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Case Study
Giant primary mediastinal liposarcoma: A rare cause of atrial flutter
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315589201 aan.sagepub.com
Miguel J Arrara´s-Martı´nez1, Cristina Rieger-Reyes2, Carolina Panadero-Paz3, Hugo S Landa-Oviedo1 and Javier Garcı´a-Tirado1
Abstract We report the case of a 68-year-old man who presented with atrial flutter and was observed radiologically to have a large mass in the posterior mediastinum. During surgical removal, spontaneous recovery of sinus rhythm occurred. There was no late recurrence of arrhythmia. The diagnosis was mediastinal liposarcoma of mixed type (extremely rare). Supraventricular arrhythmia associated with mediastinal tumors is exceptional. Surgery appears to be the only potentially curative treatment for these tumors. In cases like ours presenting with arrhythmia, surgery is considered essential for control of the arrhythmia.
Keywords Atrial fibrillation, atrial flutter, liposarcoma, mediastinal neoplasms
Introduction Mediastinal liposarcomas are extremely rare (less than 1% of mediastinal tumors). Although liposarcoma is the most common soft tissue sarcoma in adults (17% of all mesenchymal tumors), it is most commonly found in the lower extremities (75%) and retroperitoneum (20%). Clinical manifestations of mediastinal liposarcoma are more often compression than invasion.1 Symptoms have been described in 85% of cases (chest pain, dyspnea, cough).2,3 Supraventricular arrhythmias associated with mediastinal tumors are also rare, having been described in the literature as associated with pleuropericardial cysts.4 We report a case of giant primary mediastinal liposarcoma presenting as atrial flutter, which we believe has never been reported previously.
Case report The patient was a 68-year-old man with a history of hypertension but no cancer history. He came to the emergency room with palpitations and was diagnosed with atrial flutter, 150 beats min 1 (Figure 1A). During the previous month, he had suffered mild dyspnea and dysphagia. Chest radiography revealed a giant
posterior mediastinal mass. Computed tomography showed a mass of 17 cm located in the posteriormiddle mediastinum, lateral to the right hemithorax, occupying the subcarinal space and displacing the adjacent organs with compression of the left atrium (Figure 2). Positron-emission tomography detected heterogeneous metabolic activity in the mass (maximum standardized uptake value 4.9; Figure 3A). Fineneedle biopsy by endoscopic esophageal ultrasound and endobronchial ultrasound revealed epithelial cells without atypia. Echocardiography confirmed left atrial displacement and compression by an adjacent mass, with an ejection fraction of 36%. Pulmonary function 1
Thoracic Surgery Department, Miguel Servet University Hospital, Zaragoza, Spain 2 Family and Community Medicine, Torre Ramona Medical Center, Multiprofessional Teaching Unit of Family and Community CareSection II, Zaragoza, Spain 3 Service of Pneumology, Miguel Servet University Hospital, Zaragoza, Spain Corresponding author: Javier Garcı´a-Tirado, Thoracic Surgery Department, Miguel Servet University Hospital, Paseo Isabel la Cato´lica 1-3, 50006 Zaragoza, Spain. Email: [email protected]
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Figure 1. (A) An electrocardiogram performed in the emergency room, showing atrial flutter with a rate of 150 beats min 1. (B) Postoperative electrocardiogram demonstrating restoration of normal sinus rhythm.
Figure 2. Computed tomography showing a mass of 17 cm in the posterior-middle mediastinum, lateral to the right hemithorax, occupying the subcarinal space and displacing the adjacent organs with compression of the left atrium and lower pulmonary veins (in particular, the lower right pulmonary vein).
