Ann Thorac Surg 2014;98:725–7
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
We thank the Associazione Un Cuore Un Mondo (www. uncuoreunmondo.org) for the technical and financial support to the mission.
References 1. Celermajer DS, Bull C, Till JA, et al. Ebstein’s anomaly. J Am Coll Cardiol 1994;23:170–6. 2. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082–7. 3. Reemtsen BL, Polimenakos AC, Fagan BT, Wells WJ, Starnes VA. Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2007;134:1406–12. 4. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006;132:1285–90. 5. Polimenakos AC, Reemtsen BL, Wells WJ, Starnes VA. Right ventricular exclusion procedure with total cavopulmonary connection: an alternative operative approach in adults with severe Ebstein anomaly. J Thorac Cardiovasc Surg 2008;135: 1182–3. 6. Takagaki M, Ishino K, Kawada M, et al. Total right ventricular exclusion improves left ventricular function in patients with end-stage congestive right ventricular failure. Circulation 2003;108(Suppl 1):II226–9. 7. Sano S, Ishino K, Kawada M, et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002;123:640–7. 8. Cantinotti M, Bell A, Razavi R. Role of magnetic resonance imaging in different ways of presentation of Ebstein’s anomaly. J Cardiovasc Med (Hagerstown) 2008;9:628–30.
Leiomyosarcoma of the superior vena cava is a very rare tumor and only a few cases have been reported, with various techniques of vascular reconstruction. We describe a new case of leiomyosarcoma of the superior vena cava in a 61-year-old woman with extension to the brachiocephalic arterial trunk. Resection and vascular reconstruction were performed using, respectively, polytetrafluoroethylene and polyethylene terephtalate vascular grafts. (Ann Thorac Surg 2014;98:725–7) Ó 2014 by The Society of Thoracic Surgeons
V
ascular leiomyosarcoma is a very rare tumor and the inferior vena cava is the most common site (50%) [1]. The superior vena cava (SVC) is exceptionally involved and only a dozen cases have been previously reported [2]. The tumor is commonly revealed by physical signs of a SVC obstruction syndrome, less frequently by metastasis or unexpected computed tomography scan finding. Leiomyosarcoma is considered a very locally aggressive tumor with rare distant dissemination and an
FEATURE ARTICLES
rapid resolution of right-side heart failure and subsequent amelioration of LV function. Extreme cases of Ebstein’s anomaly like the one described in this case report are rare in developed countries, whereas they may more often present to a surgical team when working in a humanitarian mission in Third World countries. The choice to perform very high risk surgery within a setting of limited resources (and material) against a decision to leave such cases untreated is challenging, not to mention the ethical set of problems behind it. To operate on this case was clearly a gamble, but to wager and battle for the sake of a sick patient with no other options is a worthwhile and sometimes rewarding task.
725
Leiomyosarcoma of the Superior Vena Cava Arthus de Chaumont, MD, Charles Pierret, MD, Xavier de Kerangal, MD, Sylvestre Le Moulec, MD, and Franc¸ois Laborde, MD Departments of Vascular Surgery and Oncology, Military Hospital Val-de-Grace; and Department of Cardiac Pathology, Institut Mutualiste Montsouris, Paris, France ˇ
Accepted for publication Sept 30, 2013. Address correspondence to Dr de Chaumont, Service de Chirurgie Vasculaire, HIA Val-de-Grace, 74 Blvd de Port-Royal, 75230 Paris Cedex 05, France; e-mail: [email protected]. ˇ
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Primary leiomyosarcoma of the superior vena cava, operative findings. The arrow shows the tumor involving superior vena cava (SVC) and brachiocephalic trunk (B). (Ao ¼ aorta; RA ¼ right atrium.) 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.106
726
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
Ann Thorac Surg 2014;98:725–7
unfavorable prognosis. An aggressive therapeutic approach, including chemoradiotherapy and large surgical resection, is usually considered the treatment of choice [1]. Replacement of the SVC has been described using various techniques of vein reconstruction, on an arrested heart or not [3-6].
