- Email: [email protected]
Primary Angiosarcoma of Pericardium with Cardiac Tamponade: A Case Report
International Journal of Gerontology 9 (2015) 126e129
Contents lists available at ScienceDirect
International Journal of Gerontology journal homepage: www.ijge-online.com
Case Report
Primary Angiosarcoma of Pericardium with Cardiac Tamponade: A Case Report* Wei-Ji Chen 1, Cheng-Huang Su 2, Chi-Kuan Chen 3, Chang-Yi Lin 1 * 1
Chest Division,
2
Cardiovascular Division, Department of Internal Medicine,
3
Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan
a r t i c l e i n f o
s u m m a r y
Article history: Received 9 May 2013 Received in revised form 15 October 2013 Accepted 25 November 2013 Available online 4 June 2015
Primary tumors of the heart and major vessels are rare. Angiosarcoma is the most frequent malignant tumor of the heart and usually involves the right atrium. Angiosarcoma originating from the pericardium is extremely rare and only several cases have been reported to date. The current study presents a case of primary angiosarcoma of pericardium with cardiac tamponade. After surgical treatment to relieve symptoms, the patient refused further therapies and died 3 months after diagnosis. Copyright © 2015, Taiwan Society of Geriatric Emergency & Critical Care Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: angiosarcoma, pericardium
1. Introduction Primary malignant tumors of the heart and major vessels are rare. Amongst the malignant tumors of the heart, angiosarcoma is the most common and accounts for more than two thirds of these malignancies1,2. Angiosarcoma frequently involve the cardiac chambers, especially the right atrium. However, detection of angiosarcoma of the pericardium is very rare3. The clinical presentation of pericardial angiosarcoma depends on the location of involvement rather than histological type. The prognosis for a patient with angiosarcoma is poor with an overall survival duration of between 6 months and 12 months even under multi-modality treatments1. Patient outcome is improved if chemotherapy is applied after surgical resection. There are several reports of patients' surviving up to 40 months. 2. Case report An 83-year-old male patient presented with a history of atrial fibrillation, chronic ischemic heart disease, and congestive heart failure, for several years. He received regular medical control at out-
* Conflicts of interest: All contributing authors declare that they have no conflicts of interest. * Correspondence to: Dr Chang-Yi Lin, Chest Division, Department of Internal Medicine, Mackay Memorial Hospital, 92, Section 2, Chun-Shan North Road, Taipei, Taiwan. E-mail address: [email protected] (C.-Y. Lin).
patient clinics. One week before admission, progressive dyspnea on exertion, a dry cough, and a worsened low leg edema were present. He was brought to the out-patients department for first aid. A chest X-ray revealed a huge, bottle-shaped heart shadow (Fig. 1). Electrocardiogram revealed atrial fibrillation with diffuse low voltage amplitude. Routine laboratory studies were normal. Radiological findings indicated pericardial tamponade. An emergency transthoracic echocardiogram was arranged and showed a massive pericardial effusion with cardiac tamponade. Pericardiocentesis and drainage were performed and 500 mL hemorrhagic pericardial fluid was drained. The patient's symptoms were rapidly relieved. Cytological and bacterial examinations of the pericardial effusions were all negative. Chest computerized tomography (CT) was arranged on suspicion of a malignant tumor, and showed a 4.7 cm 2.5 cm soft tissue nodule present at the pericardial space along the left atrioventricular groove. A 3 cm 1.5 cm lesion next to the lateral wall of the left ventricular wall associated with mild pericardial effusion, and multiple mediastinal lymphadenopathy was also detected (Fig. 2). A positron emission tomography (PET) scan further confirmed a pericardial malignancy with mediastinal lymph node metastasis (Fig. 3). Surgical resection of the pericardial tumor was later performed. The pathology indicated multiple nodular proliferations on the pericardium. Tumor cells were strongly positive for CD31; weakly positive for WT-1; and negative for cytokeratin (AE1/AE3), HMB-45, and S-100 (Fig. 4). All these findings are consistent with angiosarcoma. Adjuvant chemotherapy and radiation therapies were recommended but the patient refused all treatments. He died following tumor recurrence 3 months later.
http://dx.doi.org/10.1016/j.ijge.2015.05.012 1873-9598/Copyright © 2015, Taiwan Society of Geriatric Emergency & Critical Care Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Primary Angiosarcoma of Pericardium with Cardiac Tamponade
Fig. 1. Chest X-ray reveals bottle-shaped and enlarged heart size.
3. Discussion Primary cardiac tumors are rare; their incidence was estimated to be 0.0017e0.003% in a study of routine autopsy1. The majority of cardiac tumors are benign with myxoma being the most common benign tumor of the heart (50%), followed by lipoma (~25%)4.