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Figure 3. (A) Positron-emission tomography showing a bilobed posterior mediastinal mass (white ellipse) with heterogeneous density (no increased metabolic activity in lateral inner area). (B) Surgical specimen showing the lobulated mass (white ellipse) en bloc with the right lower lobe (black arrow).
tests were normal. With a differential diagnosis of mature germinal tumor, solitary fibrous tumor, or giant neurogenic tumor (based on esophageal ultrasound, endobronchial ultrasound, and radiological signs, and considering the result of positron-emission tomography), the committee on tumors decided on surgical treatment. After a right posterior thoracotomy, a large posterior mediastinal mass was found invading the right lower lobe of the lung, with extensive contact with the esophagus. Excision of the mass was performed by en-bloc lobectomy extended to the esophageal muscular outer layer (Figure 3B). Systematic mediastinal lymph node dissection was carried out. Intraoperatively, when the tumor was resected, the patient recovered sinus rhythm. On postoperative echocardiography, normalization of the left cavities was observed, with an ejection fraction of 50%, and an
electrocardiogram confirmed sinus rhythm (Figure 1B). The postoperative course was uneventful and antiarrhythmic treatment was suspended. With no recurrence of arrhythmia on follow-up, anticoagulant treatment was stopped 3 months later. Pathological examination revealed a mass of 17.5 13 14 cm, weighing 1009 g, and consisting of a myxoid liposarcoma with high-grade pleomorphic areas (expression of vimentin and smooth muscle actin focally; no expression of desmin, CD31, CD34, and cytokeratin AE1/ AE3; S-100 protein negative in pleomorphic areas). The resection margins and all resected lymph nodes were free of tumor involvement. The patient was treated with adjuvant chemotherapy (liposomal Adriamycin), considering the therapeutic regimens employed nowadays.5 However, a year later, he presented with several pleural and chest wall metastases
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in the right hemithorax. He was treated with a 14-day continuous infusion of high-dose ifosfamide. Nevertheless, he died 3 months later.
atrium and presenting with arrhythmia, surgery is considered essential for control of the arrhythmia.4 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Discussion Liposarcomas are classified into histologic subtypes: well differentiated (45%–50%), myxoid (30%–35%), round cell (15%–20%), pleomorphic (5%), and mixed type (as in our case). The prognosis is related to the subtype as well as the surgical resectability.1–3,5 Surgery is the basis of curative treatment, and even iterative resection is considered indicated in selected cases of relapse.3,5 These are very aggressive tumors with frequent relapses despite tumor-free surgical resection margins. Therefore, adjuvant treatment is indicated.5 In our case, adjuvant chemotherapy was given because of the pathological findings. Unusually, our patient presented with atrial flutter as the alerting symptom that led to diagnosis of the neoplasm. Supraventricular arrhythmias associated with mediastinal tumors are rare, having been described in the literature primarily associated with pleuropericardial cysts.4 Current research supports the hypothesis that initiation and maintenance of atrial arrhythmia is due to pathophysiological compromise of the left atrium and the root of the pulmonary veins.6 Our case is exceptional because of the rarity of mediastinal mixed-type liposarcoma as well as the unusual clinical presentation as atrial flutter, which resolved after resection of the tumor. We assert that the arrhythmia was caused by tumor compression, and removal of the mass resulted in resolution of the arrhythmia. In cases of mediastinal tumor (of any nature) compressing the
Conflict of interest statement The authors declare no conflict of interest.
References 1. Hirano Y, Yamamoto H, Ichimura K, Toyooka S and Miyoshi S. Surgical resection of a massive primary mediastinal liposarcoma using clamshell incision combined with lower median sternotomy: report of a case. Ann Thorac Cardiovasc Surg 2014; 20(Suppl): 606–608. 2. Boland JM, Colby TV and Folpe AL. Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features. Am J Surg Pathol 2012; 36: 1395–1403. 3. Hahn HP and Fletcher CD. Primary mediastinal liposarcoma: clinicopathologic analysis of 24 cases. Am J Surg Pathol 2007; 31: 1868–1874. 4. Generali T, Garatti A, Gagliardotto P and Frigiola A. Right mesothelial pericardial cyst determining intractable atrial arrhythmias. Interact Cardiovasc Thorac Surg 2011; 12: 837–839. 5. Kolla´r A and Benson C. Current management options for liposarcoma and challenges for the future. Expert Rev Anticancer Ther 2014; 14: 297–306. 6. Pellman J and Sheikh F. Atrial fibrillation: mechanisms, therapeutics, and future directions. Compr Physiol 2015; 5: 649–665.