FEATURE ARTICLES
In January 2010, a 61-year-old woman was admitted to the hospital with facial swelling, collateral venous circulation, and tinnitus. A chest roentgenogram showed a right paravertebral mass with superior mediastinal widening, and computed tomography revealed a mediastinal tumor, 4 cm in diameter, involving the SVC and brachiocephalic artery. A positron emission tomography scan confirmed the malignant nature of the tumor, without locoregional or distant significant abnormalities. A cavography showed a very tight stenosis with thrombus, and a stent was placed to treat the SVC syndrome. Biopsy was performed through a mediastinoscopy by cervicotomy and revealed a spindle cell neoplasm. The immunohistochemical study showed the tumor was clearly positive for actin smooth muscle antigen and negative for S100 protein, CD 68, CD 34, cytokeratin AE1, AE3, H-caldesmon, and CD 117. This was in favor of an epithelioid leiomyosarcoma. In February 2010, the patient was operated on. Through a median sternotomy and after opening the pericardium, inspection showed that the tumor infiltrated the SVC 2 cm before the right atrium and also the lateral face of the brachiocephalic arterial trunk 1 cm after its origin. A cardiopulmonary bypass was placed with a cannula between the aorta and the brachiocephalic artery just before its bifurcation, on the one hand, and a cannula between the right atrium and the internal jugular vein on the other hand (Fig 1). It was used in normothermia as a circulatory support. The SVC was resected with safety margins, and the brachiocephalic artery was dissected from its origin to its bifurcation. The right phrenic nerve was infiltrated. The tumor was completely removed, requiring the section of the azygos vein and the right phrenic nerve. The SVC was reconstructed using a polytetrafluoroethylene (PTFE) ringed vascular graft, and the brachiocephalic arterial trunk was replaced with a polyethylene terephtalate vascular graft anastomosed on the aorta upstream (Fig 2). The patient’s postoperative course was complicated by a dysphonia due to recurrent laryngeal nerve paralysis, which was successfully treated by autologous fat injection. Pathology analysis confirmed the diagnosis of highgrade leiomyosarcoma of the SVC with margins of both the SVC and brachiocephalic artery free of tumor. From April to June 2010, four courses of chemotherapy with adriamycin and deticene were administered. In March 2012, the patient was restaged and no signs of mediastinal recurrences; neither were distant metastases found. Vascular grafts showed perfect patency.
Comment Primary vascular leiomyosarcomas are very rare malignant tumors, approximately 2% of all leiomyosarcomas
Fig 2. Vascular reconstruction of the superior vena cava and the brachiocephalic trunk.
[3]. The tumor originates from proliferation of smooth muscle cells of the media and may grow intravascularly or extravascularly or both [7]. Distant dissemination is rarely described. It involves large veins almost five times more than arteries, and the most common site is the inferior vena cava and its branches [3]. It occurs in women in a large majority. The SVC localization is extremely rare, and only 15 cases have been previously reported [1, 6]. It usually results in SVC syndrome of gradual onset. Computed tomography and magnetic resonance imaging may suggest the original tumor, but the diagnosis is histologic and requires mediastinoscopy, thoracotomy, or percutaneous endovascular biopsy. Therapeutic management should be aggressive and include preoperative radiotherapy to reduce the tumor volume and adjuvant chemoradiotherapy, but is mostly based on a large surgical resection when possible [3]. Several techniques for vascular reconstruction have been described. Autologous vein may be the best graft in a low-pressure venous system. The spiral vein graft offers a sufficient graft caliber but it extends the operating time [4, 5]. The use of custom-made autologous pericardial graft or bovine pericardial graft has also been proposed [6] but not reproduced. A PTFE prosthesis is most
commonly used because of its rapid endothelialization, and it produces less thrombogenicity than polyethylene terephtalate. Furthermore, PTFE has the advantage of being reinforced by rings, reducing the risk of collapse when the venous pressure drops [2]. However, in addition to the increased risk of infection, the long-term patency of synthetic implants after reconstruction of large vessels remains unclear. There is actually no evidence to confirm the superiority of one technique over the others but according to Dartevelle and associates [8], reporting a 5-year patency rate of 86%, we believe that vascular reconstruction of the SVC using PTFE prosthesis is a valuable option. In our case, 2 years of follow-up showed graft patency and no infection. We have reported the first case of primary leiomyosarcoma involving both superior vena cava and brachiocephalic arterial trunk. Whereas large surgical resection is commonly recommended, several techniques for vascular reconstruction have been described. Our preference was for PTFE ringed vascular graft, and we obtained the outcomes of a simple postoperative course and no longterm complications.
