- Email: [email protected]
Primary hemangiopericytoma of the trachea
944
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
2. Symbas PN, Goldman MD, Erbesfield MH, et al. Pulmonary arteriovenous fistula, pulmonary artery aneurysm, and other vascular. Changes of the lung from penetrating trauma. Ann Surg 1980;191:336 –40. 3. Sheikhzadeh A, Paydar MH, Ghabussi P, et al. Pulmonary arteriovenous fistulas. Case presentations and clinical recognition. Herz 1983;8:179 –86. 4. Arom KV, Lyons GW. Traumatic pulmonary arteriovenous fistula. J Thorac Cardiovasc Surg 1975;70:918 –20. 5. Shields TW. Congenital vascular lesions of the lungs. In: Shields, et al. General thoracic surgery, vol. 24, 1994;895–906. 6. Taylor BG. Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula. Am J Med 1978;64:360.
CASE REPORTS
Primary Hemangiopericytoma of the Trachea Masataka Yoshida, MD, Masafumi Morita, MD, Shotaro Kakimoto, MD, Manpei Kawakami, MD, and Shinjiro Sasaki, MD Department of Thoracic and Cardiovascular Surgery, Minamiosaka General Hospital, Osaka, and Department of Thoracic and Cardiovascular Surgery, Osaka Medical College Hospital, Takatsuki, Osaka, Japan
A 28-year-old man arrived at our hospital complaining of severe dyspnea. Bronchoscopic findings demonstrated that a tumor was located approximately 4 cm distant from the vocal cord, occupying most of the tracheal lumen. To urgently relieve the dyspnea, some parts of the tumor were cauterized with the neodymium:yttrium– aluminum garnet laser. Histopathologic examination of the cauterized specimen revealed malignant hemangiopericytoma. During the operation, the tumor was resected en bloc with a tracheal segment containing four tracheal cartilages. The patient’s postoperative course was uneventful with no evidence of recurrence 1 year after the operation. (Ann Thorac Surg 2003;76:944 – 6) © 2003 by The Society of Thoracic Surgeons
H
emangiopericytoma, which is known to be derived from the vascular pericyte, is rarely found in the tracheal wall. We believe there have been two case reports of hemangiopericytoma occurring in the trachea [1,2]. Herein, we present a surgical case of hemangiopericytoma occurring from the posterior wall of the trachea. A 28-year-old man arrived at our hospital complaining of severe dyspnea associated with bloody sputum. He had a 1-year history of a sore throat, especially in coughing and swallowing. A computed tomographic scan revealed a
Accepted for publication Jan 23, 2003. Address reprint requests to Dr Yoshida, Department of Thoracic and Cardiovascular Surgery, Osaka Medical College, 2-7 Daigakucho Takatsuki 569-8686, Japan; e-mail: [email protected].
© 2003 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2003;76:944 – 6
mass arising from the posterior wall of the trachea at the level of the upper thoracic vertebrae. Magnetic resonance imaging showed a low-intensity mass in the T1-weighted images and a high-intensity mass without invading the esophagus in the T2-weighted images. Bronchoscopic findings demonstrated that the tumor was located approximately 4 cm from the vocal cord with a stalk in the posterior wall of the trachea, occupying most of the tracheal lumen (Fig 1A). The dyspnea was so severe that some parts of the tumor were urgently cauterized by bronchoscopy with a neodymium:yttrium–aluminum garnet laser (Fig 1B). Histopathologic examination of the cauterized specimen revealed malignant hemangiopericytoma, and the decision was made to remove the tumor surgically. At the operation, the tumor was detected on the posterior wall of the trachea (membranaceous trachea) without adhering (invasion) to the esophagus. The tumor was resected en bloc with a tracheal segment containing four tracheal cartilages. Histopathologically, the tumor had a number of capillaries and small vessels with a single layer of endothelium and dense spindle-shaped tumor cells that proliferated around the blood vessels (Fig 2A). A silver stain showed that the individual tumor cells were surrounded by reticular fibers (Fig 2B). Immunohistochemically, the difference between the lining endothelial cells and the surrounding pericytes was made apparent with stains for factor VIII-related antigen, which bound to the endothelial cells present along slit-like spaces, but did not stain the pericytes. The tumor cells were clearly positive for vimentin and collagen type IV (Figs 2C , D), but negative for S-100 protein and cytokeratins. In electron microscopic findings, organelles were poorly developed, and microfilaments were observed in the cytoplasm. The cells occupying the area around the basal lamina incompletely had round or oval nuclei. The postoperative course was uneventful with no evidence of recurrence 1 year after the operation.
