- Email: [email protected]
Intimal angiosarcoma presenting with common femoral artery aneurysm
Intimal angiosarcoma presenting with common femoral artery aneurysm Sun Young Choi, MD,a Soo Kee Min, MD,b Kun Il Kim, MD,c and Ho Young Kim, MD,d Seoul and Gyeonggi-do, Republic of Korea Intimal angiosarcoma is a rare malignant vascular tumor with an aggressive natural history. Presenting symptoms vary according to the location of the lesion, but symptoms are typically due to intravascular obstruction or embolization. We present a case of an intimal angiosarcoma presenting with a common femoral artery aneurysm. ( J Vasc Surg 2012;56: 819-21.)
Intravascular endothelial lesions almost always represent benign tumors or reactive changes. Vascular malignancies are rare and account for ⬍1% of all sarcomas.1 Among them, exceptionally rare cases of intimal angiosarcoma have been reported to arise from large vessels such as the aorta or common femoral artery (CFA) or superficial femoral artery (SFA), presenting with aortic occlusion or lower extremity arterial stenosis or embolization.2-5 Here, we report a patient who initially presented with a CFA aneurysm. CASE REPORT A 34-year-old man presented with a 1-year history of a pulsating mass at the left inguinal region, which recently enlarged in the previous few weeks. He had no other relevant medical problems. He was a martial arts coach 7 years ago and had no history of trauma. On physical examination, a pulsating bulging mass was present in the left inguinal region, and no combined fever or pain was noted. Ultrasonography identified a dilated left CFA with abnormal intimal flaps and thrombi (Fig 1, A). A few small lymph nodes were noted in the left inguinal region. Computed tomography was performed, and the findings were almost identical to those of the ultrasonography (Fig 1, B). Based on these analyses, our initial diagnosis was an aneurysm caused by blunt trauma or vasculitis. However, vasculitis was ruled out because serum inflammatory markers and antibody screen test results were all within the normal range. Subsequently, surgical treatment was undertaken. Severe adhesion was noted between the aneurysm and adjacent soft tissue, and a focal full-thickness tear was observed at the distal part of the aneurysm. After arteriotomy, intimal flaps and a very soft, redFrom the Department of Radiology and Medical Research Institute, School of Medicine, Ewha Womans University, Seoula; and the Department of Pathology,b Department of Thoracic Surgery,c and Department of Internal Medicine,d Hallym University, Sacred Heart Hospital, College of Medicine, Gyeonggi-do. Author conflict of interest: none. Reprint requests: Sun Young Choi, MD, Department of Radiology and Medical Research Institute, School of Medicine, Ewha Womans University, Yangcheon-gu, Seoul, Republic of Korea (e-mail: medmath@ hanmil.net). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214/$36.00 Copyright © 2012 by the Society for Vascular Surgery. http://dx.doi.org/10.1016/j.jvs.2012.03.034
brown thrombus were found incompletely filling the aneurysm lumen (Fig 2, A). The left CFA above the aneurysm and proximal segment of the SFA and deep femoral artery below the aneurysm were excised with reconstruction using an 8-mm polytetrafluoroethylene (PTFE) graft (Gore-Tex; W. L. Gore, Flagstaff, Ariz). Inguinal lymph node dissection was also performed. Upon histopathologic examination of the permanent sections, malignant tumor cells were found in the entire intima of the CFA with the presence of malignant cells at the resection margins as well as in the thrombus. Some of them infiltrated the perivascular soft tissue through the media and adventitia of the vessel wall. Tumor cells were diverse in their morphology: epithelioid, spindle, and pleomorphic. Some showed intracytoplasmic lumen-like vacuoles, but definite vascular channels were not found. Tumor cells were positive for endothelial markers CD31 and CD34 after immunohistochemical staining (Fig 2, B). Thus, the final diagnosis was intimal angiosarcoma of the CFA. The pathologic result of lymph node specimens was reactive lymph node hyperplasia. The Musculoskeletal Tumor Society stage was IIa (G2T1M0). Pelvic magnetic resonance imaging and positron emission tomography-computed tomography were performed to evaluate for the presence of residual malignancy within the surgical field and for metastatic disease, but no such phenomena were noted. However, according to the histopathologic results, the patient underwent reoperation for complete resection of the tumor-bearing arterial segment 2 weeks after the first operation. In the second operation, we first removed the PTFE graft (Gore-Tex), and then we resected more to the proximal and distal resected margin, repeatedly checking the resection margins with intraoperatively obtained frozen sections of the specimen. Finally, we obtained a tumor-free resection margin at the distal external iliac artery as the proximal resection margin, about 5 cm above from the first proximal resection margin, and at the proximal SFA and deep femoral artery as the distal resection margin, about 2 cm below the first distal resection margin. Next, reanastomosis was performed using an 8-mm PTFE graft (Gore-Tex). No additional adjacent soft tissue resection was performed, including that of the nerve, vein, and muscle. Then, the patient was transferred to the oncology division for chemotherapy to receive six cycles of doxorubicin chemotherapy (dosage, 50 mg/m2 per cycle). During the 6-month follow-up, no local tumor recurrence or systemic metastasis was found.