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
727
References 1. Spaggiari L, Regnard JF, Nottin R, et al. Leiomyosarcoma of the superior vena cava. Ann Thorac Surg 1996;62:274–6. 2. Picquet J, Blin V, Dussaussoy C, et al. Surgical reconstruction of the superior vena cava system: indications and results. Surgery 2009;145:93–9. 3. Levett JM, Meffert WG, Strong WW, et al. Leiomyosarcoma of the superior vena cava and azygos vein. Ann Thorac Surg 1995;60:1415–7. 4. Chiu CJ, Terzis J, MacRae ML. Replacement of superior vena cava with the spiral composite vein graft. A versatile technique. Ann Thorac Surg 1974;17:555–60. 5. Doty JR, Flores JH, Doty DB. Superior vena cava obstruction: bypass using spiral vein graft. Ann Thorac Surg 1999;67:111–6. 6. Benvenuti M, Lorusso R, Gelsomino S, et al. Resection of a primary leiomyosarcoma of the superior vena cava and right atrium on a beating heart. Int J Cardiol 2011;15:151–3. 7. Tovar-Martin E, Tovar-Pardo AE, Marini M, Pimentel Y, Rois JM. Intraluminal leiomyosarcoma of the superior vena cava: a cause of superior vena cava syndrome. J Cardiovasc Surg 1997;38:33–5. 8. Dartevelle P, Chapelier A, Navajas M, et al. Replacement of the superior vena cava with polytetrafluoroethylene grafts combined with resection of mediastinal-pulmonary malignant tumors. Report of thirteen cases. J Thorac Cardiovasc Surg 1987;94:361–6.
FEATURE ARTICLES
Ann Thorac Surg 2014;98:725–7
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
We thank the Associazione Un Cuore Un Mondo (www. uncuoreunmondo.org) for the technical and financial support to the mission.
References 1. Celermajer DS, Bull C, Till JA, et al. Ebstein’s anomaly. J Am Coll Cardiol 1994;23:170–6. 2. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082–7. 3. Reemtsen BL, Polimenakos AC, Fagan BT, Wells WJ, Starnes VA. Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2007;134:1406–12. 4. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006;132:1285–90. 5. Polimenakos AC, Reemtsen BL, Wells WJ, Starnes VA. Right ventricular exclusion procedure with total cavopulmonary connection: an alternative operative approach in adults with severe Ebstein anomaly. J Thorac Cardiovasc Surg 2008;135: 1182–3. 6. Takagaki M, Ishino K, Kawada M, et al. Total right ventricular exclusion improves left ventricular function in patients with end-stage congestive right ventricular failure. Circulation 2003;108(Suppl 1):II226–9. 7. Sano S, Ishino K, Kawada M, et al. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 2002;123:640–7. 8. Cantinotti M, Bell A, Razavi R. Role of magnetic resonance imaging in different ways of presentation of Ebstein’s anomaly. J Cardiovasc Med (Hagerstown) 2008;9:628–30.
Leiomyosarcoma of the superior vena cava is a very rare tumor and only a few cases have been reported, with various techniques of vascular reconstruction. We describe a new case of leiomyosarcoma of the superior vena cava in a 61-year-old woman with extension to the brachiocephalic arterial trunk. Resection and vascular reconstruction were performed using, respectively, polytetrafluoroethylene and polyethylene terephtalate vascular grafts. (Ann Thorac Surg 2014;98:725–7) Ó 2014 by The Society of Thoracic Surgeons
V
ascular leiomyosarcoma is a very rare tumor and the inferior vena cava is the most common site (50%) [1]. The superior vena cava (SVC) is exceptionally involved and only a dozen cases have been previously reported [2]. The tumor is commonly revealed by physical signs of a SVC obstruction syndrome, less frequently by metastasis or unexpected computed tomography scan finding. Leiomyosarcoma is considered a very locally aggressive tumor with rare distant dissemination and an
FEATURE ARTICLES
rapid resolution of right-side heart failure and subsequent amelioration of LV function. Extreme cases of Ebstein’s anomaly like the one described in this case report are rare in developed countries, whereas they may more often present to a surgical team when working in a humanitarian mission in Third World countries. The choice to perform very high risk surgery within a setting of limited resources (and material) against a decision to leave such cases untreated is challenging, not to mention the ethical set of problems behind it. To operate on this case was clearly a gamble, but to wager and battle for the sake of a sick patient with no other options is a worthwhile and sometimes rewarding task.
