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Surgical management of pulmonary metastatic leiomyosarcoma with gross endobronchial extension
Surgical Management of Pulmonary Metastatic Leiomyosarcoma With Gross Endobronchial Extension William H. Warren, MD, Phyllis Bleck, MD, C. Frederick Kittle, MD, and L. Penfield Faber, MD Department of Cardiovascular-ThoracicSurgery, Rush-Presbyterian-St. Luke’s Medical Center, Chicago, Illinois
Metastatic leiomyosarcoma occasionally is seen with gross endobronchial extension without invasion of the bronchial wall. These patients have major airway obstruction and partial or total atelectasis of the lung. Precise bronchoscopic assessment coupled with intraoperative bronchotomy allows the surgeon to determine the
origin of the tumor and to save uninvolved pulmonary parenchyma. Our experience with 4 such patients illustrates the possibility of saving lung tissue despite total bronchial obstruction.
A
within the lower lobe was not invaded. Upon reassessment, it may have been possible to save the left upper lobe if the endobronchial growth pattern had been recognized. The patient did well postoperatively without adjuvant chemotherapy or radiotherapy and was lost to follow-up 5% years later without evidence of metastases.
n important principle in the surgical management of pulmonary metastases is the conservation of any uninvolved lung. In most cases, the metastases are peripheral and resection can be accomplished with the loss of little lung tissue. In some cases, however, the tumor is more central and larger resections are required. We have operated on 4 patients with pulmonary metastases from leiomyosarcoma who had the unique feature of massive endobronchial extension. The clinical significance of this growth pattern prompted this report.
Case Reports
Patient 1 A 53-year-old woman had a dry cough and a low-grade fever. Seven years earlier, she had undergone a total abdominal hysterectomy and a bilateral salpingo-oophorectomy for a leiomyosarcoma of the uterus. On examination, the patient was found to have absent breath sounds over the left hemithorax. The chest roentgenogram showed opacification of the left lung with shift of the mediastinum and elevation of the left hemidiaphragm. At bronchoscopy, a firm polypoid mass was seen occluding the left main bronchus 2 cm from its origin. The tumor was assessed to be invading the anterior wall of the bronchus. Nuclear liver-spleen and bone scan results were negative. At thoracotomy, a large mass was palpated within the left lower lobe. A frozen section confirmed that the mass was a spindle cell tumor, and a left pneumonectomy was performed. The malignancy was found to be a 9-cm metastatic leiomyosarcoma in the lower lobe with endobronchial tumor extending from the left lower lobe bronchus to the carina; notably, the bronchial wall above the site of origin Accepted for publication June 19, 1990. Address reprint requests to Dr Warren, 1725 W Harrison St, Chicago, IL 60612.
0 1990 by The
Society of Thoracic Surgeons
(Ann Thoruc Surg 2990;50:73942)
Patient 2 A 40-year-old woman had dyspnea, palpitations, and increasing chest pain. She gave a 1-month history of fever and productive cough. Six years previously, she had undergone a total abdominal hysterectomy for “fibroids.” The physical examination revealed dullness to percussion and markedly decreased breath sounds over the right hemithorax. A chest roentgenogram revealed opacification of the right lung. At bronchoscopy, a polypoid mass was seen to obstruct the right main bronchus. A bronchial biopsy established the diagnosis of a malignani spindle cell neoplasm. Roentgenogram after bronchoscopy revealed reexpansion of the upper lobe and a discrete mass within the right lower lobe. Computed tomographic scans of the head and chest and a nuclear bone scan showed no metastases. At thoracotomy, multiple metastatic nodules were identified in the lower lobe. A bronchotomy was performed that confirmed the bronchoscopic impression that the endobronchial component of the tumor did not invade the wall of the bronchus intermedius. The endobronchial extension of tumor was resected en bloc with the right lower lobe. No adjuvant chemotherapy was administered. The patient died 22 months later of widespread metastatic disease.
