Complete Resection of a Giant Mediastinal Leiomyosarcoma Fengshi Chen, MD, Yusuke Muranishi, MD, Kenji Minakata, MD, Kojiro Taura, MD, Hiroshi Okabe, MD, Toshiyuki Mizota, MD, Makoto Sonobe, MD, and Hiroshi Date, MD Departments of Thoracic Surgery, Cardiovascular Surgery, Surgery, and Anesthesia, Kyoto University Graduate School of Medicine, Kyoto, Japan
Primary mediastinal leiomyosarcoma is an extremely rare malignancy, and the only opportunity for a cure lies with an aggressive surgical approach. We report a 66-year-old woman who underwent complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm. The tumor encased the inferior vena cava and compressed the adjacent structures. Using cardiopulmonary bypass with 20 minutes of hepatic ischemia, the tumor was completely resected with combined resection and reconstruction of the surrounding structures. Because of the tumor size and location in the boundary area between thoracic and abdominal surgeries, the procedure necessitated the cooperation of many expert surgeons. (Ann Thorac Surg 2015;99:e69–71) Ó 2015 by The Society of Thoracic Surgeons
P
rimary mediastinal leiomyosarcoma is an extremely rare malignancy [1, 2], and complete resection is the most significant factor for survival [3–5]. Herein, we report a case of complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm with combined resection and reconstruction of the adjacent structures. A 66-year-old woman presented with acute chest pain. Chest computed tomography showed a huge mediastinal mass with the largest diameter of 21 cm located on the bilateral diaphragm. The inferior vena cava (IVC) was almost entirely surrounded by the tumor circum-
Accepted for publication Dec 8, 2014. Address correspondence to Dr Date, Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin Kawaharacho, Sakyo-ku, Kyoto 606-8507, Japan; e-mail: [email protected].
ferentially (Fig 1A). Although the branches of the hepatic veins were also surrounded by the tumor (Fig 1B), magnetic resonance imaging did not show any tumor invasion across the diaphragm into the liver. The esophagus was similarly displaced, but upper gastrointestinal endoscopy showed only compression without evidence of luminal involvement. The mass was diagnosed as a leiomyosarcoma by endoscopic ultrasound-guided fine-needle aspiration. As complete surgical resection was considered the only curative treatment option, we organized a special team to discuss the operability of the tumor and the surgical strategy for complete resection. Eventually, we agreed that exploratory surgery for complete resection using cardiopulmonary bypass should be performed as the only curative treatment option unless the tumor invades the liver directly. Thoracotomy and laparotomy were performed through a bilateral clamshell, and median and right lateral abdominal incisions, respectively. First, we confirmed that the tumor did not directly invade the liver (Fig 2A). The tumor was fused to the lungs, requiring a partial resection of the right middle and lower lobes. Because of the adhesion between the tumor and the diaphragm, combined partial resection of the diaphragm was mandatory. The right phrenic nerve was invaded by the tumor and was resected, but the left phrenic nerve was carefully preserved. The esophagus was completely dissected from the tumor despite adhesions. Cardiopulmonary bypass was established with cannulation of the ascending aorta and right atrium. Another venous cannula was inserted into the IVC at a level just distal to the hepatic vein confluence through the right femoral vein. After cardiopulmonary bypass was established, the IVC was transected with a vascular stapler at the level of the IVC confluence into the right atrium (Fig 2A). This facilitated dissection of the posterior side of the tumor, providing excellent exposure for the dissection of the suprahepatic IVC from the tumor at the level of hepatic vein confluence, and this allowed enough transection of the suprahepatic IVC cranial to avoid complicated reconstruction of the IVC and hepatic veins. Under clamping of the infrahepatic IVC and hepatoduodenal ligament, the suprahepatic IVC was transected without placing a vascular clamp, and the tumor was extracted en bloc with combined partial resection of the bilateral Fig 1. (A) Transverse section of contrastenhanced chest computed tomography showing a huge mediastinal mass with the largest diameter of 21 cm located on the bilateral diaphragm. The inferior vena cava was surrounded almost entirely by the tumor circumferentially below the right atrium (arrow). (B) Coronal section of contrastenhanced chest computed tomography showing a huge mediastinal mass on the bilateral diaphragm. The branches of the hepatic veins were also surrounded by the tumor (arrow). The esophagus was also compressed extensively.
