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Diagnosis and Treatment of a Symptomatic Primary Thoracic Aortic Tumor: Endovascular Exclusion to Prevent Recurrent Embolization
Diagnosis and Treatment of a Symptomatic Primary Thoracic Aortic Tumor: Endovascular Exclusion to Prevent Recurrent Embolization Javier Peinado Cebrian,1 Gaspar Mestres Alomar,2 Ruben Rodriguez Carvajal,1 Juan Pablo Uribe Larach,3 and Vicente Riambau Alonso,2 Toledo and Barcelona, Spain, Santiago, Chile
Intraluminal aortic malignant masses are extremely rare. Clinical symptoms of these tumors include locally occlusive aortic disease, peripheral emboli, or mesenteric emboli. Eventually, general symptoms, such as fatigue, weight loss, or fever, will occur. Nevertheless, the diagnosis is most often made after surgery or autopsy, when histologic data can be examined. Few cases of intraaortic masses treated endovascularly have been reported, most of them related to intraluminal blood clot formation. We present a case of intraaortic malignant tumor formation with distal embolization to the legs, in which the diagnosis of malignant disease was reached after analysis of the material obtained during embolectomy. Endovascular exclusion was performed as a transient treatment to avoid new embolic events.
Intraluminal aortic malignant masses are extremely rare. Clinical symptoms of these tumors include locally occlusive aortic disease, peripheral emboli, or mesenteric emboli. Eventually, general symptoms, such as fatigue, weight loss, or fever, will also occur. Nevertheless, the diagnosis is most often made after surgery or autopsy, when histologic findings can be assessed. Few cases of intraaortic masses treated endovascularly have been reported. Most of these cases have been related to intraluminal blood clot formation. In this report we present a case of intraaortic malignant tumor formation with distal embolization to the legs. The diagnosis of malignant disease was reached after analysis of the material obtained
1 Department of Angiology and Vascular Surgery, Hospital Virgen de la Salud, Toledo, Spain. 2 Department of Angiology and Vascular Surgery, Hospital Clinic, Barcelona, Spain. 3 Department of Vascular Surgery, Clı´nica las Condes, Santiago de Chile, Chile.
Correspondence to: Javier Peinado Cebrian, MD, Department of Angiology and Vascular Surgery, Hospital Virgen de la Salud, Avda Barber 3, TO 45003, Spain; E-mail: [email protected] Ann Vasc Surg 2014; 28: 492.e5–492.e9 http://dx.doi.org/10.1016/j.avsg.2012.12.014 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: October 15, 2012; manuscript accepted: December 12, 2012; published online: December 2, 2013.
during embolectomy. Endovascular exclusion was performed as a temporary treatment to avoid new embolic events.
CASE REPORT We present the case of a 62-year-old man who was being examined for back pain and constitutional syndrome. During spinal gadolinium-enhanced magnetic resonance (GE- MR) assessment, the patient had acute bilateral leg pain, accompanied by pallor and decreased temperature. Under suspected acute ischemia of legs, a surgical radioguided embolectomy was performed with excellent technical and clinical results (Fig. 1), in which an atypical hyaline material, without appearance of coagulated blood was obtained. Histopathologic intraoperative examination of this material revealed metastatic pleomorphic malignant tissue. GE-MR revealed an intraluminal mass inside the descending thoracic aorta at 3 cm above the origin of celiac trunk. This mass had a jagged edge, which was attached to the aortic wall and had a floating portion. Multiple images indicated metastasis affecting the lumbar vertebral column. The definitive histopathologic analysis of the embolic material revealed malignant necrotizing intravascular neoplasis (Fig. 2). Anticoagulation treatment was then initiated and maintained during follow-up. A computed tomography angiogram (CTAngio) was performed 2 weeks after the intervention. The aortic mass maintained a floating intraluminal portion, with a high risk for embolization (Fig. 3). Although surgical 492.e5
492.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Radioguided embolectomy of bilateral lower extremities before (A, B) and after (A0 , B0 ) arteriography. resection and prosthetic aortic replacement should be considered as the first option, general medical conditions and the metastatic status suggested palliative treatment, which was agreed upon with the oncology staff. An endovascular approach was utilized to prevent further embolic events. A relay thoracic stent graft (Bolton Medical, Sunrise, FL, USA) was successfully applied. We selected this type of endograft because it has a double sheath deployment system: a rigid hydrochloride polyvinyl outer sheath, with a smooth surface, which allows the device to be easily advanced through the aorta, and a softer outer sheath made of thin polyester, with a rougher surface, which allows retraction of the distal portion of the stent graft for a more precise and smooth delivery. The endoprosthesis was placed in the desired position to completely cover the mass. Balloon remodeling was not performed in order to avoid embolization. The postoperative period was uneventful and the patient was discharged 48 hours after the intervention. He remained free of new embolic events during the entire surveillance period. The patient continued with multi-chemotherapy.