Patient 3 A 71-year-old woman had an &week history of dyspnea on exertion, cough, and left-sided chest pain. Seven years previously, the patient had undergone resection of a low-grade retroperitoneal leiomyosarcoma. On examina0003-4975/90/$3.50
740
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
Ann Thorac Surg 1990;50:739-42
abdominal hysterectomy and a bilateral salpingo-oophorectomy for a low-grade leiomyosarcoma with a recuirrence in the ovary. Six months before the current admission, she underwent a wedge excision of a solitary and asymptomatic metastasis in the right lower lobe. On physical examination, the patient had no appreciable air entry into the right hemithorax. A chest roentgenogram demonstrated complete opacification of the right lung with a shift of the mediastinum to that side (Fig 2A). At bronchoscopy, tumor was visualized within the lumen of the right main bronchus as a glistening, gray-white polypoid mass without recognizable attachment to the bronchial wall (Fig 2B). The right upper lobe bronchus could not be seen. At the time of thoracotomy, a 9-cm tumor was seen to largely replace the right middle lobe. A bronchotomy was performed at the level of the bronchus intermedius and demonstrated that the tumor did not invade the bronchial wall proximal to this point. A right middle and lower lobectomy was performed, and the tumor was found to arise from the spur between the right middle and lower lobes (Fig 2C). All gross disease was resected and bronchial margins were free of tumor. The postoperative chest roentgenogram demonstrated complete reexpansion of the right upper lobe (Fig 2D). The patient did well after operation with marked improvement in the dyspnea. No adjuvant chemotherapy was given. She died 7 months later from a cardiac arrhythmia without clinical evidence of tumor recurrence. Fig 1 . (Patient 3.) Operative photograph illustrating the gross endobronchial extension of a leiomyosarcoma metastatic to the left lower lobe. The endobronchial component had obstructed the left upper lobe bronchus.
tion, there were markedly decreased breath sounds over the left hemithorax. A chest roentgenogram demonstrated opacification of the left lung with mediastinal shift. A computed tomographic scan of the chest and upper abdomen failed to disclose any evidence of metastatic disease in the right chest or liver. At bronchoscopy, a glistening polypoid mass filled the left main bronchus. Large fragments of the tumor were removed, which established the diagnosis of a malignant spindle cell neoplasm and reaerated the left upper lobe. It was the bronchoscopic impression that the tumor did not invade the bronchial wall proximal to the left upper lobe orifice. At thoracotomy, a bronchotomy at the left lower lobe bronchus revealed that the 3-cm tumor originated from the superior segment with endobronchial extension but without invasion of the bronchial wall proximal to the superior segmental bronchus. A left lower lobectomy was performed (Fig 1). Eighteen months after operation, hepatic metastases developed, which were treated with chemotherapy, and the patient died 2 years after the pulmonary resection.
Patient 4 A 42-year-old woman had a cough and mild shortness of breath. Two years prior, she had undergone a total
Comment Leiomyosarcoma is an uncommon malignancy usually arising in the uterus or in the retroperitoneum. The differential diagnosis between a leiomyosarcoma and simple degeneration of a uterine leiomyoma may be difficult, and in 2 of our patients, the primary tumors were originally misdiagnosed. These sarcomas tend to metastasize hematogenously to the lungs and liver. Because the diagnosis of the primary tumor is occasionally erroneous and because there is often a prolonged disease-free interval, the clinician may not be alert to this diagnostic possibility. The pulmonary metastases are usually large and limited in number. The tendency of these tumors to extend into the pulmonary vein has been recognized and should be considered when performing a resection [l].The gross extension through the bronchus to obstruct a main bronchus without direct involvement, however, has not been adequately appreciated. In our experience, leiomyosarcomas also may extend from the pulmonary parenchyma through the airway for a considerable distance without involving the bronchial wall. This growth pattern has been described in both metastatic and primary leiomyosarcomas of the lung [2-4]. The bronchoscopic appearance in the former is that of an endobronchial polypoid mass without involvement of the bronchial wall. It is important to assess the origin of the tumor mass as far distal as possible because this determines the site for an intraoperative bronchotomy. Such a bronchotomy was per-