819
JOURNAL OF VASCULAR SURGERY September 2012
820 Choi et al
DISCUSSION In 1985, primary sarcomas of the aorta were classified as intimal or mural sarcomas according to their clinicopathologic characteristics.6 This classification was expanded by differentiating intimal sarcomas based on their cells of origin as determined by the immunohistochemical staining technique by Thalheimer et al, who defined angiosarcomas as intimal sarcomas of endothelial origin and myofibroblastic sarcomas as sarcomas of mesenchymal origin.2 Intimal angiosarcomas form plaque-like structures along the intima or, more frequently, polypoid intraluminal growth.2,7 According to a review by Sebenik et al, more than 90% of intimal sarcoma lesions had arisen in the aorta, and the iliac/femoral artery involvement rate was 10.1%.8 Thus, clinical symptoms usually relate to the level of aortic lumen obstruction and the presence or absence of thromboembolic metastasis; in addition, preoperative diagnosis is difficult because intimal angiosarcomas present with diverse and nonspecific symptoms, and imaging studies often fail to differentiate among atheroma, mural thrombus, aneurysm, and tumors.2-4,9,10 According to the histologic site of origin, primary malignant tumors of arteries have been classified as mural tumors when they originate from the media or adventitia (these tumors usually present as aneurysms or masses), and luminal tumors originate from the intima, forming a polypoid mass that grows along the lumen of the vessel, presenting as luminal obstructions and/or embolic events.6 However, published cases of intimal angiosarcoma presenting with aneurysm and/or arterial rupture are very rare (15 cases: 14 involving the aorta and one involving the carotid artery), and preoperative and intraoperative clinical and radiological evidence could not predict a malignant condition.7,11-14 No standard treatment method exists for soft tissue sarcoma; however, in the absence of metastasis, en bloc surgical resection of the affected vasculature and surrounding soft tissue with graft interposition is the preferred therapy.2,12,15 The surgical specimens should be examined intraoperatively on frozen sections to ensure complete tumor resection.2,15 The role of chemotherapy and radiotherapy in intimal angiosarcoma remains unclear; however, they have been shown to increase survival and should be offered as adjuvants to surgery.16,17 In the current patient, we did not perform complete resection of the adjacent organs. Malignant cells were found in the adventitia of the specimen from the first operation; thus,
Fig 1. A, Ultrasonography shows a dilated left common femoral artery with a largest diameter of 4 cm and extending into the bifurcation level of the superficial and deep femoral arteries. An abnormal intimal flap (arrow) in the dilated vessel is also noted. Partial thrombus formation is combined in the aneurysm, and abnormal wall thickening and significant stenosis are combined at the proximal segment of the superficial femoral artery (not shown). B, Volume-rendering computed tomography scan showing a dilated left common femoral artery (arrow), identical to that in ultrasonography.