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.12.024
e70
CASE REPORT CHEN ET AL GIANT MEDIASTINAL LEIOMYOSARCOMA
Ann Thorac Surg 2015;99:e69–71
Fig 2. Diagram of the surgical field (A) before tumor resection and (B) during replacement of the inferior vena cava (IVC). (PTFE ¼ polytetrafluoroethylene.)
diaphragm, lungs, right phrenic nerve, pericardium, and IVC (Fig 3). The IVC was subsequently reconstructed using a polytetrafluoroethylene prosthesis (Fig 2B). The distal anastomosis was carried out primarily. The polytetrafluoroethylene graft was then occluded with a vascular clamp to allow release of the infrahepatic vascular clamps and minimize ischemic time of the liver (20 minutes). Then the proximal anastomosis was carried out and the cardiopulmonary bypass was weaned smoothly. Reconstruction of the bilateral diaphragm and pericardium was performed using polytetrafluoroethylene prosthesis. The cardiopulmonary bypass time was 95 minutes, and total operation time was 11 hours. The postoperative course was uneventful. She could be extubated on the day after the operation. She did not
show any gastrointestinal problems and was discharged home 3 weeks postoperatively. Histologic examination of the tumor showed no tumor invasion into the surrounding organs, which resulted in a diagnosis of primary mediastinal leiomyosarcoma arising from the visceral mediastinum. Now, 3 months after the operation, she is well without any limitations in her daily life.
Comment As long-term prognosis in mediastinal leiomyosarcoma depends on the ability to completely resect the tumor, surgeons must know that the only opportunity for a cure lies with a very aggressive surgical approach [3–5]. In this patient, the tumor was diagnosed as a primary mediastinal leiomyosarcoma originating from the visceral mediastinum [1, 5]. Located on the bilateral diaphragm, this tumor encased and compressed many surrounding structures. Because of the tumor’s size and location in the boundary between thoracic and abdominal surgeries, the procedure necessitated the cooperation of expert surgeons from four departments. Because of the adhesions to the surrounding structures, a single surgeon could not accomplish the whole procedure. In conclusion, we report a case of complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm with combined resection and reconstruction of the surrounding structures.
References Fig 3. Macroscopic view of the tumor extracted en bloc with combined resection of one third of the bilateral diaphragm, a part of the lungs, a part of the right phrenic nerve, one half of the pericardium, and a part of the cylindrical inferior vena cava.
1. Moran CA, Suster S, Perino G, Kaneko M, Koss MN. Malignant smooth muscle tumors presenting as mediastinal soft tissue masses: a clinicopathologic study of 10 cases. Cancer 1994;74:2251–60.
Ann Thorac Surg 2015;99:e69–71
2. Burt M, Ihde JK, Hajdu SI, et al. Primary sarcomas of the mediastinum: results of therapy. J Thorac Cardiovasc Surg 1998;115:671–80. 3. D’Auito M, Veronesi G, Pompilio G, et al. Extended right pneumonectomy with partial left atrial resection for primary leiomyosarcoma of the mediastinum. J Thorac Cardiovasc Surg 2005;129:694–5.
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4. Conner WC, Fink GW, McGinnis KM, Alfieris GM. Surgical management of leiomyosarcoma of the mediastinum. Ann Thorac Surg 2004;77:334–6. 5. Iwata T, Miura T, Inoue K, Hanada S, Inoue H, Miyamoto Y. Primary leiomyosarcoma of the anterior mediastinum encasing the aortic arch, left common carotid and left subclavian arteries. Ann Thorac Cardiovasc Surg 2012;18:140–3.