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
741
C
A
R
D Fig 2 . (Patirnt 4.) ( A ) Prrop~ratinrposteroanterior chrst roentgenogram demonstrating a completely atelectatic right lung with no air bronchogram. (B)Endoscopic appearance of the tumor in ( A ) with endobronchial extension of tumor u p to the level of the right main bronchus but without inva
formed to determine the exact origin of the tumor in the 3 patients in whom lung parenchyma was saved. A distinction must be made between these tumors and true endobronchial metastases, which arise in the hronchial wall. The latter tumors are usually limited in size at the time of clinical presentation and lend themselves to endobronchial laser or brachytherapy salvaging most of the obstructed lung [5, 61. Carcinomas of the breast, kidney, and colon and melanomas are the most likely primary sites for endobronchial metastases (7--101. How-
ever, in patients with metastatic leiomyosarcoma with gross endobronchial extension, the endobronchial component is bulky and part of the distal atelectatic lung usually i s replaced with tumor, making laser photoablation and brachytherapy unfavorable modes of therapy. Because most of the bronchial wall is uninvolved, surgical resection with conservation of the uninvolved lung parenchyma is the procedure of choice. This distinction between endobronchial extension and true metastases to the bronchial submucosa must be made on bron-
742
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
Ann Thorac Surg
choscopy, which should be performed before resection of metastatic sarcoma with bulky tumor load, and radiologic or clinical evidence of airway obstruction. Previous reports of metastatic leiomyosarcoma with endobronchial extension emphasize the potential for the development of obstructive pneumonia, airway bleeding, and cardiac arrhythmias secondary to hypoxia in these patients [2,3]. As these sarcomas tend to be unresponsive to either chemotherapy or radiotherapy, we agree with others that resection of the metastases, while conserving obstructed but otherwise uninvolved lung tissue, should be performed for the palliation of these airway symptoms [ll-141. Adjuvant chemotherapy after complete surgical resections has not been found to be of benefit. In none of our 4 patients did local recurrence develop, and 1 longterm survivor is reported.
neodymium-YAG laser ablations of obstructing tracheal neoplasms. Ann Thorac Surg 1984;38:374-81. Schray MF, Martinez A, McDougall JC, Edmundson GK, Cortese DA, Brutinel WM. Malignant airway obstruction: management with temporary intraluminal brachytherapy and laser photoablation. Endocurie Hypertherm Oncol 1985; 1:237-45. Braman SS, Whitcomb ME. Endobronchial metastasis. Arch Intern Med 1975;135:543-7. Fitzgerald RH. Endobronchial metastases. South Med J 1977; 70:44O-1. Baumgartner WA, Mark JBD. Metastatic malignancies from distant sites to the tracheobronchial tree. J Thorac Cardiovasc Surg 1980;79:499-503. Kenny JN, Smith WL, Brawer MK. Endobronchial metastases from prostatic carcinoma. Ann Thorac Surg 1988;45:223-4. Flye MW, Woltering G, Rosenburg SA. Aggressive pulmonary resection for metastatic osteosarcoma and soft tissue sarcomas. Ann Thorac Surg 1984;37123-7. Roth JA, Putman JB, Wesley MN, Rosenberg SA. Differing determinants of prognosis following resection of pulmonary metastases from osteogenic and soft tissue sarcoma patients. Cancer 1985;55:1361-6. Rizzoni WE, Pass HI, Wesley MN, Rosenberg SA, Roth JA. Resection of recurrent pulmonary metastases in patients with soft-tissue sarcomas. Arch Surg 1986;121:1248-52. Udelsman R, Roth JA, Lees D, Jelenich SE, Pass HI. Endobronchial metastases from soft tissue sarcoma. J Surg Oncol 1986;32:145-9.
1990:50:739--42
6.
7.
8. 9. 10. 11.
References 1. Mansour KA, Malone CE, Craver JM. Left atrial tumor embolization during pulmonary resection: review of literature and report of two cases. Ann Thorac Surg 1988;46:455-6. 2. Flynn KJ, Kim H-S. Endobronchial metastasis of uterine leiomyosarcoma. JAMA 1978;240:2080. 3. Findice JC, Komansky H, Gordon R. Endobronchial metastasis of uterine leiomyosarcoma. JAMA 1979;241:1684. 4. Ramanathan T. Primary leiomyosarcoma of the lung. Thorax 1974;29:482-9. 5. Parr GVS, Unger M, Trout RG, Atkinson WG. One hundred
12.
