204
Rigid bronchoscopy and laser Thoracotomy Fiberoptic bronchoscopy with forceps resection No treatment
No. of Patients (%) 17 (47.2) 5 (13.9) 2 (5.6) 12 (33.3) Clinical Investigations
carcinomas, 40 with bronchial adenomas, and 27 with lung hamartomas, of which only 8% were EHs. In the largest published series of pulmonary hamartomas, Gjevre et al7 analyzed 215 cases of hamartoma, of which only 1.4% were located endobronchially; the authors concluded that the hamartoma was a benign neoplasm and frequently asymptomatic. Our retrospective series demonstrates that EHs, in comparison with previously described parenchymal hamartomas, are frequently symptomatic, and for this reason treatment is frequently indicated. The mean age of the patient at presentation was between the sixth and seventh decades, as has been found in previous series,7 with male predominance. In the population of the study by Gjevre et al,7 most of the patients were asymptomatic, and the hamartoma was an incidental finding. In our series, 80% of the patients had at least one respiratory complaint. The clinical features most frequently found were those of hemoptysis and obstructive pneumonia. This likely can be explained by the fact that we are a reference center for endoscopic therapy in Spain and, surely, we are not seeing the patients without severe clinical manifestations. The high incidence of radiographic abnormalities is similar to those in previous reports.9 We found no cases of multiple hamartoma, and no association between parenchymal hamartoma and EH, as previously has been reported.12 Lung hamartomas are benign neoplasms with very low risk of malignancy and a low rate of recurrence. We have not found any case of malignancy, although we have observed the growth of benign hamartomas in four patients during follow-up, three of whom had to undergo a second endoscopic procedure. Although there was concurrent tumor-related lung disease in seven patients, we could not find any association between hamartoma and tumor location. The management of EHs must be individualized according to the characteristics of each patient and each hamartoma. In our experience, endoscopic treatment with rigid bronchoscopy and laser is a good therapeutic choice in the symptomatic patient or in the patient with serious radiographic abnormalities. This technique leads to the control of symptoms and might avoid the risk of thoracotomy.13
www.chestjournal.org
In conclusion, EHs are benign neoplasms of the tracheobronchial tree that potentially can be dangerous due to bronchial obstruction or bleeding. Endoscopic treatment with rigid bronchoscopy and lasers provides an excellent outcome when performed by expert hands. Therefore, such treatment should be recommended in highly symptomatic patients or in those patients with significant bronchial obstructions. ACKNOWLEDGMENT: The authors gratefully acknowledge Dr. Robert Stirling for his help in editing this manuscript.
References 1 Albrecht E. Ueber hamartome. Verh Dtsch Ges Pathol 1904; 7:153–157 2 Goldsworthy NE. Chondroma of lung (hamartoma chondromatosum polmonis), with report of case. J Pathol Bacteriol 1934; 39:291–298 3 Bateson EM. So-called hamartoma of the lung-a true neoplasm of fibrous connective tissue of the bronchi. Cancer 1973; 31:1458 –1467 4 Fletcher JA, Pinkus GS, Donovan K, et al. Clonal rearrangement of chromosome band 6p21 in the mesenchymal component of pulmonary chondroid hamartoma. Cancer Res 1992; 52:6224 – 6228 5 McDonald JR, Harrington SW, Clagett OT. Hamartoma (often called chondroma) of the lung. J Thorac Surg 1945; 14:128 –143 6 Murray J, Kielkowski D, Leiman G. The prevalence and age distribution of peripheral pulmonary hamartoma in adult males: an autopsy based study. S Afr Med J 1991; 79:247–249 7 Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996; 71:14 –20 8 Sibala JL. Endobronchial hamartomas. Chest 1972; 62:631– 634 9 Tomashefski JF. Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas. Am J Pathol 1982; 6:531–540 10 Van den Bosch JM, Wagenaar SS, Corrin B, et al. Mesenchymoma of the lung (so called hamartoma): a review of 154 parenchymal and endobronchial cases. Thorax 1987; 42:790 – 793 11 Le Roux BT. Pulmonary hamartoma. Thorax 1964; 19:236 – 243 12 Mompoint D, Groussard O, Grenier P. Endobronchial hamartoma associated with bronchioloalveolar cell carcinoma. Chest 1988; 94:1094 –1096 13 Sahin AA, Aydiner A, Kalyoncu F, et al. Endobronchial hamartoma removed by rigid bronchoscope. Eur Respir J 1989; 2:479 – 480