An angioTC performed 1 month after the procedure showed no evidence of tumor masses protruding in the aortic lumen. Eight months after the diagnosis the patient died while in the oncology unit due to multiorgan failure related to the cancer. Unfortunately, clinical autopsy was not performed, and not enough embolic material was available for immunohistochemical analysis.
DISCUSSION More than 85% of major arterial emboli are of cardiac origin. In recent years, noncardiac sources of arterial embolism have been recognized with increasing frequency, whereas idiopathic causes have become less common. The incidence of arterial embolism of aortic origin has been reported in up to 5% of cases. Mural thrombus in the aneurysmatic or atherosclerotic abdominal aorta may be the source of major arterial emboli.1,2 Large emboli originating
Vol. 28, No. 2, February 2014
Case reports 492.e7
Fig. 2. Atypical tumoral cells with mitosis (circle) showing a poorly differentiated pattern. This finding is compatible with angiosarcoma.
from a nonatheromatous and nonaneurysmatic aorta are infrequent; most have been reported to originate from the abdominal aorta.3,4 Much more unusual is the normal thoracic aorta as a source of this peripheral arterial embolism. The presence of tumor thrombus in the thoracic aorta is a rare entity,5e8 and more frequently derived from intravascular protrusion or metastatic dissemination of neoplasm located in adjacent organs, particularly lung and esophagus.9e11 Even rarer is the formation of primary malignant tumors of the aorta (PMTAs). Published studies on this entity are limited to few case reports and systematic reviews. Oldenburg et al. collected data on 86 primary aortic neoplasms reported between the years 1873 and 2002, with 15 different anatomopathologic diagnoses (Table I).12 Up to that point, only 5 thoracic aorta angiosarcomas had been reported. Of the 86 cases of primary aortic tumors, 71% indicated metastatic disease at autopsy. The mean surveillance time was 14 months (median 7 months, range 0.25e168 months). More recently, Rusthoven et al. reviewed 199 case reports, of which 58% were from males with a mean age at diagnosis of 59.1 years.13 These tumors can be observed in various parts of the aorta. Chiche et al. reported that 34.9% of the tumors were located in the descending thoracic aorta, 27.3% in abdominal aorta, 26.5% in the thoracoabdominal aorta, and 11.3% in the aortic arch.14 PMTAs can contain pedunculated floating portions, which can move freely in the aortic lumen with each cardiac cycle, and their fragmentation can lead to acute ischemic episodes due to arterial embolization in 20% of the patients. These embolus
Fig. 3. Floating intraluminal thoracic aortic tumor. (A) Rendering three-dimensional angioTC reconstruction. (B) Axial angio-TC.