13. 14.
Metastatic leiomyosarcoma occasionally is seen with gross endobronchial extension without invasion of the bronchial wall. These patients have major airway obstruction and partial or total atelectasis of the lung. Precise bronchoscopic assessment coupled with intraoperative bronchotomy allows the surgeon to determine the
origin of the tumor and to save uninvolved pulmonary parenchyma. Our experience with 4 such patients illustrates the possibility of saving lung tissue despite total bronchial obstruction.
A
within the lower lobe was not invaded. Upon reassessment, it may have been possible to save the left upper lobe if the endobronchial growth pattern had been recognized. The patient did well postoperatively without adjuvant chemotherapy or radiotherapy and was lost to follow-up 5% years later without evidence of metastases.
n important principle in the surgical management of pulmonary metastases is the conservation of any uninvolved lung. In most cases, the metastases are peripheral and resection can be accomplished with the loss of little lung tissue. In some cases, however, the tumor is more central and larger resections are required. We have operated on 4 patients with pulmonary metastases from leiomyosarcoma who had the unique feature of massive endobronchial extension. The clinical significance of this growth pattern prompted this report.
Case Reports
Patient 1 A 53-year-old woman had a dry cough and a low-grade fever. Seven years earlier, she had undergone a total abdominal hysterectomy and a bilateral salpingo-oophorectomy for a leiomyosarcoma of the uterus. On examination, the patient was found to have absent breath sounds over the left hemithorax. The chest roentgenogram showed opacification of the left lung with shift of the mediastinum and elevation of the left hemidiaphragm. At bronchoscopy, a firm polypoid mass was seen occluding the left main bronchus 2 cm from its origin. The tumor was assessed to be invading the anterior wall of the bronchus. Nuclear liver-spleen and bone scan results were negative. At thoracotomy, a large mass was palpated within the left lower lobe. A frozen section confirmed that the mass was a spindle cell tumor, and a left pneumonectomy was performed. The malignancy was found to be a 9-cm metastatic leiomyosarcoma in the lower lobe with endobronchial tumor extending from the left lower lobe bronchus to the carina; notably, the bronchial wall above the site of origin Accepted for publication June 19, 1990. Address reprint requests to Dr Warren, 1725 W Harrison St, Chicago, IL 60612.
0 1990 by The
Society of Thoracic Surgeons
(Ann Thoruc Surg 2990;50:73942)
Patient 2 A 40-year-old woman had dyspnea, palpitations, and increasing chest pain. She gave a 1-month history of fever and productive cough. Six years previously, she had undergone a total abdominal hysterectomy for “fibroids.” The physical examination revealed dullness to percussion and markedly decreased breath sounds over the right hemithorax. A chest roentgenogram revealed opacification of the right lung. At bronchoscopy, a polypoid mass was seen to obstruct the right main bronchus. A bronchial biopsy established the diagnosis of a malignani spindle cell neoplasm. Roentgenogram after bronchoscopy revealed reexpansion of the upper lobe and a discrete mass within the right lower lobe. Computed tomographic scans of the head and chest and a nuclear bone scan showed no metastases. At thoracotomy, multiple metastatic nodules were identified in the lower lobe. A bronchotomy was performed that confirmed the bronchoscopic impression that the endobronchial component of the tumor did not invade the wall of the bronchus intermedius. The endobronchial extension of tumor was resected en bloc with the right lower lobe. No adjuvant chemotherapy was administered. The patient died 22 months later of widespread metastatic disease.