CHEST / 122 / 1 / JULY, 2002
205
Objectives: To describe clinical, endoscopic, radiographic, and follow-up characteristics of a series of patients in whom endobronchial hamartoma (EH) had been diagnosed. Methods: Retrospective study of all cases of hamartoma diagnosed by bronchial biopsy between 1974 and 1997 in a tertiary referral hospital in Madrid, Spain. Results: EH was diagnosed 47 patients during the study period. Four patients were excluded from the study because no clinical history was available. We analyzed the cases of 43 patients (37 men and 6 women), with a mean (ⴞ SD) age of 62 ⴞ 12 years. Seven patients had a concurrent lung neoplasm, and the EH was an incidental endoscopic finding. Among the other 36 patients, 31 had a new onset of respiratory symptoms, most commonly, recurrent respiratory infections in 16 patients (44%) and hemoptysis in a further 12 patients (33.4%). Chest radiograph findings were abnormal in 38 of 43 patients. At bronchoscopy, the lesions were equally distributed throughout the right and left lungs with no clear lobar predilection. Endobronchial obstruction was evident in 26 patients (72.2%) without concurrent neoplasm, 17 of whom underwent resection with a rigid bronchoscope and laser, with total resolution in 13 patients. Partial resolution was achieved in four patients, two of whom needed a second endoscopic procedure. Five patients were treated with open lung surgery. Clinical and endoscopic follow-up was performed in 23 patients at 1 to 73 months (mean, 17 months), and recurrence was found in 4 patients. Conclusion: EH frequently produces respiratory complaints and radiographic abnormalities. Patients with endobronchial obstructions had satisfactory responses to endoscopic therapy. (CHEST 2002; 122:202–205) Key words: benign lung neoplasm; endobronchial; hamartoma Abbreviation: EH ⫽ endobronchial hamartoma
hamartomas was coined by Albrecht in T he1904termto describe tumor-like malformations result1
ing from a presumptive developmental abnormality. In 1934, Goldsworthy2 applied this term to benign tumors located in the lung that were composed predominantly of a combination of fat and cartilage. Other authors have proposed that the hamartoma represents a true neoplasm instead of a developmental abnormality.3 Cytogenetic studies have identified chromosomal bands of recombination located at positions 6p21 and 14q24, supporting the idea that hamartomas represent mesenchymal clonal neoplasms.4 Lung hamartomas are the most common form of *From the Respiratory Department (Drs. Cosı´o, Villena, EchaveSustaeta, de Miguel, Alfaro, and Hernandez) and the Pathology Department (Dr. Sotelo), Hospital 12 de Octubre, Madrid, Spain. Presented at the European Respiratory Society Congress in Madrid, October 1999. Manuscript received November 14, 2000; revision accepted December 24, 2001. Correspondence to: B. G. Cosı´o, MD, Servicio de Neumologı´a, Hospital 12 de Octubre, Cra De Andalucı´a Km 5,400, 28041 Madrid, Spain; e-mail: [email protected] 202
benign lung tumors, with an incidence between 0.025% and 0.32%5,6 according to different necropsy studies. In large clinical series of patients with lung hamartomas, most patients are asymptomatic at the time of diagnosis, and the hamartoma is a radiographic finding. Endobronchial hamartomas (EHs) have a low frequency in all these studies, and their characteristics are poorly described. In the largest review series that has been published (n ⫽ 215),7 only 1.4% of hamartomas had an endobronchial location, the remainder being located within the parenchyma. Other series estimate the incidence of EHs between 10% and 20% of all lung hamartomas.8 –10 This is the first review specifically addressing the characteristics of EHs. The aim of this study is to analyze the clinical, radiographic, and endoscopic characteristics of the EHs, and to discuss the outcome of treated cases in this the largest series described. Materials and Methods The Bronchoscopy Unit of the 12 de Octubre Hospital, a tertiary hospital with 1,800 beds, performed about 31,000 bronClinical Investigations