JOURNAL OF VASCULAR SURGERY Volume 56, Number 3
Choi et al 821
chemotherapy; based on literature, asserted chemotherapy is advocated in cases of intimal angiosarcoma where embolic, metastatic, or unresectable disease is present.2,12,16 One oversight regarding this patient was omitting the frozen sections intraoperatively at the initial operation, because malignancy was not suspected. However, aneurysm in a young, healthy patient with no evidence of combined trauma or vasculitis is very unusual. Thus, more precise assessment during the operation should be required, including frozen section assessments. REFERENCES
Fig 2. A, Intraoperative photography showing full-thickness tear (arrow) in the aneurysm lumen. Severe adhesion is noted between this aneurysm and adjacent soft tissue. B, Immunohistochemical staining of endothelial specific antigen CD31 demonstrating many positive tumor cells infiltrating the vascular wall (⫻200).
malignant invasion may have occurred into the adjacent organs. If we wanted to perform a complete resection of the angiosarcoma, we should have performed complete resection of the adjacent organs. However, the femoral nerve innervates through the thigh muscles and skin; thus, if the femoral nerve is removed, gate disturbance could result. Also, we did not perform adjacent muscle resection in order to preserve gate function. Venous resection is possible, however, even after the vein is resected; the possibility of residual malignancy still exists. Based on an overall estimation, we concluded that additional resection of adjacent organs had little clinical significance. Although some literature favors adjuvant radiotherapy rather than chemotherapy, the role of both in intimal angiosarcoma remains unclear.2,7,18 In this patient, we did not perform adjuvant radiotherapy. Because of inguinal lymph node dissections, adjuvant radiotherapy could aggravate leg edema. Furthermore, malignant dissemination could occur, as malignant cells were found at the thrombus in the aneurysm. Thus, we planned to perform adjuvant
1. Leowardi C, Hinz U, Hormann Y, Wente MN, Mechtersheimer G, Willeke F, et al. Malignant vascular tumors: clinical presentation, surgical therapy, and long-term prognosis. Ann Surg Oncol 2005;12:1090-101. 2. Thalheimer A, Fein M, Geissinger E, Franke S. Intimal angiosarcoma of the aorta: report of a case and review of the literature. J Vasc Surg 2004;40:548-53. 3. Sattout AH, Deolekar MV, Tait WF, Williams GT. Femoral artery angiosarcoma presenting with distal embolization: report of a case. Surg Today 2008;38:541-4. 4. Miyamoto T, Mihara M, Iwasaki K, Mishima E, Shimao S, Miyamoto H. Angiosarcoma of the thigh with thrombosis in the femoral artery. J Dermatol 1992;19:242-5. 5. Otto RC, Pouliadis GP, Bollinger A. Angiosarcoma of the superficial femoral artery with distal embolization. Radiology 1977;123:310. 6. Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985;9:890-7. 7. Naughton PA, Wandling M, Phade S, Garcia-Toca M, Carr JC, Rodriguez HE. Intimal angiosarcoma causing abdominal aortic rupture. J Vasc Surg 2011;53:818-21. 8. Sebenik M, Ricci A Jr, DiPasquale B, Mody K, Pytel P, Jee KJ, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005;29:1184-93. 9. Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic study. Cancer 1993;71:1761-73. 10. Hagspiel KD, Hunter YR, Ahmed HK, Lu P, Spinosa DJ, Angle JF, et al. Primary sarcoma of the distal abdominal aorta: CT angiography findings. Abdom Imaging 2004;29:507-10. 11. Al-Robaie B, Stephenson M, Evans GH, Sandison AJ. Angiosarcoma mimicking a recurrent carotid artery aneurysm. Ann Vasc Surg 2011;25:e19-23. 12. Ramjee V, Ellozy S. Aortic angiosarcoma masquerading as a thoracic aortic aneurysm. J Vasc Surg 2009;50:1477-80. 13. Hales SL, Locke R, Sandison A, Jenkins M, Hamady M. Aortic angiosarcoma: a rare cause for leaking thoracic aneurysm. Cardiovasc Interv Radiol 2011;34;Suppl 2:S20-4. 14. Schmehl J, Scharpf M, Brechtel K, Kalender G, Heller S, Claussen CD, et al. Epithelioid angiosarcoma with metastatic disease after endovascular therapy of abdominal aortic aneurysm. Cardiovasc Interv Radiol 2011;35:190-3. 15. Karamlou T, Li MK, Williamson WK, Heller L, Wiest JW. Angiosarcoma of the thoracoabdominal aorta presenting with systemic hypertension, anemia, and visceral ischemia. Ann Vasc Surg 2008;22:459-64. 16. Brylka D, Demos TC, Pierce K. Primary angiosarcoma of the abdominal aorta: a case report and literature review (aortic angiosarcoma). Abdom Imaging 2009;34:239-42. 17. Chiche L, Mongrédien B, Brocheriou I, Kieffer E. Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature. Ann Vasc Surg 2003;17:354-64. 18. Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, et al. The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations, in of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 1982;196:305-15. Submitted Jan 9, 2012; accepted Mar 10, 2012.