Patient 3 A 71-year-old woman had an &week history of dyspnea on exertion, cough, and left-sided chest pain. Seven years previously, the patient had undergone resection of a low-grade retroperitoneal leiomyosarcoma. On examina0003-4975/90/$3.50
740
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
Ann Thorac Surg 1990;50:739-42
abdominal hysterectomy and a bilateral salpingo-oophorectomy for a low-grade leiomyosarcoma with a recuirrence in the ovary. Six months before the current admission, she underwent a wedge excision of a solitary and asymptomatic metastasis in the right lower lobe. On physical examination, the patient had no appreciable air entry into the right hemithorax. A chest roentgenogram demonstrated complete opacification of the right lung with a shift of the mediastinum to that side (Fig 2A). At bronchoscopy, tumor was visualized within the lumen of the right main bronchus as a glistening, gray-white polypoid mass without recognizable attachment to the bronchial wall (Fig 2B). The right upper lobe bronchus could not be seen. At the time of thoracotomy, a 9-cm tumor was seen to largely replace the right middle lobe. A bronchotomy was performed at the level of the bronchus intermedius and demonstrated that the tumor did not invade the bronchial wall proximal to this point. A right middle and lower lobectomy was performed, and the tumor was found to arise from the spur between the right middle and lower lobes (Fig 2C). All gross disease was resected and bronchial margins were free of tumor. The postoperative chest roentgenogram demonstrated complete reexpansion of the right upper lobe (Fig 2D). The patient did well after operation with marked improvement in the dyspnea. No adjuvant chemotherapy was given. She died 7 months later from a cardiac arrhythmia without clinical evidence of tumor recurrence. Fig 1 . (Patient 3.) Operative photograph illustrating the gross endobronchial extension of a leiomyosarcoma metastatic to the left lower lobe. The endobronchial component had obstructed the left upper lobe bronchus.
tion, there were markedly decreased breath sounds over the left hemithorax. A chest roentgenogram demonstrated opacification of the left lung with mediastinal shift. A computed tomographic scan of the chest and upper abdomen failed to disclose any evidence of metastatic disease in the right chest or liver. At bronchoscopy, a glistening polypoid mass filled the left main bronchus. Large fragments of the tumor were removed, which established the diagnosis of a malignant spindle cell neoplasm and reaerated the left upper lobe. It was the bronchoscopic impression that the tumor did not invade the bronchial wall proximal to the left upper lobe orifice. At thoracotomy, a bronchotomy at the left lower lobe bronchus revealed that the 3-cm tumor originated from the superior segment with endobronchial extension but without invasion of the bronchial wall proximal to the superior segmental bronchus. A left lower lobectomy was performed (Fig 1). Eighteen months after operation, hepatic metastases developed, which were treated with chemotherapy, and the patient died 2 years after the pulmonary resection.
Patient 4 A 42-year-old woman had a cough and mild shortness of breath. Two years prior, she had undergone a total
Comment Leiomyosarcoma is an uncommon malignancy usually arising in the uterus or in the retroperitoneum. The differential diagnosis between a leiomyosarcoma and simple degeneration of a uterine leiomyoma may be difficult, and in 2 of our patients, the primary tumors were originally misdiagnosed. These sarcomas tend to metastasize hematogenously to the lungs and liver. Because the diagnosis of the primary tumor is occasionally erroneous and because there is often a prolonged disease-free interval, the clinician may not be alert to this diagnostic possibility. The pulmonary metastases are usually large and limited in number. The tendency of these tumors to extend into the pulmonary vein has been recognized and should be considered when performing a resection [l].The gross extension through the bronchus to obstruct a main bronchus without direct involvement, however, has not been adequately appreciated. In our experience, leiomyosarcomas also may extend from the pulmonary parenchyma through the airway for a considerable distance without involving the bronchial wall. This growth pattern has been described in both metastatic and primary leiomyosarcomas of the lung [2-4]. The bronchoscopic appearance in the former is that of an endobronchial polypoid mass without involvement of the bronchial wall. It is important to assess the origin of the tumor mass as far distal as possible because this determines the site for an intraoperative bronchotomy. Such a bronchotomy was per-