choscopy procedures between 1974 and 1997. Since 1980, this hospital has served as a referral center for endoscopic therapy of endobronchial lesions by rigid bronchoscope and laser therapy. In this study, we retrospectively analyzed the cases of all patients with bronchial biopsy-proven EH during a period of 23 years. The Bronchoscopy Unit database was analyzed in order to identify the patients in whom EHs had been diagnosed by bronchial biopsy. Pathology department files were reviewed to confirm the diagnosis. Once the patients were identified and the diagnosis was confirmed, the clinical history, radiology findings, and endoscopic follow-up procedures were reviewed. The following variables were studied: age at diagnosis; gender; symptoms at diagnosis; indication for bronchoscopy; medical history with special emphasis on previous or concurrent neoplasm; radiographic abnormalities; endobronchial location of the hamartomas; endoscopic appearance; pathologic characteristics; grade of bronchial obstruction; therapy, if any; clinical and endoscopic follow-up; and recurrence. The indication for therapy was based on the presence of severe symptoms such as hemoptysis or recurrent obstructive pneumonia. The selection of therapy depended on the size and site of the obstruction and the technical applicability of each technique. Surgery has not been performed in the management of EH in this center since 1980 due to the highly successful outcomes achieved with bronchoscopy. The rigid bronchoscopy procedures included mechanical resection of the hamartoma and the application of an Nd-YAG laser to its borders when necessary due to insufficient resection or excessive bleeding. In the evaluation of the endoscopic therapy, the reestablishment of the bronchial lumen to ⬎ 80% of the original lumen diameter with radiologic resolution was considered to be successful therapy. In all cases, successful endobronchial therapy was accompanied by radiologic resolution as well. Partial success was considered when the bronchial lumen was reestablished between 50% and 80% without complete radiologic resolution. Recurrence was considered to have occurred when growth of the lesion was observed with progression of the obstruction in ⬎ 20% of the previously reported lumen diameter.
Table 1—Indication for Bronchoscopy Indication
No. of Patients (%)
Recurrent pneumonia Hemoptysis Radiographic abnormalities Volume loss Others Persistent cough Endoscopic finding in a review procedure
16 (37.2) 10 (23.3) 5 (11.6) 10 (23.3) 1 (2.3) 1 (2.3)
tree are shown in Figure 1. There was not a predominant location for EHs in our series. Endoscopic features that suggested the presence of a hamartoma were the presence of an exophytic or polypoidal mass with a smooth, well-limited surface, and without signs of submucosal infiltration (Fig 2). At biopsy, spongiform consistency was always described. The endoscopic appearance of the lesion later diagnosed as an hamartoma was described as suggestive of benign neoplasm in all 43 cases. The histologic pattern most frequently described was that of chondroid hamartoma in 16 cases (37.2%) and lipoid hamartoma in 13 cases (30.2%). A lipoid hamartoma is shown in detail in Figure 3. In the other 14 samples, no predominant cellular component was identified.
Results During the study period, 47 EHs were diagnosed in the Hospital 12 de Octubre. Four patients were excluded because their clinical histories were not available. Forty-three patients were studied (37 men and 6 women), with a mean (⫾ SD) age of 62 ⫾ 12 years (range, 29 to 81 years). Only nine patients were asymptomatic at the time of diagnosis. On presentation, the most frequent clinical symptoms were recurrent respiratory infections or obstructive pneumonia in 16 patients (37%) and hemoptysis in 14 patients (32%), with or without other respiratory complaints such as cough or dyspnea. Indications for bronchoscopy (Table 1) in these patients can be classified into the following three groups: (1) bronchial symptoms such as persistent cough or hemoptysis; (2) radiographic abnormalities such as volume loss, solitary pulmonary nodule, interstitial pattern, or recurrent alveolar infiltrates; and (3) recurrent pneumonia. The locations of the EHs in the tracheobronchial www.chestjournal.org
Figure 1. Distribution of the 43 EHs within the tracheobronchial tree as follows: trachea, 4; right main bronchus, 2; left main bronchus, 6; intermediate bronchus, 3; middle lobe bronchus, 3; right upper lobe bronchus, 8; left upper lobe bronchus, 8; left upper lobe bronchus, 3; right lower lobe bronchus, 3; and left lower lobe bronchus, 3. CHEST / 122 / 1 / JULY, 2002
203
Table 2—Symptoms at Diagnosis
Figure 2. Endoscopic appearance of EH.