Fig. 2. A computed tomography scan reveals a 4.7 cm 2.5 cm soft tissue nodule present in the pericardial space along the left atrioventricular groove with mild pericardial effusion.
127
Malignant cardiac tumors are estimated to account for 25% of all cardiac tumors5. Angiosarcoma is the most frequent malignant tumor of the heart. Malignant cardiac tumors frequently tend to involve the pericardium and right heart chambers, especially the posterior wall of the right atrium, as was seen in a report of 119 cases by Meng et al4. The prevalence does not differ by sex1, however; a greater tendency was reported in male patients6. Angiosarcoma is the most common malignant tumor of the heart and its incidence is estimated to be about 31% of all malignant tumors. Angiosarcoma tends to occur in people aged 30e50 years. The initial diagnosis of angiosarcoma is usually delayed because of its nonspecific characteristics and detection depends on the surgical biopsy of tumors. Symptoms of angiosarcoma include dyspnea, chest discomfort, heart failure, palpitations, and general weakness. The most common site of involvement is the right atrium, followed by left atrium, right ventricle, and left ventricle7. Metastasis is about 80% when angiosarcoma is diagnosed. The lungs, mediastinal lymph nodes, and vertebrae are the most common metastatic sites. A chest radiograph may disclose cardiomegaly, pulmonary congestion, or pericardial effusion. Transthoracic echocardiography is also an important tool used to detect cardiac tumors. Transesophageal echocardiography might have better ability to delineate the tumor location and the severity of local infiltration. Chest CT is useful to identify metastasis. Cytological analysis of pericardial effusion is also helpful; with the positive rate ranging from 75% to 87%.A transvenous endocardial biopsy procedure was reported in one study8. Despite all the above methods, surgical biopsy is always necessary to yield an adequate sample for diagnosis. Immunohistochemical stains are positive for CD31, CD34, and Factor VIII, which are characteristic features of vascular origin. The overall average survival time is between 6 months and 12 months. Rare cases may survive up to 2e4 years. Tumor staging of cardiac angiosarcoma is currently not available, which may be due to its rarity. There is also a lack of large studies to compare the effects of different therapies in combination. Surgical resection remains important in order to examine the tissue and for rapid relief of symptoms. Even though total surgical resection is the treatment of choice, recurrence is common6. Angiosarcoma generally responds poorly to chemotherapy. The most frequently used chemotherapy drugs include Adriamycin, ifosfamide, cyclophosphamide, vincristine, and dacarbazine. There are not many studies comparing different chemotherapeutic regimens because of the rarity of cardiac angiosarcoma. Adriamycin-based regimens are the most commonly used regimens by oncologists. Pegylated liposomal doxorubicin has been used for the treatment of Kaposi's sarcoma in AIDS, its use was also reported for the treatment of angiosarcoma in one study9. Both Kaposi's sarcoma and angiosarcoma are tumors of the endothelial cells and may share some biological properties10. Recently, paclitaxel combined with liposomal doxorubicin has also been reported to demonstrate favorable results in the treatment of angiosarcoma11. Even though the overall response is poor with chemotherapy, patients treated with chemotherapy had better survival outcomes than did those without chemotherapy12. Adjuvant radiotherapy has a limited role of therapy due to its cardiotoxicity. It may play a role in patients with brain metastasis or superior vena cava syndromes13. Patients who avail of multidisciplinary therapies survived longer than those who were treated with a single therapy6,12. Overall survival may be up to 19 months in patients who undergo surgical resection and chemotherapy. Orthotopic cardiac transplantation has been tried in rare cases of angiosarcoma but patient survival did not differ from those without transplantation14. In conclusion, pericardial tamponade is an emergent medical condition which could be caused by a number of systemic diseases
128
W.-J. Chen et al.
Fig. 3. The F-18 FDG whole body positron emission tomography scan reveals pericardial malignancy with mediastinal lymph nodes metastasis.