Primary mediastinal leiomyosarcoma is an extremely rare malignancy, and the only opportunity for a cure lies with an aggressive surgical approach. We report a 66-year-old woman who underwent complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm. The tumor encased the inferior vena cava and compressed the adjacent structures. Using cardiopulmonary bypass with 20 minutes of hepatic ischemia, the tumor was completely resected with combined resection and reconstruction of the surrounding structures. Because of the tumor size and location in the boundary area between thoracic and abdominal surgeries, the procedure necessitated the cooperation of many expert surgeons. (Ann Thorac Surg 2015;99:e69–71) Ó 2015 by The Society of Thoracic Surgeons
P
rimary mediastinal leiomyosarcoma is an extremely rare malignancy [1, 2], and complete resection is the most significant factor for survival [3–5]. Herein, we report a case of complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm with combined resection and reconstruction of the adjacent structures. A 66-year-old woman presented with acute chest pain. Chest computed tomography showed a huge mediastinal mass with the largest diameter of 21 cm located on the bilateral diaphragm. The inferior vena cava (IVC) was almost entirely surrounded by the tumor circum-
Accepted for publication Dec 8, 2014. Address correspondence to Dr Date, Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin Kawaharacho, Sakyo-ku, Kyoto 606-8507, Japan; e-mail: [email protected].
ferentially (Fig 1A). Although the branches of the hepatic veins were also surrounded by the tumor (Fig 1B), magnetic resonance imaging did not show any tumor invasion across the diaphragm into the liver. The esophagus was similarly displaced, but upper gastrointestinal endoscopy showed only compression without evidence of luminal involvement. The mass was diagnosed as a leiomyosarcoma by endoscopic ultrasound-guided fine-needle aspiration. As complete surgical resection was considered the only curative treatment option, we organized a special team to discuss the operability of the tumor and the surgical strategy for complete resection. Eventually, we agreed that exploratory surgery for complete resection using cardiopulmonary bypass should be performed as the only curative treatment option unless the tumor invades the liver directly. Thoracotomy and laparotomy were performed through a bilateral clamshell, and median and right lateral abdominal incisions, respectively. First, we confirmed that the tumor did not directly invade the liver (Fig 2A). The tumor was fused to the lungs, requiring a partial resection of the right middle and lower lobes. Because of the adhesion between the tumor and the diaphragm, combined partial resection of the diaphragm was mandatory. The right phrenic nerve was invaded by the tumor and was resected, but the left phrenic nerve was carefully preserved. The esophagus was completely dissected from the tumor despite adhesions. Cardiopulmonary bypass was established with cannulation of the ascending aorta and right atrium. Another venous cannula was inserted into the IVC at a level just distal to the hepatic vein confluence through the right femoral vein. After cardiopulmonary bypass was established, the IVC was transected with a vascular stapler at the level of the IVC confluence into the right atrium (Fig 2A). This facilitated dissection of the posterior side of the tumor, providing excellent exposure for the dissection of the suprahepatic IVC from the tumor at the level of hepatic vein confluence, and this allowed enough transection of the suprahepatic IVC cranial to avoid complicated reconstruction of the IVC and hepatic veins. Under clamping of the infrahepatic IVC and hepatoduodenal ligament, the suprahepatic IVC was transected without placing a vascular clamp, and the tumor was extracted en bloc with combined partial resection of the bilateral Fig 1. (A) Transverse section of contrastenhanced chest computed tomography showing a huge mediastinal mass with the largest diameter of 21 cm located on the bilateral diaphragm. The inferior vena cava was surrounded almost entirely by the tumor circumferentially below the right atrium (arrow). (B) Coronal section of contrastenhanced chest computed tomography showing a huge mediastinal mass on the bilateral diaphragm. The branches of the hepatic veins were also surrounded by the tumor (arrow). The esophagus was also compressed extensively.