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
741
C
A
R
D Fig 2 . (Patirnt 4.) ( A ) Prrop~ratinrposteroanterior chrst roentgenogram demonstrating a completely atelectatic right lung with no air bronchogram. (B)Endoscopic appearance of the tumor in ( A ) with endobronchial extension of tumor u p to the level of the right main bronchus but without inva
formed to determine the exact origin of the tumor in the 3 patients in whom lung parenchyma was saved. A distinction must be made between these tumors and true endobronchial metastases, which arise in the hronchial wall. The latter tumors are usually limited in size at the time of clinical presentation and lend themselves to endobronchial laser or brachytherapy salvaging most of the obstructed lung [5, 61. Carcinomas of the breast, kidney, and colon and melanomas are the most likely primary sites for endobronchial metastases (7--101. How-
ever, in patients with metastatic leiomyosarcoma with gross endobronchial extension, the endobronchial component is bulky and part of the distal atelectatic lung usually i s replaced with tumor, making laser photoablation and brachytherapy unfavorable modes of therapy. Because most of the bronchial wall is uninvolved, surgical resection with conservation of the uninvolved lung parenchyma is the procedure of choice. This distinction between endobronchial extension and true metastases to the bronchial submucosa must be made on bron-
742
WARREN ET AL LEIOMYOSARCOMA WITH ENDOBRONCHIAL EXTENSION
Ann Thorac Surg
choscopy, which should be performed before resection of metastatic sarcoma with bulky tumor load, and radiologic or clinical evidence of airway obstruction. Previous reports of metastatic leiomyosarcoma with endobronchial extension emphasize the potential for the development of obstructive pneumonia, airway bleeding, and cardiac arrhythmias secondary to hypoxia in these patients [2,3]. As these sarcomas tend to be unresponsive to either chemotherapy or radiotherapy, we agree with others that resection of the metastases, while conserving obstructed but otherwise uninvolved lung tissue, should be performed for the palliation of these airway symptoms [ll-141. Adjuvant chemotherapy after complete surgical resections has not been found to be of benefit. In none of our 4 patients did local recurrence develop, and 1 longterm survivor is reported.
neodymium-YAG laser ablations of obstructing tracheal neoplasms. Ann Thorac Surg 1984;38:374-81. Schray MF, Martinez A, McDougall JC, Edmundson GK, Cortese DA, Brutinel WM. Malignant airway obstruction: management with temporary intraluminal brachytherapy and laser photoablation. Endocurie Hypertherm Oncol 1985; 1:237-45. Braman SS, Whitcomb ME. Endobronchial metastasis. Arch Intern Med 1975;135:543-7. Fitzgerald RH. Endobronchial metastases. South Med J 1977; 70:44O-1. Baumgartner WA, Mark JBD. Metastatic malignancies from distant sites to the tracheobronchial tree. J Thorac Cardiovasc Surg 1980;79:499-503. Kenny JN, Smith WL, Brawer MK. Endobronchial metastases from prostatic carcinoma. Ann Thorac Surg 1988;45:223-4. Flye MW, Woltering G, Rosenburg SA. Aggressive pulmonary resection for metastatic osteosarcoma and soft tissue sarcomas. Ann Thorac Surg 1984;37123-7. Roth JA, Putman JB, Wesley MN, Rosenberg SA. Differing determinants of prognosis following resection of pulmonary metastases from osteogenic and soft tissue sarcoma patients. Cancer 1985;55:1361-6. Rizzoni WE, Pass HI, Wesley MN, Rosenberg SA, Roth JA. Resection of recurrent pulmonary metastases in patients with soft-tissue sarcomas. Arch Surg 1986;121:1248-52. Udelsman R, Roth JA, Lees D, Jelenich SE, Pass HI. Endobronchial metastases from soft tissue sarcoma. J Surg Oncol 1986;32:145-9.
1990:50:739--42
6.
7.
8. 9. 10. 11.
References 1. Mansour KA, Malone CE, Craver JM. Left atrial tumor embolization during pulmonary resection: review of literature and report of two cases. Ann Thorac Surg 1988;46:455-6. 2. Flynn KJ, Kim H-S. Endobronchial metastasis of uterine leiomyosarcoma. JAMA 1978;240:2080. 3. Findice JC, Komansky H, Gordon R. Endobronchial metastasis of uterine leiomyosarcoma. JAMA 1979;241:1684. 4. Ramanathan T. Primary leiomyosarcoma of the lung. Thorax 1974;29:482-9. 5. Parr GVS, Unger M, Trout RG, Atkinson WG. One hundred
12.
13. 14.