Primary Angiosarcoma of Pericardium with Cardiac Tamponade
129
Fig. 4. (A, B) Pathology shows the tumor is composed of spindle to pleomorphic cells; (C) immunohistochemical staining is positive for CD31; and (D) immunohistochemical staining is negative for cytokeratin (AE1/AE3).
such as uremia or cancer to name but a few. Apart from metastatic cancers, primary cardiac tumors, including pericardial angiosarcoma, should also be suspected. Although the outcome of angiosarcoma is poor, multiple modalities therapies, including surgical resection, chemotherapy, and radiotherapy, should be considered to prolong survival time. References 1. Odim J, Reehal V, Laks H, et al. Surgical pathology of cardiac tumors. Two decades at an urban institution. Cardiovasc Pathol. 2003;12:267e270. 2. Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol. 1994;10:374e382. 3. Yao H, Miyamoto T, Mukai S, et al. Angiosarcoma of the pericardium. Review of 9 reports from Japan. Jpn J Thorac Cardiovasc Surg. 2003;51:65e67. 4. Meng Q, Lai H, Lima J, et al. Echocardiographic and pathologic characteristics of primary cardiac tumors: A study of 149 cases. Int J Cardiol. 2002;84:69e75. 5. Silverman NA. Primary cardiac tumors. Ann Surg. 1980;191:127e138. 6. Look Hong NJ, Pandalai PK, Hornick JL, et al. Cardiac angiosarcoma management and outcomes: 20-year single-institution experience. Ann Surg Oncol. 2012;19:2707e2715.
7. Zhang PJ, Brooks JS, Goldblum JR, et al. Primary cardiac sarcomas: A clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol. 2008;39:1385e1395. 8. Hosokawa Y, Kodani E, Kusama Y, et al. Cardiac angiosarcoma diagnosed by transvenous endomyocardial biopsy with the aid of transesophageal echocardiography and intra-procedural consultation. Int Heart J. 2010;51:367e369. 9. Judson I, Radford JA, Harris M, et al. Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: A study by the EORTC soft tissue and bone sarcoma group. Eur J Cancer. 2001;37:870e877. 10. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer. 1986;57:852e859. 11. Bafaloukos D, Papadimitriou C, Linardou H, et al. Combination of pegylated liposomal doxorubicin (PLD) and paclitaxel in patients with advanced soft tissue sarcoma: A phase II study of the hellenic cooperative oncology group. Br J Cancer. 2004;91:1639e1644. 12. Fatima J, Duncan AA, Maleszewski JJ, et al. Primary angiosarcoma of the aorta, great vessels, and the heart. J Vasc Surg. 2013;57:756e764. 13. Sorlie D, Myhre ES, Stalsberg H. Angiosarcoma of the heart. Unusual presentation and survival after treatment. Br Heart J. 1984;51:94e97. 14. Crespo MG, Pulpon LA, Pradas G, et al. Heart transplantation for cardiac angiosarcoma: Should its indication be questioned? J Heart Lung Transplant. 1993;12:527e530.
Contents lists available at ScienceDirect
International Journal of Gerontology journal homepage: www.ijge-online.com
Case Report
Primary Angiosarcoma of Pericardium with Cardiac Tamponade: A Case Report* Wei-Ji Chen 1, Cheng-Huang Su 2, Chi-Kuan Chen 3, Chang-Yi Lin 1 * 1
Chest Division,
2
Cardiovascular Division, Department of Internal Medicine,
3
Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan
a r t i c l e i n f o
s u m m a r y
Article history: Received 9 May 2013 Received in revised form 15 October 2013 Accepted 25 November 2013 Available online 4 June 2015
Primary tumors of the heart and major vessels are rare. Angiosarcoma is the most frequent malignant tumor of the heart and usually involves the right atrium. Angiosarcoma originating from the pericardium is extremely rare and only several cases have been reported to date. The current study presents a case of primary angiosarcoma of pericardium with cardiac tamponade. After surgical treatment to relieve symptoms, the patient refused further therapies and died 3 months after diagnosis. Copyright © 2015, Taiwan Society of Geriatric Emergency & Critical Care Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Keywords: angiosarcoma, pericardium
1. Introduction Primary malignant tumors of the heart and major vessels are rare. Amongst the malignant tumors of the heart, angiosarcoma is the most common and accounts for more than two thirds of these malignancies1,2. Angiosarcoma frequently involve the cardiac chambers, especially the right atrium. However, detection of angiosarcoma of the pericardium is very rare3. The clinical presentation of pericardial angiosarcoma depends on the location of involvement rather than histological type. The prognosis for a patient with angiosarcoma is poor with an overall survival duration of between 6 months and 12 months even under multi-modality treatments1. Patient outcome is improved if chemotherapy is applied after surgical resection. There are several reports of patients' surviving up to 40 months. 2. Case report An 83-year-old male patient presented with a history of atrial fibrillation, chronic ischemic heart disease, and congestive heart failure, for several years. He received regular medical control at out-
* Conflicts of interest: All contributing authors declare that they have no conflicts of interest. * Correspondence to: Dr Chang-Yi Lin, Chest Division, Department of Internal Medicine, Mackay Memorial Hospital, 92, Section 2, Chun-Shan North Road, Taipei, Taiwan. E-mail address: [email protected] (C.-Y. Lin).