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.12.024
e70
CASE REPORT CHEN ET AL GIANT MEDIASTINAL LEIOMYOSARCOMA
Ann Thorac Surg 2015;99:e69–71
Fig 2. Diagram of the surgical field (A) before tumor resection and (B) during replacement of the inferior vena cava (IVC). (PTFE ¼ polytetrafluoroethylene.)
diaphragm, lungs, right phrenic nerve, pericardium, and IVC (Fig 3). The IVC was subsequently reconstructed using a polytetrafluoroethylene prosthesis (Fig 2B). The distal anastomosis was carried out primarily. The polytetrafluoroethylene graft was then occluded with a vascular clamp to allow release of the infrahepatic vascular clamps and minimize ischemic time of the liver (20 minutes). Then the proximal anastomosis was carried out and the cardiopulmonary bypass was weaned smoothly. Reconstruction of the bilateral diaphragm and pericardium was performed using polytetrafluoroethylene prosthesis. The cardiopulmonary bypass time was 95 minutes, and total operation time was 11 hours. The postoperative course was uneventful. She could be extubated on the day after the operation. She did not
show any gastrointestinal problems and was discharged home 3 weeks postoperatively. Histologic examination of the tumor showed no tumor invasion into the surrounding organs, which resulted in a diagnosis of primary mediastinal leiomyosarcoma arising from the visceral mediastinum. Now, 3 months after the operation, she is well without any limitations in her daily life.
Comment As long-term prognosis in mediastinal leiomyosarcoma depends on the ability to completely resect the tumor, surgeons must know that the only opportunity for a cure lies with a very aggressive surgical approach [3–5]. In this patient, the tumor was diagnosed as a primary mediastinal leiomyosarcoma originating from the visceral mediastinum [1, 5]. Located on the bilateral diaphragm, this tumor encased and compressed many surrounding structures. Because of the tumor’s size and location in the boundary between thoracic and abdominal surgeries, the procedure necessitated the cooperation of expert surgeons from four departments. Because of the adhesions to the surrounding structures, a single surgeon could not accomplish the whole procedure. In conclusion, we report a case of complete resection of a giant mediastinal leiomyosarcoma located on the bilateral diaphragm with combined resection and reconstruction of the surrounding structures.
References Fig 3. Macroscopic view of the tumor extracted en bloc with combined resection of one third of the bilateral diaphragm, a part of the lungs, a part of the right phrenic nerve, one half of the pericardium, and a part of the cylindrical inferior vena cava.
1. Moran CA, Suster S, Perino G, Kaneko M, Koss MN. Malignant smooth muscle tumors presenting as mediastinal soft tissue masses: a clinicopathologic study of 10 cases. Cancer 1994;74:2251–60.
Ann Thorac Surg 2015;99:e69–71
2. Burt M, Ihde JK, Hajdu SI, et al. Primary sarcomas of the mediastinum: results of therapy. J Thorac Cardiovasc Surg 1998;115:671–80. 3. D’Auito M, Veronesi G, Pompilio G, et al. Extended right pneumonectomy with partial left atrial resection for primary leiomyosarcoma of the mediastinum. J Thorac Cardiovasc Surg 2005;129:694–5.
CASE REPORT CHEN ET AL GIANT MEDIASTINAL LEIOMYOSARCOMA
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4. Conner WC, Fink GW, McGinnis KM, Alfieris GM. Surgical management of leiomyosarcoma of the mediastinum. Ann Thorac Surg 2004;77:334–6. 5. Iwata T, Miura T, Inoue K, Hanada S, Inoue H, Miyamoto Y. Primary leiomyosarcoma of the anterior mediastinum encasing the aortic arch, left common carotid and left subclavian arteries. Ann Thorac Cardiovasc Surg 2012;18:140–3.