Comment Hemangiopericytoma was first reported by Stout and Murray [3] in 1942; it is a vascular tumor that occurs from pericytes. The tumor occurs most commonly in the skin, subcutaneous soft tissues, muscles of the extremities, retroperioneum, but rarely in the lung or the trachea. In cases in which the tumor occurs in the tracheal wall, the symptoms include coughing, dyspnea, or bloodcontained sputum. In this case, in which the tumor occupied most of the tracheal lumen, some parts of the tumor were urgently cauterized with a neodymium: yttrium–aluminum garnet laser. Because this is a vascular tumor, usually demonstrating hypervascularity, magnetic resonance imaging or angiography is an effective diagnostic tool rather than a computed tomographic scan. Histopathologically, the tumor consists of a large number of capillaries and small vessels, dense spindleshaped tumor cells, and reticular fibers [4]. Immunohistochemically, hemangiopericytomas are known to show a positive response to antibodies against vimentin and 0003-4975/03/$30.00 PII S0003-4975(03)00137-1
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
945
CASE REPORTS
Ann Thorac Surg 2003;76:944 – 6
Fig 1. (A) Bronchoscopic findings demonstrated that the tumor was occupying most of the tracheal lumen. (B) Some parts of the tumor were cauterized with the neodymium:yttrium–aluminum garnet laser.
type IV collagen, and a negative response to VIII-related antigen, S-100 protein, neuronspecific enolase carcinoembryonic antigen, desmins, laminin, and cytokeratins [5]. Surgical resection is the first choice of treatment, although the criteria for determining the area of resection have not been established. Hansen and colleagues [6] suggested that all hemangiopericytomas should be considered as malignant features, and they recommended extended surgery. In the cases of primary pulmonary hemangiopericytoma, despite the performance of transbronchial lung biopsies and percutaneous needle lung biopsies, there have been no reports of cases in which a definite diagnosis was achieved before surgery. In our case, because the tracheal lumen was occupied by more than 75% tumor with serious complaint of dyspnea, transtracheal neodymium:yttrium–aluminum garnet la-
ser therapy was performed before operative resection. With respect to postoperative adjuvant therapy, irradiation has been recommended by Razzuk and colleagues [7]; however, it is generally considered to be almost ineffective. On the other hand, combination therapy or single therapy with Adriamycin (Kyowa Hakko Kogyo, Tokyo, Japan) is reported to be effective against metastases [5]. Although currently developed transtracheal radiation therapy may be a possible option for treatment of the tumor, operative resection must first be considered. The prognosis of this tumor is left to be determined, because it depends on reports of whether it is benign or malignant. Approximately 50% of hemangiopericytomas have been reported to recur within 5 years [6]. Distant metastases to liver [6], brain, and bone have also been reported in cases of primary pulmonary hemangioperiFig 2. (A) This tumor was rich in capillaries and small vessels with a single layer of endothelium and dense spindle-shaped tumor cells that proliferated around blood vessels in hematoxylin & eosin stain. (B) A silver stain showed the individual tumor cells surrounded by reticular fibers. The tumor cells were clearly positive for (C) vimentin and (D) collagen type IV (all stains, original magnification ⫻400).
946
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
cytoma. Therefore, long-term, careful follow-up for this tumor is required. We thank Dr Kotaro Isoda for his useful advice on the histopathologic analysis.
References 1. Bremond G, Garcia M, Bonnaud G, Magnan J, De Micco C, Abdul S. Les hemangiopericytomas de la splere ORL Jr Fr Otorhinolaryngol Audiophonol Chir Maxillofoc 1975;24:381– 6. 2. Ballard RW, Yarington CT Jr. Hemangiopericytoma of the tracheal wall. Arch Otolaryngol 1981;107:558 –60.
Ann Thorac Surg 2003;76:944 – 6
3. Stout AP, Murray MR. Hemangiopericytoma. A vascular tumor featuring Zimmerman’s pericytes. Ann Surg 1942;116: 26 –33. 4. Enzinger FM, Smith BH. Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 1976;7:61–82. 5. Rusch VW, Shuman WP, Schmidt R, Laramore GE. Massive pulmonary hemangiopericytoma. An innovative approach to evaluation and treatment. Cancer 1989;64:1928 – 36. 6. Hansen CP, Francis D, Bertelsen S. Primary hemangiopericytoma of the lung. Case report. Scand J Thorac Cardiovasc Surg 1990;24:89 –92. 7. Razzuk MK, Nassur A, Gardner MA, Martine J, Gogara SF, Urchel HC Jr. Primary pulmonary hemangiopericytoma. J Thorac Cardiovasc Surg 1977;74:227–9.