patient clinics. One week before admission, progressive dyspnea on exertion, a dry cough, and a worsened low leg edema were present. He was brought to the out-patients department for first aid. A chest X-ray revealed a huge, bottle-shaped heart shadow (Fig. 1). Electrocardiogram revealed atrial fibrillation with diffuse low voltage amplitude. Routine laboratory studies were normal. Radiological findings indicated pericardial tamponade. An emergency transthoracic echocardiogram was arranged and showed a massive pericardial effusion with cardiac tamponade. Pericardiocentesis and drainage were performed and 500 mL hemorrhagic pericardial fluid was drained. The patient's symptoms were rapidly relieved. Cytological and bacterial examinations of the pericardial effusions were all negative. Chest computerized tomography (CT) was arranged on suspicion of a malignant tumor, and showed a 4.7 cm 2.5 cm soft tissue nodule present at the pericardial space along the left atrioventricular groove. A 3 cm 1.5 cm lesion next to the lateral wall of the left ventricular wall associated with mild pericardial effusion, and multiple mediastinal lymphadenopathy was also detected (Fig. 2). A positron emission tomography (PET) scan further confirmed a pericardial malignancy with mediastinal lymph node metastasis (Fig. 3). Surgical resection of the pericardial tumor was later performed. The pathology indicated multiple nodular proliferations on the pericardium. Tumor cells were strongly positive for CD31; weakly positive for WT-1; and negative for cytokeratin (AE1/AE3), HMB-45, and S-100 (Fig. 4). All these findings are consistent with angiosarcoma. Adjuvant chemotherapy and radiation therapies were recommended but the patient refused all treatments. He died following tumor recurrence 3 months later.
http://dx.doi.org/10.1016/j.ijge.2015.05.012 1873-9598/Copyright © 2015, Taiwan Society of Geriatric Emergency & Critical Care Medicine. Published by Elsevier Taiwan LLC. All rights reserved.
Primary Angiosarcoma of Pericardium with Cardiac Tamponade
Fig. 1. Chest X-ray reveals bottle-shaped and enlarged heart size.
3. Discussion Primary cardiac tumors are rare; their incidence was estimated to be 0.0017e0.003% in a study of routine autopsy1. The majority of cardiac tumors are benign with myxoma being the most common benign tumor of the heart (50%), followed by lipoma (~25%)4.
Fig. 2. A computed tomography scan reveals a 4.7 cm 2.5 cm soft tissue nodule present in the pericardial space along the left atrioventricular groove with mild pericardial effusion.
127
Malignant cardiac tumors are estimated to account for 25% of all cardiac tumors5. Angiosarcoma is the most frequent malignant tumor of the heart. Malignant cardiac tumors frequently tend to involve the pericardium and right heart chambers, especially the posterior wall of the right atrium, as was seen in a report of 119 cases by Meng et al4. The prevalence does not differ by sex1, however; a greater tendency was reported in male patients6. Angiosarcoma is the most common malignant tumor of the heart and its incidence is estimated to be about 31% of all malignant tumors. Angiosarcoma tends to occur in people aged 30e50 years. The initial diagnosis of angiosarcoma is usually delayed because of its nonspecific characteristics and detection depends on the surgical biopsy of tumors. Symptoms of angiosarcoma include dyspnea, chest discomfort, heart failure, palpitations, and general weakness. The most common site of involvement is the right atrium, followed by left atrium, right ventricle, and left ventricle7. Metastasis is about 80% when angiosarcoma is diagnosed. The lungs, mediastinal lymph nodes, and vertebrae are the most common metastatic sites. A chest radiograph may disclose cardiomegaly, pulmonary congestion, or pericardial effusion. Transthoracic echocardiography is also an important tool used to detect cardiac tumors. Transesophageal echocardiography might have better ability to delineate the tumor location and the severity of local infiltration. Chest CT is useful to identify metastasis. Cytological analysis of pericardial effusion is also helpful; with the positive rate ranging from 75% to 87%.A transvenous endocardial biopsy procedure was reported in one study8. Despite all the above methods, surgical biopsy is always necessary to yield an adequate sample for diagnosis. Immunohistochemical stains are positive for CD31, CD34, and Factor VIII, which are characteristic features of vascular origin. The overall average survival time is between 6 months and 12 months. Rare cases may survive up to 2e4 years. Tumor staging of cardiac angiosarcoma is currently not available, which may be due to its rarity. There is also a lack of large studies to compare the effects of