can affect limbs, visceral or cerebral arterial areas, making the clinical presentation widely variable. The most frequent complaint is pain.5e8 Histopathologic examination of the thrombectomy or the tumor resection material, when performed, is the only way to confirm the neoplastic origin of these intravascular masses. In some cases, it may be the index test for diagnosis of malignant disease. Therefore, when tumor embolization is suspected, it is essential that the diagnostic process include submitting the material for pathologic assessment. An algorithm has been proposed to help in the early diagnosis of PMTAs. When splanchnic or inferior extremity embolization occurs, a cardiac origin must be initially suspected, but can be ruled out with echocardiography. If a cardiac source is not found, contrast-enhanced magnetic resonance angiography (MRA) of the thorax and abdomen is preferred over TC, as it is considered more precise in distinguishing healthy aorta from tumor. Late diagnosis is a major reason for negative prognosis of this disease. It is essential to maintain a high index of suspicion and follow a diagnostic algorithm. Regarding anatomopathologic diagnosis, many different staining procedures have been suggested
492.e8 Case reports
Annals of Vascular Surgery
Table I. Distribution of histopathologic types of PMATs Histology
Thoracic aorta Thoracoabdominal (N ¼ 53) aorta (N ¼ 10)
Sarcoma 15 Malignant fibrous 11 histiocytoma Angiosarcoma 5 Leiomyosarcoma 6 Fibrosarcoma 4 Myxoma 3 Fibromyxosarcoma 1 Hemangiopericytoma 2 Hemangioendothelioma 2 Malignant endothelioma 2 Aortic intimal sarcoma 0 Myxosarcoma 0 Endotheliosarcoma 1 Fibromyxoma 0 Fibroxanthosarcoma 1
1 1 0 2 0 1 1 0 0 0 2 1 0 1 0
Adapted from Meis-Kindblom and Kindblom.16
for confirmation of the vascular origin of these tumors, of which inmunohistochemical analysis (IHC) is currently the most frequently utilized.15,16 Based on IHC analysis, Thalheimer et al. proposed in 2004 a new classification system for PMTAs, which divides them into two main groups: (1) a mural origin, histologically originating from the media or adventitia and expressing mesenchymally specific antigens on IHC; or (2) an intimal origin, which can be subdivided into intimal angiosarcomas and intimal myofibroblastic sarcomas.17,18 Due to the absence of a large series addressing this pathologic entity, the optimal treatment of these tumors remains undefined. Long-term anticoagulation has been recommended to prevent ischemic events. When the mass persists in the aorta, however, recurrent embolization or aortic occlusion occurs and anticoagulation has not been demonstrated to avoid further embolic episodes. Based on the limited data, the current ‘‘gold standard’’ treatment is surgical resection and aortic reconstruction with graft interposition. However, it is difficult to determine survival improvement after surgery, mostly because of the disparity of results in the cases reported. The mortality of these interventions is high (16.7% within the first month) and median survival has been estimated to be extended by only 3 months (from 7 to 10).14 Furthermore, the risk for surgery-related complications is enhanced, because, to perform a curative surgery, it is necessary to resect all intercostals and lumbar arteries rising from the involved segment of the aorta. Further, risk for embolic events is high because of
the particular thrombogenic predisposition of this disease.14 The poor results obtained by radical surgery have prompted a search for alternative treatments. Few reports of endovascular treatment for PMTAs have been published worldwide,14,19,20 but only one study reported a diagnosis of PMTA established before treatment. Herein we have presented a second case in which the endovascular treatment was decided upon with a confirmed diagnosis of PMTA, followed by embolic prophylaxis. This was intended to be a temporary treatment to avoid recurrent embolic events. Unfortunately, we were unable to perform radical treatment because early metastatic complications made the patient unfit for surgery. Nevertheless, survival (8 months after diagnosis) was slightly longer than the median survival described in the literature, and the objective of avoiding new embolic events