CASE REPORTS
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
2. Symbas PN, Goldman MD, Erbesfield MH, et al. Pulmonary arteriovenous fistula, pulmonary artery aneurysm, and other vascular. Changes of the lung from penetrating trauma. Ann Surg 1980;191:336 –40. 3. Sheikhzadeh A, Paydar MH, Ghabussi P, et al. Pulmonary arteriovenous fistulas. Case presentations and clinical recognition. Herz 1983;8:179 –86. 4. Arom KV, Lyons GW. Traumatic pulmonary arteriovenous fistula. J Thorac Cardiovasc Surg 1975;70:918 –20. 5. Shields TW. Congenital vascular lesions of the lungs. In: Shields, et al. General thoracic surgery, vol. 24, 1994;895–906. 6. Taylor BG. Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula. Am J Med 1978;64:360.
CASE REPORTS
Primary Hemangiopericytoma of the Trachea Masataka Yoshida, MD, Masafumi Morita, MD, Shotaro Kakimoto, MD, Manpei Kawakami, MD, and Shinjiro Sasaki, MD Department of Thoracic and Cardiovascular Surgery, Minamiosaka General Hospital, Osaka, and Department of Thoracic and Cardiovascular Surgery, Osaka Medical College Hospital, Takatsuki, Osaka, Japan
A 28-year-old man arrived at our hospital complaining of severe dyspnea. Bronchoscopic findings demonstrated that a tumor was located approximately 4 cm distant from the vocal cord, occupying most of the tracheal lumen. To urgently relieve the dyspnea, some parts of the tumor were cauterized with the neodymium:yttrium– aluminum garnet laser. Histopathologic examination of the cauterized specimen revealed malignant hemangiopericytoma. During the operation, the tumor was resected en bloc with a tracheal segment containing four tracheal cartilages. The patient’s postoperative course was uneventful with no evidence of recurrence 1 year after the operation. (Ann Thorac Surg 2003;76:944 – 6) © 2003 by The Society of Thoracic Surgeons
H
emangiopericytoma, which is known to be derived from the vascular pericyte, is rarely found in the tracheal wall. We believe there have been two case reports of hemangiopericytoma occurring in the trachea [1,2]. Herein, we present a surgical case of hemangiopericytoma occurring from the posterior wall of the trachea. A 28-year-old man arrived at our hospital complaining of severe dyspnea associated with bloody sputum. He had a 1-year history of a sore throat, especially in coughing and swallowing. A computed tomographic scan revealed a
Accepted for publication Jan 23, 2003. Address reprint requests to Dr Yoshida, Department of Thoracic and Cardiovascular Surgery, Osaka Medical College, 2-7 Daigakucho Takatsuki 569-8686, Japan; e-mail: [email protected].
© 2003 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2003;76:944 – 6
mass arising from the posterior wall of the trachea at the level of the upper thoracic vertebrae. Magnetic resonance imaging showed a low-intensity mass in the T1-weighted images and a high-intensity mass without invading the esophagus in the T2-weighted images. Bronchoscopic findings demonstrated that the tumor was located approximately 4 cm from the vocal cord with a stalk in the posterior wall of the trachea, occupying most of the tracheal lumen (Fig 1A). The dyspnea was so severe that some parts of the tumor were urgently cauterized by bronchoscopy with a neodymium:yttrium–aluminum garnet laser (Fig 1B). Histopathologic examination of the cauterized specimen revealed malignant hemangiopericytoma, and the decision was made to remove the tumor surgically. At the operation, the tumor was detected on the posterior wall of the trachea (membranaceous trachea) without adhering (invasion) to the esophagus. The tumor was resected en bloc with a tracheal segment containing four tracheal cartilages. Histopathologically, the tumor had a number of capillaries and small vessels with a single layer of endothelium and dense spindle-shaped tumor cells that proliferated around the blood vessels (Fig 2A). A silver stain showed that the individual tumor cells were surrounded by reticular fibers (Fig 2B). Immunohistochemically, the difference between the lining endothelial cells and the surrounding pericytes was made apparent with stains for factor VIII-related antigen, which bound to the endothelial cells present along slit-like spaces, but did not stain the pericytes. The tumor cells were clearly positive for vimentin and collagen type IV (Figs 2C , D), but negative for S-100 protein and cytokeratins. In electron microscopic findings, organelles were poorly developed, and microfilaments were observed in the cytoplasm. The cells occupying the area around the basal lamina incompletely had round or oval nuclei. The postoperative course was uneventful with no evidence of recurrence 1 year after the operation.