Intravascular endothelial lesions almost always represent benign tumors or reactive changes. Vascular malignancies are rare and account for ⬍1% of all sarcomas.1 Among them, exceptionally rare cases of intimal angiosarcoma have been reported to arise from large vessels such as the aorta or common femoral artery (CFA) or superficial femoral artery (SFA), presenting with aortic occlusion or lower extremity arterial stenosis or embolization.2-5 Here, we report a patient who initially presented with a CFA aneurysm. CASE REPORT A 34-year-old man presented with a 1-year history of a pulsating mass at the left inguinal region, which recently enlarged in the previous few weeks. He had no other relevant medical problems. He was a martial arts coach 7 years ago and had no history of trauma. On physical examination, a pulsating bulging mass was present in the left inguinal region, and no combined fever or pain was noted. Ultrasonography identified a dilated left CFA with abnormal intimal flaps and thrombi (Fig 1, A). A few small lymph nodes were noted in the left inguinal region. Computed tomography was performed, and the findings were almost identical to those of the ultrasonography (Fig 1, B). Based on these analyses, our initial diagnosis was an aneurysm caused by blunt trauma or vasculitis. However, vasculitis was ruled out because serum inflammatory markers and antibody screen test results were all within the normal range. Subsequently, surgical treatment was undertaken. Severe adhesion was noted between the aneurysm and adjacent soft tissue, and a focal full-thickness tear was observed at the distal part of the aneurysm. After arteriotomy, intimal flaps and a very soft, redFrom the Department of Radiology and Medical Research Institute, School of Medicine, Ewha Womans University, Seoula; and the Department of Pathology,b Department of Thoracic Surgery,c and Department of Internal Medicine,d Hallym University, Sacred Heart Hospital, College of Medicine, Gyeonggi-do. Author conflict of interest: none. Reprint requests: Sun Young Choi, MD, Department of Radiology and Medical Research Institute, School of Medicine, Ewha Womans University, Yangcheon-gu, Seoul, Republic of Korea (e-mail: medmath@ hanmil.net). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214/$36.00 Copyright © 2012 by the Society for Vascular Surgery. http://dx.doi.org/10.1016/j.jvs.2012.03.034
brown thrombus were found incompletely filling the aneurysm lumen (Fig 2, A). The left CFA above the aneurysm and proximal segment of the SFA and deep femoral artery below the aneurysm were excised with reconstruction using an 8-mm polytetrafluoroethylene (PTFE) graft (Gore-Tex; W. L. Gore, Flagstaff, Ariz). Inguinal lymph node dissection was also performed. Upon histopathologic examination of the permanent sections, malignant tumor cells were found in the entire intima of the CFA with the presence of malignant cells at the resection margins as well as in the thrombus. Some of them infiltrated the perivascular soft tissue through the media and adventitia of the vessel wall. Tumor cells were diverse in their morphology: epithelioid, spindle, and pleomorphic. Some showed intracytoplasmic lumen-like vacuoles, but definite vascular channels were not found. Tumor cells were positive for endothelial markers CD31 and CD34 after immunohistochemical staining (Fig 2, B). Thus, the final diagnosis was intimal angiosarcoma of the CFA. The pathologic result of lymph node specimens was reactive lymph node hyperplasia. The Musculoskeletal Tumor Society stage was IIa (G2T1M0). Pelvic magnetic resonance imaging and positron emission tomography-computed tomography were performed to evaluate for the presence of residual malignancy within the surgical field and for metastatic disease, but no such phenomena were noted. However, according to the histopathologic results, the patient underwent reoperation for complete resection of the tumor-bearing arterial segment 2 weeks after the first operation. In the second operation, we first removed the PTFE graft (Gore-Tex), and then we resected more to the proximal and distal resected margin, repeatedly checking the resection margins with intraoperatively obtained frozen sections of the specimen. Finally, we obtained a tumor-free resection margin at the distal external iliac artery as the proximal resection margin, about 5 cm above from the first proximal resection margin, and at the proximal SFA and deep femoral artery as the distal resection margin, about 2 cm below the first distal resection margin. Next, reanastomosis was performed using an 8-mm PTFE graft (Gore-Tex). No additional adjacent soft tissue resection was performed, including that of the nerve, vein, and muscle. Then, the patient was transferred to the oncology division for chemotherapy to receive six cycles of doxorubicin chemotherapy (dosage, 50 mg/m2 per cycle). During the 6-month follow-up, no local tumor recurrence or systemic metastasis was found.