was successfully achieved. This study has provided new data regarding endovascular treatment of PMTAs, which should be considered as an alternative, particularly in metastatic disseminated disease and in inoperable patients with a high risk for embolization. The avoidance of embolic events could improve survival and quality of life in these patients who, regrettably, have a poor prognosis in the short and medium term. For cases in which endovascular treatment is used, exhaustive planning should be performed, focusing all efforts on avoiding embolization during and after surgery. REFERENCES 1. Fueglistaler P, Wolff T, Guerke L, et al. Endovascular stent graft for symptomatic mobile thrombus of the thoracic aorta. J Vasc Surg 2005;42:781e3. 2. Lopez S, Tarmiz A, Rousseau H, et al. Floating aortic thrombus: aortic trauma treated by heparin and delayed covered stent. Ann Vasc Surg 2011;25:984.e1e3. 3. Lozano P, Gomez F, Jaume J, et al. Recurrent embolism caused by floating thrombus in the thoracic aorta. Ann Vasc Surg 1998;12:609e11. 4. Mohammadi S, Trahan S, Miro S, et al. Large freefloating intra-aortic thrombus. Circulation 2007;116: e142e3. 5. Daniel L, Figarella-Branger D, Tournigard P, et al. Aortic sarcomas with peripheral emboli: apropos of 2 cases. Rev Med Intern 1997;18:799e805. 6. Mohsen NA, Haber M, Urrutia VC, et al. Intimal sarcoma of the aorta. AJR Am J Roentgenol 2000;175:1289e90. 7. Szekely E, Kulka J, Miklos I, et al. Leiomyosarcomas of great vessels. Pathol Oncol Res 2000;6:233e6. 8. Shuster TA, Dall’Olmo CA, Spandone D, et al. Abdominal aortic sarcoma: report of a case with long-term survival and review of the literature. Ann Vasc Surg 2002;16: 545e9.
Vol. 28, No. 2, February 2014
9. Pimiento JM, Teso D, Malkan A, et al. Cancer of unknown primary origin: a decade of experience in a communitybased hospital. Am J Surg 2007;194:833e7. 10. Ozcan T, Horoz M, Abali H, et al. Unusual aortic localization of a malignant epithelial tumor metastasis of unknown origin. J Cardiovasc Med 2009;10:78e80. 11. Schr€ oder C, Sch€ onhofer B, Vogel B. Transesophageal echographic determination of aortic invasion by lung cancer. Chest 2005;127:438e42. 12. Oldenburg WA. Primary Tumors of Major Blood Vessels. 6th ed. Philadelphia: Elsevier Saunders, 2005. 13. Rusthoven C, Shames LM, Marilyn MB, et al. High-grade undifferentiated pleomorphic sarcoma of the aortic arch: a case of endovascular therapy for embolic prophylaxis and review of the literature. Vasc Endovasc Surg 2010;44:385e91. 14. Chiche L, Mongredien B, Brocheriou I, et al. Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature.
Case reports 492.e9
15. Lin JS. Diagnostic application of immunohistochemistry in pleomorphic sarcomas. Zhonghua Bing Li Xue Za Zhi 1990;19:67e70. 16. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 1998;22: 683e97. 17. Thalheimer A, Fein M, Geissinger E, et al. Intimal angiosarcoma of the aorta: report of a case and review of the literature. J Vasc Surg 2004;40:548e53. 18. Muhelsted S, Mallery S, Joyce L, et al. Myxoid leiomyosarcoma of the descending thoracic aorta. J Thorac Cardiovasc Surg 2001;121:590e2. 19. Utsunomiya D, Ikeda O, Ideta I, et al. Malignant fibrous histiocytoma arising from the aortic wall mimicking a pseudoaneurism with ulceration. Circ J 2007;71:1659e61. 20. Ramjee V, Ellozy S. Aortic angiosarcoma masquerading as a thoracic aortic aneurysm: a case report and literature. J Vasc Surg 2009;50:1477e80.
Intraluminal aortic malignant masses are extremely rare. Clinical symptoms of these tumors include locally occlusive aortic disease, peripheral emboli, or mesenteric emboli. Eventually, general symptoms, such as fatigue, weight loss, or fever, will occur. Nevertheless, the diagnosis is most often made after surgery or autopsy, when histologic data can be examined. Few cases of intraaortic masses treated endovascularly have been reported, most of them related to intraluminal blood clot formation. We present a case of intraaortic malignant tumor formation with distal embolization to the legs, in which the diagnosis of malignant disease was reached after analysis of the material obtained during embolectomy. Endovascular exclusion was performed as a transient treatment to avoid new embolic events.