different therapies in combination. Surgical resection remains important in order to examine the tissue and for rapid relief of symptoms. Even though total surgical resection is the treatment of choice, recurrence is common6. Angiosarcoma generally responds poorly to chemotherapy. The most frequently used chemotherapy drugs include Adriamycin, ifosfamide, cyclophosphamide, vincristine, and dacarbazine. There are not many studies comparing different chemotherapeutic regimens because of the rarity of cardiac angiosarcoma. Adriamycin-based regimens are the most commonly used regimens by oncologists. Pegylated liposomal doxorubicin has been used for the treatment of Kaposi's sarcoma in AIDS, its use was also reported for the treatment of angiosarcoma in one study9. Both Kaposi's sarcoma and angiosarcoma are tumors of the endothelial cells and may share some biological properties10. Recently, paclitaxel combined with liposomal doxorubicin has also been reported to demonstrate favorable results in the treatment of angiosarcoma11. Even though the overall response is poor with chemotherapy, patients treated with chemotherapy had better survival outcomes than did those without chemotherapy12. Adjuvant radiotherapy has a limited role of therapy due to its cardiotoxicity. It may play a role in patients with brain metastasis or superior vena cava syndromes13. Patients who avail of multidisciplinary therapies survived longer than those who were treated with a single therapy6,12. Overall survival may be up to 19 months in patients who undergo surgical resection and chemotherapy. Orthotopic cardiac transplantation has been tried in rare cases of angiosarcoma but patient survival did not differ from those without transplantation14. In conclusion, pericardial tamponade is an emergent medical condition which could be caused by a number of systemic diseases
128
W.-J. Chen et al.
Fig. 3. The F-18 FDG whole body positron emission tomography scan reveals pericardial malignancy with mediastinal lymph nodes metastasis.
Primary Angiosarcoma of Pericardium with Cardiac Tamponade
129
Fig. 4. (A, B) Pathology shows the tumor is composed of spindle to pleomorphic cells; (C) immunohistochemical staining is positive for CD31; and (D) immunohistochemical staining is negative for cytokeratin (AE1/AE3).
such as uremia or cancer to name but a few. Apart from metastatic cancers, primary cardiac tumors, including pericardial angiosarcoma, should also be suspected. Although the outcome of angiosarcoma is poor, multiple modalities therapies, including surgical resection, chemotherapy, and radiotherapy, should be considered to prolong survival time. References 1. Odim J, Reehal V, Laks H, et al. Surgical pathology of cardiac tumors. Two decades at an urban institution. Cardiovasc Pathol. 2003;12:267e270. 2. Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol. 1994;10:374e382. 3. Yao H, Miyamoto T, Mukai S, et al. Angiosarcoma of the pericardium. Review of 9 reports from Japan. Jpn J Thorac Cardiovasc Surg. 2003;51:65e67. 4. Meng Q, Lai H, Lima J, et al. Echocardiographic and pathologic characteristics of primary cardiac tumors: A study of 149 cases. Int J Cardiol. 2002;84:69e75. 5. Silverman NA. Primary cardiac tumors. Ann Surg. 1980;191:127e138. 6. Look Hong NJ, Pandalai PK, Hornick JL, et al. Cardiac angiosarcoma management and outcomes: 20-year single-institution experience. Ann Surg Oncol. 2012;19:2707e2715.
7. Zhang PJ, Brooks JS, Goldblum JR, et al. Primary cardiac sarcomas: A clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol. 2008;39:1385e1395. 8. Hosokawa Y, Kodani E, Kusama Y, et al. Cardiac angiosarcoma diagnosed by transvenous endomyocardial biopsy with the aid of transesophageal echocardiography and intra-procedural consultation. Int Heart J. 2010;51:367e369. 9. Judson I, Radford JA, Harris M, et al. Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: A study by the EORTC soft tissue and bone sarcoma group. Eur J Cancer. 2001;37:870e877. 10. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer. 1986;57:852e859. 11. Bafaloukos D, Papadimitriou C, Linardou H, et al. Combination of pegylated liposomal doxorubicin (PLD) and paclitaxel in patients with advanced soft tissue sarcoma: A phase II study of the hellenic cooperative oncology group. Br J Cancer. 2004;91:1639e1644. 12. Fatima J, Duncan AA, Maleszewski JJ, et al. Primary angiosarcoma of the aorta, great vessels, and the heart. J Vasc Surg. 2013;57:756e764. 13. Sorlie D, Myhre ES, Stalsberg H. Angiosarcoma of the heart. Unusual presentation and survival after treatment. Br Heart J. 1984;51:94e97. 14. Crespo MG, Pulpon LA, Pradas G, et al. Heart transplantation for cardiac angiosarcoma: Should its indication be questioned? J Heart Lung Transplant. 1993;12:527e530.