725
Leiomyosarcoma of the Superior Vena Cava Arthus de Chaumont, MD, Charles Pierret, MD, Xavier de Kerangal, MD, Sylvestre Le Moulec, MD, and Franc¸ois Laborde, MD Departments of Vascular Surgery and Oncology, Military Hospital Val-de-Grace; and Department of Cardiac Pathology, Institut Mutualiste Montsouris, Paris, France ˇ
Accepted for publication Sept 30, 2013. Address correspondence to Dr de Chaumont, Service de Chirurgie Vasculaire, HIA Val-de-Grace, 74 Blvd de Port-Royal, 75230 Paris Cedex 05, France; e-mail: [email protected]. ˇ
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Primary leiomyosarcoma of the superior vena cava, operative findings. The arrow shows the tumor involving superior vena cava (SVC) and brachiocephalic trunk (B). (Ao ¼ aorta; RA ¼ right atrium.) 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.09.106
726
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
Ann Thorac Surg 2014;98:725–7
unfavorable prognosis. An aggressive therapeutic approach, including chemoradiotherapy and large surgical resection, is usually considered the treatment of choice [1]. Replacement of the SVC has been described using various techniques of vein reconstruction, on an arrested heart or not [3-6].
FEATURE ARTICLES
In January 2010, a 61-year-old woman was admitted to the hospital with facial swelling, collateral venous circulation, and tinnitus. A chest roentgenogram showed a right paravertebral mass with superior mediastinal widening, and computed tomography revealed a mediastinal tumor, 4 cm in diameter, involving the SVC and brachiocephalic artery. A positron emission tomography scan confirmed the malignant nature of the tumor, without locoregional or distant significant abnormalities. A cavography showed a very tight stenosis with thrombus, and a stent was placed to treat the SVC syndrome. Biopsy was performed through a mediastinoscopy by cervicotomy and revealed a spindle cell neoplasm. The immunohistochemical study showed the tumor was clearly positive for actin smooth muscle antigen and negative for S100 protein, CD 68, CD 34, cytokeratin AE1, AE3, H-caldesmon, and CD 117. This was in favor of an epithelioid leiomyosarcoma. In February 2010, the patient was operated on. Through a median sternotomy and after opening the pericardium, inspection showed that the tumor infiltrated the SVC 2 cm before the right atrium and also the lateral face of the brachiocephalic arterial trunk 1 cm after its origin. A cardiopulmonary bypass was placed with a cannula between the aorta and the brachiocephalic artery just before its bifurcation, on the one hand, and a cannula between the right atrium and the internal jugular vein on the other hand (Fig 1). It was used in normothermia as a circulatory support. The SVC was resected with safety margins, and the brachiocephalic artery was dissected from its origin to its bifurcation. The right phrenic nerve was infiltrated. The tumor was completely removed, requiring the section of the azygos vein and the right phrenic nerve. The SVC was reconstructed using a polytetrafluoroethylene (PTFE) ringed vascular graft, and the brachiocephalic arterial trunk was replaced with a polyethylene terephtalate vascular graft anastomosed on the aorta upstream (Fig 2). The patient’s postoperative course was complicated by a dysphonia due to recurrent laryngeal nerve paralysis, which was successfully treated by autologous fat injection. Pathology analysis confirmed the diagnosis of highgrade leiomyosarcoma of the SVC with margins of both the SVC and brachiocephalic artery free of tumor. From April to June 2010, four courses of chemotherapy with adriamycin and deticene were administered. In March 2012, the patient was restaged and no signs of mediastinal recurrences; neither were distant metastases found. Vascular grafts showed perfect patency.