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(AAN)
[1–4] [PREPRINTER stage]
Case Study
Giant primary mediastinal liposarcoma: A rare cause of atrial flutter
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315589201 aan.sagepub.com
Miguel J Arrara´s-Martı´nez1, Cristina Rieger-Reyes2, Carolina Panadero-Paz3, Hugo S Landa-Oviedo1 and Javier Garcı´a-Tirado1
Abstract We report the case of a 68-year-old man who presented with atrial flutter and was observed radiologically to have a large mass in the posterior mediastinum. During surgical removal, spontaneous recovery of sinus rhythm occurred. There was no late recurrence of arrhythmia. The diagnosis was mediastinal liposarcoma of mixed type (extremely rare). Supraventricular arrhythmia associated with mediastinal tumors is exceptional. Surgery appears to be the only potentially curative treatment for these tumors. In cases like ours presenting with arrhythmia, surgery is considered essential for control of the arrhythmia.
Keywords Atrial fibrillation, atrial flutter, liposarcoma, mediastinal neoplasms
Introduction Mediastinal liposarcomas are extremely rare (less than 1% of mediastinal tumors). Although liposarcoma is the most common soft tissue sarcoma in adults (17% of all mesenchymal tumors), it is most commonly found in the lower extremities (75%) and retroperitoneum (20%). Clinical manifestations of mediastinal liposarcoma are more often compression than invasion.1 Symptoms have been described in 85% of cases (chest pain, dyspnea, cough).2,3 Supraventricular arrhythmias associated with mediastinal tumors are also rare, having been described in the literature as associated with pleuropericardial cysts.4 We report a case of giant primary mediastinal liposarcoma presenting as atrial flutter, which we believe has never been reported previously.
Case report The patient was a 68-year-old man with a history of hypertension but no cancer history. He came to the emergency room with palpitations and was diagnosed with atrial flutter, 150 beats min 1 (Figure 1A). During the previous month, he had suffered mild dyspnea and dysphagia. Chest radiography revealed a giant
posterior mediastinal mass. Computed tomography showed a mass of 17 cm located in the posteriormiddle mediastinum, lateral to the right hemithorax, occupying the subcarinal space and displacing the adjacent organs with compression of the left atrium (Figure 2). Positron-emission tomography detected heterogeneous metabolic activity in the mass (maximum standardized uptake value 4.9; Figure 3A). Fineneedle biopsy by endoscopic esophageal ultrasound and endobronchial ultrasound revealed epithelial cells without atypia. Echocardiography confirmed left atrial displacement and compression by an adjacent mass, with an ejection fraction of 36%. Pulmonary function 1
Thoracic Surgery Department, Miguel Servet University Hospital, Zaragoza, Spain 2 Family and Community Medicine, Torre Ramona Medical Center, Multiprofessional Teaching Unit of Family and Community CareSection II, Zaragoza, Spain 3 Service of Pneumology, Miguel Servet University Hospital, Zaragoza, Spain Corresponding author: Javier Garcı´a-Tirado, Thoracic Surgery Department, Miguel Servet University Hospital, Paseo Isabel la Cato´lica 1-3, 50006 Zaragoza, Spain. Email: [email protected]
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Figure 1. (A) An electrocardiogram performed in the emergency room, showing atrial flutter with a rate of 150 beats min 1. (B) Postoperative electrocardiogram demonstrating restoration of normal sinus rhythm.
Figure 2. Computed tomography showing a mass of 17 cm in the posterior-middle mediastinum, lateral to the right hemithorax, occupying the subcarinal space and displacing the adjacent organs with compression of the left atrium and lower pulmonary veins (in particular, the lower right pulmonary vein).
Downloaded from aan.sagepub.com at UNIV PRINCE EDWARD ISLAND on September 8, 2015
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Figure 3. (A) Positron-emission tomography showing a bilobed posterior mediastinal mass (white ellipse) with heterogeneous density (no increased metabolic activity in lateral inner area). (B) Surgical specimen showing the lobulated mass (white ellipse) en bloc with the right lower lobe (black arrow).