Intraluminal aortic malignant masses are extremely rare. Clinical symptoms of these tumors include locally occlusive aortic disease, peripheral emboli, or mesenteric emboli. Eventually, general symptoms, such as fatigue, weight loss, or fever, will also occur. Nevertheless, the diagnosis is most often made after surgery or autopsy, when histologic findings can be assessed. Few cases of intraaortic masses treated endovascularly have been reported. Most of these cases have been related to intraluminal blood clot formation. In this report we present a case of intraaortic malignant tumor formation with distal embolization to the legs. The diagnosis of malignant disease was reached after analysis of the material obtained
1 Department of Angiology and Vascular Surgery, Hospital Virgen de la Salud, Toledo, Spain. 2 Department of Angiology and Vascular Surgery, Hospital Clinic, Barcelona, Spain. 3 Department of Vascular Surgery, Clı´nica las Condes, Santiago de Chile, Chile.
Correspondence to: Javier Peinado Cebrian, MD, Department of Angiology and Vascular Surgery, Hospital Virgen de la Salud, Avda Barber 3, TO 45003, Spain; E-mail: [email protected] Ann Vasc Surg 2014; 28: 492.e5–492.e9 http://dx.doi.org/10.1016/j.avsg.2012.12.014 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: October 15, 2012; manuscript accepted: December 12, 2012; published online: December 2, 2013.
during embolectomy. Endovascular exclusion was performed as a temporary treatment to avoid new embolic events.
CASE REPORT We present the case of a 62-year-old man who was being examined for back pain and constitutional syndrome. During spinal gadolinium-enhanced magnetic resonance (GE- MR) assessment, the patient had acute bilateral leg pain, accompanied by pallor and decreased temperature. Under suspected acute ischemia of legs, a surgical radioguided embolectomy was performed with excellent technical and clinical results (Fig. 1), in which an atypical hyaline material, without appearance of coagulated blood was obtained. Histopathologic intraoperative examination of this material revealed metastatic pleomorphic malignant tissue. GE-MR revealed an intraluminal mass inside the descending thoracic aorta at 3 cm above the origin of celiac trunk. This mass had a jagged edge, which was attached to the aortic wall and had a floating portion. Multiple images indicated metastasis affecting the lumbar vertebral column. The definitive histopathologic analysis of the embolic material revealed malignant necrotizing intravascular neoplasis (Fig. 2). Anticoagulation treatment was then initiated and maintained during follow-up. A computed tomography angiogram (CTAngio) was performed 2 weeks after the intervention. The aortic mass maintained a floating intraluminal portion, with a high risk for embolization (Fig. 3). Although surgical 492.e5
492.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Radioguided embolectomy of bilateral lower extremities before (A, B) and after (A0 , B0 ) arteriography. resection and prosthetic aortic replacement should be considered as the first option, general medical conditions and the metastatic status suggested palliative treatment, which was agreed upon with the oncology staff. An endovascular approach was utilized to prevent further embolic events. A relay thoracic stent graft (Bolton Medical, Sunrise, FL, USA) was successfully applied. We selected this type of endograft because it has a double sheath deployment system: a rigid hydrochloride polyvinyl outer sheath, with a smooth surface, which allows the device to be easily advanced through the aorta, and a softer outer sheath made of thin polyester, with a rougher surface, which allows retraction of the distal portion of the stent graft for a more precise and smooth delivery. The endoprosthesis was placed in the desired position to completely cover the mass. Balloon remodeling was not performed in order to avoid embolization. The postoperative period was uneventful and the patient was discharged 48 hours after the intervention. He remained free of new embolic events during the entire surveillance period. The patient continued with multi-chemotherapy.
An angioTC performed 1 month after the procedure showed no evidence of tumor masses protruding in the aortic lumen. Eight months after the diagnosis the patient died while in the oncology unit due to multiorgan failure related to the cancer. Unfortunately, clinical autopsy was not performed, and not enough embolic material was available for immunohistochemical analysis.