In seven patients, the EH was an endoscopic finding during the assessment of a concurrent lung neoplasm (squamous carcinoma, three patients; adenocarcinoma, one patient; large cell carcinoma, one patient; oat cell carcinoma, one patient; and lymphoma, one patient). In only one case was the hamartoma in the same lung as the neoplasm, albeit in a different lobe. Of the 36 patients without concurrent lung neoplasms, 31 (86.1%) had the onset of new respiratory complaints at the time of diagnosis. The most common complaint, recurrent respiratory infections, was present in 16 patients (44.4%), while hemoptysis was present in 12 patients (33.4%) [Table 2]. Chest radiography findings for this group were normal in just five patients (13.9%). The radiographic abnormalities most frequently observed were alveolar infiltrates (11 patients; 30.6%), atelectasis or volume loss (9 patients; 25%), or both (8 patients; 22.2%). Other radiographic patterns were observed in three patients (8.3%) in association with non-neoplastic
Clinical Presentation
No. of Patients (%)
Obstructive pneumonia Hemoptysis Cough and hemoptysis Cough Dyspnea and hemoptysis Dyspnea Asymptomatic
16 (44.4) 6 (16.7) 4 (11.1) 2 (5.6) 2 (5.6) 1 (2.8) 5 (13.9)
parenchymal disease (benign solitary pulmonary nodules, two patients; and interstitial disease, one patient). EHs produced a degree of bronchial obstruction in 26 of the 36 patients with no concurrent tumoral lung disease related to a tumor (tracheal obstruction, 1 patient; main bronchus obstruction, 5 patients; lobar bronchus obstruction, 10 patients; and segmental bronchus obstruction, 10 patients). Of the 36 patients with EHs but no concurrent malignant neoplasms, 24 underwent treatment that was aimed at eliminating the EH (Table 3). The therapeutic method most frequently used was rigid bronchoscopy with or without laser application. Five patients were treated surgically, with three undergoing bronchotomies and two undergoing lobectomies. Endoscopic reevaluation was performed in 22 of the 24 patients that were treated. The treatment was considered to be successful in 18 patients (81.8%), and partially successful in 4 patients (18.2%). Of the 17 patients treated with rigid bronchoscopy, the treatment was considered to be successful in 13 and partially successful in 4. There was an endoscopic follow-up in 15 of the 17 patients treated by this method, during a mean period of 16.2 months (range, 1 to 72 months). In this group, recurrence was observed in four patients, and a second endoscopic procedure was performed in three of them. Discussion The endobronchial location of the benign hamartoma is a rare occurrence. In a 1950 study of lung carcinoma, bronchial adenoma, and lung hamartoma, Le Roux11 found 3,000 patients with lung
Table 3—Management of EHs Treatment
Figure 3. Lipoid hamartoma. (hematoxylin-eosin, original ⫻400). Components shown in detail are adipoid and mesenchymal tissue. 204
Rigid bronchoscopy and laser Thoracotomy Fiberoptic bronchoscopy with forceps resection No treatment
No. of Patients (%) 17 (47.2) 5 (13.9) 2 (5.6) 12 (33.3) Clinical Investigations
carcinomas, 40 with bronchial adenomas, and 27 with lung hamartomas, of which only 8% were EHs. In the largest published series of pulmonary hamartomas, Gjevre et al7 analyzed 215 cases of hamartoma, of which only 1.4% were located endobronchially; the authors concluded that the hamartoma was a benign neoplasm and frequently asymptomatic. Our retrospective series demonstrates that EHs, in comparison with previously described parenchymal hamartomas, are frequently symptomatic, and for this reason treatment is frequently indicated. The mean age of the patient at presentation was between the sixth and seventh decades, as