819
JOURNAL OF VASCULAR SURGERY September 2012
820 Choi et al
DISCUSSION In 1985, primary sarcomas of the aorta were classified as intimal or mural sarcomas according to their clinicopathologic characteristics.6 This classification was expanded by differentiating intimal sarcomas based on their cells of origin as determined by the immunohistochemical staining technique by Thalheimer et al, who defined angiosarcomas as intimal sarcomas of endothelial origin and myofibroblastic sarcomas as sarcomas of mesenchymal origin.2 Intimal angiosarcomas form plaque-like structures along the intima or, more frequently, polypoid intraluminal growth.2,7 According to a review by Sebenik et al, more than 90% of intimal sarcoma lesions had arisen in the aorta, and the iliac/femoral artery involvement rate was 10.1%.8 Thus, clinical symptoms usually relate to the level of aortic lumen obstruction and the presence or absence of thromboembolic metastasis; in addition, preoperative diagnosis is difficult because intimal angiosarcomas present with diverse and nonspecific symptoms, and imaging studies often fail to differentiate among atheroma, mural thrombus, aneurysm, and tumors.2-4,9,10 According to the histologic site of origin, primary malignant tumors of arteries have been classified as mural tumors when they originate from the media or adventitia (these tumors usually present as aneurysms or masses), and luminal tumors originate from the intima, forming a polypoid mass that grows along the lumen of the vessel, presenting as luminal obstructions and/or embolic events.6 However, published cases of intimal angiosarcoma presenting with aneurysm and/or arterial rupture are very rare (15 cases: 14 involving the aorta and one involving the carotid artery), and preoperative and intraoperative clinical and radiological evidence could not predict a malignant condition.7,11-14 No standard treatment method exists for soft tissue sarcoma; however, in the absence of metastasis, en bloc surgical resection of the affected vasculature and surrounding soft tissue with graft interposition is the preferred therapy.2,12,15 The surgical specimens should be examined intraoperatively on frozen sections to ensure complete tumor resection.2,15 The role of chemotherapy and radiotherapy in intimal angiosarcoma remains unclear; however, they have been shown to increase survival and should be offered as adjuvants to surgery.16,17 In the current patient, we did not perform complete resection of the adjacent organs. Malignant cells were found in the adventitia of the specimen from the first operation; thus,
Fig 1. A, Ultrasonography shows a dilated left common femoral artery with a largest diameter of 4 cm and extending into the bifurcation level of the superficial and deep femoral arteries. An abnormal intimal flap (arrow) in the dilated vessel is also noted. Partial thrombus formation is combined in the aneurysm, and abnormal wall thickening and significant stenosis are combined at the proximal segment of the superficial femoral artery (not shown). B, Volume-rendering computed tomography scan showing a dilated left common femoral artery (arrow), identical to that in ultrasonography.
JOURNAL OF VASCULAR SURGERY Volume 56, Number 3
Choi et al 821
chemotherapy; based on literature, asserted chemotherapy is advocated in cases of intimal angiosarcoma where embolic, metastatic, or unresectable disease is present.2,12,16 One oversight regarding this patient was omitting the frozen sections intraoperatively at the initial operation, because malignancy was not suspected. However, aneurysm in a young, healthy patient with no evidence of combined trauma or vasculitis is very unusual. Thus, more precise assessment during the operation should be required, including frozen section assessments. REFERENCES
Fig 2. A, Intraoperative photography showing full-thickness tear (arrow) in the aneurysm lumen. Severe adhesion is noted between this aneurysm and adjacent soft tissue. B, Immunohistochemical staining of endothelial specific antigen CD31 demonstrating many positive tumor cells infiltrating the vascular wall (⫻200).