tests were normal. With a differential diagnosis of mature germinal tumor, solitary fibrous tumor, or giant neurogenic tumor (based on esophageal ultrasound, endobronchial ultrasound, and radiological signs, and considering the result of positron-emission tomography), the committee on tumors decided on surgical treatment. After a right posterior thoracotomy, a large posterior mediastinal mass was found invading the right lower lobe of the lung, with extensive contact with the esophagus. Excision of the mass was performed by en-bloc lobectomy extended to the esophageal muscular outer layer (Figure 3B). Systematic mediastinal lymph node dissection was carried out. Intraoperatively, when the tumor was resected, the patient recovered sinus rhythm. On postoperative echocardiography, normalization of the left cavities was observed, with an ejection fraction of 50%, and an
electrocardiogram confirmed sinus rhythm (Figure 1B). The postoperative course was uneventful and antiarrhythmic treatment was suspended. With no recurrence of arrhythmia on follow-up, anticoagulant treatment was stopped 3 months later. Pathological examination revealed a mass of 17.5 13 14 cm, weighing 1009 g, and consisting of a myxoid liposarcoma with high-grade pleomorphic areas (expression of vimentin and smooth muscle actin focally; no expression of desmin, CD31, CD34, and cytokeratin AE1/ AE3; S-100 protein negative in pleomorphic areas). The resection margins and all resected lymph nodes were free of tumor involvement. The patient was treated with adjuvant chemotherapy (liposomal Adriamycin), considering the therapeutic regimens employed nowadays.5 However, a year later, he presented with several pleural and chest wall metastases
Downloaded from aan.sagepub.com at UNIV PRINCE EDWARD ISLAND on September 8, 2015
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in the right hemithorax. He was treated with a 14-day continuous infusion of high-dose ifosfamide. Nevertheless, he died 3 months later.
atrium and presenting with arrhythmia, surgery is considered essential for control of the arrhythmia.4 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Discussion Liposarcomas are classified into histologic subtypes: well differentiated (45%–50%), myxoid (30%–35%), round cell (15%–20%), pleomorphic (5%), and mixed type (as in our case). The prognosis is related to the subtype as well as the surgical resectability.1–3,5 Surgery is the basis of curative treatment, and even iterative resection is considered indicated in selected cases of relapse.3,5 These are very aggressive tumors with frequent relapses despite tumor-free surgical resection margins. Therefore, adjuvant treatment is indicated.5 In our case, adjuvant chemotherapy was given because of the pathological findings. Unusually, our patient presented with atrial flutter as the alerting symptom that led to diagnosis of the neoplasm. Supraventricular arrhythmias associated with mediastinal tumors are rare, having been described in the literature primarily associated with pleuropericardial cysts.4 Current research supports the hypothesis that initiation and maintenance of atrial arrhythmia is due to pathophysiological compromise of the left atrium and the root of the pulmonary veins.6 Our case is exceptional because of the rarity of mediastinal mixed-type liposarcoma as well as the unusual clinical presentation as atrial flutter, which resolved after resection of the tumor. We assert that the arrhythmia was caused by tumor compression, and removal of the mass resulted in resolution of the arrhythmia. In cases of mediastinal tumor (of any nature) compressing the
Conflict of interest statement The authors declare no conflict of interest.
References 1. Hirano Y, Yamamoto H, Ichimura K, Toyooka S and Miyoshi S. Surgical resection of a massive primary mediastinal liposarcoma using clamshell incision combined with lower median sternotomy: report of a case. Ann Thorac Cardiovasc Surg 2014; 20(Suppl): 606–608. 2. Boland JM, Colby TV and Folpe AL. Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features. Am J Surg Pathol 2012; 36: 1395–1403. 3. Hahn HP and Fletcher CD. Primary mediastinal liposarcoma: clinicopathologic analysis of 24 cases. Am J Surg Pathol 2007; 31: 1868–1874. 4. Generali T, Garatti A, Gagliardotto P and Frigiola A. Right mesothelial pericardial cyst determining intractable atrial arrhythmias. Interact Cardiovasc Thorac Surg 2011; 12: 837–839. 5. Kolla´r A and Benson C. Current management options for liposarcoma and challenges for the future. Expert Rev Anticancer Ther 2014; 14: 297–306. 6. Pellman J and Sheikh F. Atrial fibrillation: mechanisms, therapeutics, and future directions. Compr Physiol 2015; 5: 649–665.
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