Comment Primary vascular leiomyosarcomas are very rare malignant tumors, approximately 2% of all leiomyosarcomas
Fig 2. Vascular reconstruction of the superior vena cava and the brachiocephalic trunk.
[3]. The tumor originates from proliferation of smooth muscle cells of the media and may grow intravascularly or extravascularly or both [7]. Distant dissemination is rarely described. It involves large veins almost five times more than arteries, and the most common site is the inferior vena cava and its branches [3]. It occurs in women in a large majority. The SVC localization is extremely rare, and only 15 cases have been previously reported [1, 6]. It usually results in SVC syndrome of gradual onset. Computed tomography and magnetic resonance imaging may suggest the original tumor, but the diagnosis is histologic and requires mediastinoscopy, thoracotomy, or percutaneous endovascular biopsy. Therapeutic management should be aggressive and include preoperative radiotherapy to reduce the tumor volume and adjuvant chemoradiotherapy, but is mostly based on a large surgical resection when possible [3]. Several techniques for vascular reconstruction have been described. Autologous vein may be the best graft in a low-pressure venous system. The spiral vein graft offers a sufficient graft caliber but it extends the operating time [4, 5]. The use of custom-made autologous pericardial graft or bovine pericardial graft has also been proposed [6] but not reproduced. A PTFE prosthesis is most
commonly used because of its rapid endothelialization, and it produces less thrombogenicity than polyethylene terephtalate. Furthermore, PTFE has the advantage of being reinforced by rings, reducing the risk of collapse when the venous pressure drops [2]. However, in addition to the increased risk of infection, the long-term patency of synthetic implants after reconstruction of large vessels remains unclear. There is actually no evidence to confirm the superiority of one technique over the others but according to Dartevelle and associates [8], reporting a 5-year patency rate of 86%, we believe that vascular reconstruction of the SVC using PTFE prosthesis is a valuable option. In our case, 2 years of follow-up showed graft patency and no infection. We have reported the first case of primary leiomyosarcoma involving both superior vena cava and brachiocephalic arterial trunk. Whereas large surgical resection is commonly recommended, several techniques for vascular reconstruction have been described. Our preference was for PTFE ringed vascular graft, and we obtained the outcomes of a simple postoperative course and no longterm complications.
CASE REPORT DE CHAUMONT ET AL LEIOMYOSARCOMA OF THE SUPERIOR VENA CAVA
727
References 1. Spaggiari L, Regnard JF, Nottin R, et al. Leiomyosarcoma of the superior vena cava. Ann Thorac Surg 1996;62:274–6. 2. Picquet J, Blin V, Dussaussoy C, et al. Surgical reconstruction of the superior vena cava system: indications and results. Surgery 2009;145:93–9. 3. Levett JM, Meffert WG, Strong WW, et al. Leiomyosarcoma of the superior vena cava and azygos vein. Ann Thorac Surg 1995;60:1415–7. 4. Chiu CJ, Terzis J, MacRae ML. Replacement of superior vena cava with the spiral composite vein graft. A versatile technique. Ann Thorac Surg 1974;17:555–60. 5. Doty JR, Flores JH, Doty DB. Superior vena cava obstruction: bypass using spiral vein graft. Ann Thorac Surg 1999;67:111–6. 6. Benvenuti M, Lorusso R, Gelsomino S, et al. Resection of a primary leiomyosarcoma of the superior vena cava and right atrium on a beating heart. Int J Cardiol 2011;15:151–3. 7. Tovar-Martin E, Tovar-Pardo AE, Marini M, Pimentel Y, Rois JM. Intraluminal leiomyosarcoma of the superior vena cava: a cause of superior vena cava syndrome. J Cardiovasc Surg 1997;38:33–5. 8. Dartevelle P, Chapelier A, Navajas M, et al. Replacement of the superior vena cava with polytetrafluoroethylene grafts combined with resection of mediastinal-pulmonary malignant tumors. Report of thirteen cases. J Thorac Cardiovasc Surg 1987;94:361–6.
FEATURE ARTICLES
Ann Thorac Surg 2014;98:725–7