malignant invasion may have occurred into the adjacent organs. If we wanted to perform a complete resection of the angiosarcoma, we should have performed complete resection of the adjacent organs. However, the femoral nerve innervates through the thigh muscles and skin; thus, if the femoral nerve is removed, gate disturbance could result. Also, we did not perform adjacent muscle resection in order to preserve gate function. Venous resection is possible, however, even after the vein is resected; the possibility of residual malignancy still exists. Based on an overall estimation, we concluded that additional resection of adjacent organs had little clinical significance. Although some literature favors adjuvant radiotherapy rather than chemotherapy, the role of both in intimal angiosarcoma remains unclear.2,7,18 In this patient, we did not perform adjuvant radiotherapy. Because of inguinal lymph node dissections, adjuvant radiotherapy could aggravate leg edema. Furthermore, malignant dissemination could occur, as malignant cells were found at the thrombus in the aneurysm. Thus, we planned to perform adjuvant
1. Leowardi C, Hinz U, Hormann Y, Wente MN, Mechtersheimer G, Willeke F, et al. Malignant vascular tumors: clinical presentation, surgical therapy, and long-term prognosis. Ann Surg Oncol 2005;12:1090-101. 2. Thalheimer A, Fein M, Geissinger E, Franke S. Intimal angiosarcoma of the aorta: report of a case and review of the literature. J Vasc Surg 2004;40:548-53. 3. Sattout AH, Deolekar MV, Tait WF, Williams GT. Femoral artery angiosarcoma presenting with distal embolization: report of a case. Surg Today 2008;38:541-4. 4. Miyamoto T, Mihara M, Iwasaki K, Mishima E, Shimao S, Miyamoto H. Angiosarcoma of the thigh with thrombosis in the femoral artery. J Dermatol 1992;19:242-5. 5. Otto RC, Pouliadis GP, Bollinger A. Angiosarcoma of the superficial femoral artery with distal embolization. Radiology 1977;123:310. 6. Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985;9:890-7. 7. Naughton PA, Wandling M, Phade S, Garcia-Toca M, Carr JC, Rodriguez HE. Intimal angiosarcoma causing abdominal aortic rupture. J Vasc Surg 2011;53:818-21. 8. Sebenik M, Ricci A Jr, DiPasquale B, Mody K, Pytel P, Jee KJ, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005;29:1184-93. 9. Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic study. Cancer 1993;71:1761-73. 10. Hagspiel KD, Hunter YR, Ahmed HK, Lu P, Spinosa DJ, Angle JF, et al. Primary sarcoma of the distal abdominal aorta: CT angiography findings. Abdom Imaging 2004;29:507-10. 11. Al-Robaie B, Stephenson M, Evans GH, Sandison AJ. Angiosarcoma mimicking a recurrent carotid artery aneurysm. Ann Vasc Surg 2011;25:e19-23. 12. Ramjee V, Ellozy S. Aortic angiosarcoma masquerading as a thoracic aortic aneurysm. J Vasc Surg 2009;50:1477-80. 13. Hales SL, Locke R, Sandison A, Jenkins M, Hamady M. Aortic angiosarcoma: a rare cause for leaking thoracic aneurysm. Cardiovasc Interv Radiol 2011;34;Suppl 2:S20-4. 14. Schmehl J, Scharpf M, Brechtel K, Kalender G, Heller S, Claussen CD, et al. Epithelioid angiosarcoma with metastatic disease after endovascular therapy of abdominal aortic aneurysm. Cardiovasc Interv Radiol 2011;35:190-3. 15. Karamlou T, Li MK, Williamson WK, Heller L, Wiest JW. Angiosarcoma of the thoracoabdominal aorta presenting with systemic hypertension, anemia, and visceral ischemia. Ann Vasc Surg 2008;22:459-64. 16. Brylka D, Demos TC, Pierce K. Primary angiosarcoma of the abdominal aorta: a case report and literature review (aortic angiosarcoma). Abdom Imaging 2009;34:239-42. 17. Chiche L, Mongrédien B, Brocheriou I, Kieffer E. Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature. Ann Vasc Surg 2003;17:354-64. 18. Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, et al. The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations, in of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 1982;196:305-15. Submitted Jan 9, 2012; accepted Mar 10, 2012.