Comment Hemangiopericytoma was first reported by Stout and Murray [3] in 1942; it is a vascular tumor that occurs from pericytes. The tumor occurs most commonly in the skin, subcutaneous soft tissues, muscles of the extremities, retroperioneum, but rarely in the lung or the trachea. In cases in which the tumor occurs in the tracheal wall, the symptoms include coughing, dyspnea, or bloodcontained sputum. In this case, in which the tumor occupied most of the tracheal lumen, some parts of the tumor were urgently cauterized with a neodymium: yttrium–aluminum garnet laser. Because this is a vascular tumor, usually demonstrating hypervascularity, magnetic resonance imaging or angiography is an effective diagnostic tool rather than a computed tomographic scan. Histopathologically, the tumor consists of a large number of capillaries and small vessels, dense spindleshaped tumor cells, and reticular fibers [4]. Immunohistochemically, hemangiopericytomas are known to show a positive response to antibodies against vimentin and 0003-4975/03/$30.00 PII S0003-4975(03)00137-1
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
945
CASE REPORTS
Ann Thorac Surg 2003;76:944 – 6
Fig 1. (A) Bronchoscopic findings demonstrated that the tumor was occupying most of the tracheal lumen. (B) Some parts of the tumor were cauterized with the neodymium:yttrium–aluminum garnet laser.
type IV collagen, and a negative response to VIII-related antigen, S-100 protein, neuronspecific enolase carcinoembryonic antigen, desmins, laminin, and cytokeratins [5]. Surgical resection is the first choice of treatment, although the criteria for determining the area of resection have not been established. Hansen and colleagues [6] suggested that all hemangiopericytomas should be considered as malignant features, and they recommended extended surgery. In the cases of primary pulmonary hemangiopericytoma, despite the performance of transbronchial lung biopsies and percutaneous needle lung biopsies, there have been no reports of cases in which a definite diagnosis was achieved before surgery. In our case, because the tracheal lumen was occupied by more than 75% tumor with serious complaint of dyspnea, transtracheal neodymium:yttrium–aluminum garnet la-
ser therapy was performed before operative resection. With respect to postoperative adjuvant therapy, irradiation has been recommended by Razzuk and colleagues [7]; however, it is generally considered to be almost ineffective. On the other hand, combination therapy or single therapy with Adriamycin (Kyowa Hakko Kogyo, Tokyo, Japan) is reported to be effective against metastases [5]. Although currently developed transtracheal radiation therapy may be a possible option for treatment of the tumor, operative resection must first be considered. The prognosis of this tumor is left to be determined, because it depends on reports of whether it is benign or malignant. Approximately 50% of hemangiopericytomas have been reported to recur within 5 years [6]. Distant metastases to liver [6], brain, and bone have also been reported in cases of primary pulmonary hemangioperiFig 2. (A) This tumor was rich in capillaries and small vessels with a single layer of endothelium and dense spindle-shaped tumor cells that proliferated around blood vessels in hematoxylin & eosin stain. (B) A silver stain showed the individual tumor cells surrounded by reticular fibers. The tumor cells were clearly positive for (C) vimentin and (D) collagen type IV (all stains, original magnification ⫻400).
946
CASE REPORT YOSHIDA ET AL PRIMARY HEMANGIOPERICYTOMA OF THE TRACHEA
cytoma. Therefore, long-term, careful follow-up for this tumor is required. We thank Dr Kotaro Isoda for his useful advice on the histopathologic analysis.
References 1. Bremond G, Garcia M, Bonnaud G, Magnan J, De Micco C, Abdul S. Les hemangiopericytomas de la splere ORL Jr Fr Otorhinolaryngol Audiophonol Chir Maxillofoc 1975;24:381– 6. 2. Ballard RW, Yarington CT Jr. Hemangiopericytoma of the tracheal wall. Arch Otolaryngol 1981;107:558 –60.
Ann Thorac Surg 2003;76:944 – 6
3. Stout AP, Murray MR. Hemangiopericytoma. A vascular tumor featuring Zimmerman’s pericytes. Ann Surg 1942;116: 26 –33. 4. Enzinger FM, Smith BH. Hemangiopericytoma. An analysis of 106 cases. Hum Pathol 1976;7:61–82. 5. Rusch VW, Shuman WP, Schmidt R, Laramore GE. Massive pulmonary hemangiopericytoma. An innovative approach to evaluation and treatment. Cancer 1989;64:1928 – 36. 6. Hansen CP, Francis D, Bertelsen S. Primary hemangiopericytoma of the lung. Case report. Scand J Thorac Cardiovasc Surg 1990;24:89 –92. 7. Razzuk MK, Nassur A, Gardner MA, Martine J, Gogara SF, Urchel HC Jr. Primary pulmonary hemangiopericytoma. J Thorac Cardiovasc Surg 1977;74:227–9.
CASE REPORTS