has been found in previous series,7 with male predominance. In the population of the study by Gjevre et al,7 most of the patients were asymptomatic, and the hamartoma was an incidental finding. In our series, 80% of the patients had at least one respiratory complaint. The clinical features most frequently found were those of hemoptysis and obstructive pneumonia. This likely can be explained by the fact that we are a reference center for endoscopic therapy in Spain and, surely, we are not seeing the patients without severe clinical manifestations. The high incidence of radiographic abnormalities is similar to those in previous reports.9 We found no cases of multiple hamartoma, and no association between parenchymal hamartoma and EH, as previously has been reported.12 Lung hamartomas are benign neoplasms with very low risk of malignancy and a low rate of recurrence. We have not found any case of malignancy, although we have observed the growth of benign hamartomas in four patients during follow-up, three of whom had to undergo a second endoscopic procedure. Although there was concurrent tumor-related lung disease in seven patients, we could not find any association between hamartoma and tumor location. The management of EHs must be individualized according to the characteristics of each patient and each hamartoma. In our experience, endoscopic treatment with rigid bronchoscopy and laser is a good therapeutic choice in the symptomatic patient or in the patient with serious radiographic abnormalities. This technique leads to the control of symptoms and might avoid the risk of thoracotomy.13
www.chestjournal.org
In conclusion, EHs are benign neoplasms of the tracheobronchial tree that potentially can be dangerous due to bronchial obstruction or bleeding. Endoscopic treatment with rigid bronchoscopy and lasers provides an excellent outcome when performed by expert hands. Therefore, such treatment should be recommended in highly symptomatic patients or in those patients with significant bronchial obstructions. ACKNOWLEDGMENT: The authors gratefully acknowledge Dr. Robert Stirling for his help in editing this manuscript.
References 1 Albrecht E. Ueber hamartome. Verh Dtsch Ges Pathol 1904; 7:153–157 2 Goldsworthy NE. Chondroma of lung (hamartoma chondromatosum polmonis), with report of case. J Pathol Bacteriol 1934; 39:291–298 3 Bateson EM. So-called hamartoma of the lung-a true neoplasm of fibrous connective tissue of the bronchi. Cancer 1973; 31:1458 –1467 4 Fletcher JA, Pinkus GS, Donovan K, et al. Clonal rearrangement of chromosome band 6p21 in the mesenchymal component of pulmonary chondroid hamartoma. Cancer Res 1992; 52:6224 – 6228 5 McDonald JR, Harrington SW, Clagett OT. Hamartoma (often called chondroma) of the lung. J Thorac Surg 1945; 14:128 –143 6 Murray J, Kielkowski D, Leiman G. The prevalence and age distribution of peripheral pulmonary hamartoma in adult males: an autopsy based study. S Afr Med J 1991; 79:247–249 7 Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996; 71:14 –20 8 Sibala JL. Endobronchial hamartomas. Chest 1972; 62:631– 634 9 Tomashefski JF. Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas. Am J Pathol 1982; 6:531–540 10 Van den Bosch JM, Wagenaar SS, Corrin B, et al. Mesenchymoma of the lung (so called hamartoma): a review of 154 parenchymal and endobronchial cases. Thorax 1987; 42:790 – 793 11 Le Roux BT. Pulmonary hamartoma. Thorax 1964; 19:236 – 243 12 Mompoint D, Groussard O, Grenier P. Endobronchial hamartoma associated with bronchioloalveolar cell carcinoma. Chest 1988; 94:1094 –1096 13 Sahin AA, Aydiner A, Kalyoncu F, et al. Endobronchial hamartoma removed by rigid bronchoscope. Eur Respir J 1989; 2:479 – 480
CHEST / 122 / 1 / JULY, 2002
205