DISCUSSION More than 85% of major arterial emboli are of cardiac origin. In recent years, noncardiac sources of arterial embolism have been recognized with increasing frequency, whereas idiopathic causes have become less common. The incidence of arterial embolism of aortic origin has been reported in up to 5% of cases. Mural thrombus in the aneurysmatic or atherosclerotic abdominal aorta may be the source of major arterial emboli.1,2 Large emboli originating
Vol. 28, No. 2, February 2014
Case reports 492.e7
Fig. 2. Atypical tumoral cells with mitosis (circle) showing a poorly differentiated pattern. This finding is compatible with angiosarcoma.
from a nonatheromatous and nonaneurysmatic aorta are infrequent; most have been reported to originate from the abdominal aorta.3,4 Much more unusual is the normal thoracic aorta as a source of this peripheral arterial embolism. The presence of tumor thrombus in the thoracic aorta is a rare entity,5e8 and more frequently derived from intravascular protrusion or metastatic dissemination of neoplasm located in adjacent organs, particularly lung and esophagus.9e11 Even rarer is the formation of primary malignant tumors of the aorta (PMTAs). Published studies on this entity are limited to few case reports and systematic reviews. Oldenburg et al. collected data on 86 primary aortic neoplasms reported between the years 1873 and 2002, with 15 different anatomopathologic diagnoses (Table I).12 Up to that point, only 5 thoracic aorta angiosarcomas had been reported. Of the 86 cases of primary aortic tumors, 71% indicated metastatic disease at autopsy. The mean surveillance time was 14 months (median 7 months, range 0.25e168 months). More recently, Rusthoven et al. reviewed 199 case reports, of which 58% were from males with a mean age at diagnosis of 59.1 years.13 These tumors can be observed in various parts of the aorta. Chiche et al. reported that 34.9% of the tumors were located in the descending thoracic aorta, 27.3% in abdominal aorta, 26.5% in the thoracoabdominal aorta, and 11.3% in the aortic arch.14 PMTAs can contain pedunculated floating portions, which can move freely in the aortic lumen with each cardiac cycle, and their fragmentation can lead to acute ischemic episodes due to arterial embolization in 20% of the patients. These embolus
Fig. 3. Floating intraluminal thoracic aortic tumor. (A) Rendering three-dimensional angioTC reconstruction. (B) Axial angio-TC.
can affect limbs, visceral or cerebral arterial areas, making the clinical presentation widely variable. The most frequent complaint is pain.5e8 Histopathologic examination of the thrombectomy or the tumor resection material, when performed, is the only way to confirm the neoplastic origin of these intravascular masses. In some cases, it may be the index test for diagnosis of malignant disease. Therefore, when tumor embolization is suspected, it is essential that the diagnostic process include submitting the material for pathologic assessment. An algorithm has been proposed to help in the early diagnosis of PMTAs. When splanchnic or inferior extremity embolization occurs, a cardiac origin must be initially suspected, but can be ruled out with echocardiography. If a cardiac source is not found, contrast-enhanced magnetic resonance angiography (MRA) of the thorax and abdomen is preferred over TC, as it is considered more precise in distinguishing healthy aorta from tumor. Late diagnosis is a major reason for negative prognosis of this disease. It is essential to maintain a high index of suspicion and follow a diagnostic algorithm. Regarding anatomopathologic diagnosis, many different staining procedures have been suggested
492.e8 Case reports
Annals of Vascular Surgery
Table I. Distribution of histopathologic types of PMATs Histology
Thoracic aorta Thoracoabdominal (N ¼ 53) aorta (N ¼ 10)
Sarcoma 15 Malignant fibrous 11 histiocytoma Angiosarcoma 5 Leiomyosarcoma 6 Fibrosarcoma 4 Myxoma 3 Fibromyxosarcoma 1 Hemangiopericytoma 2 Hemangioendothelioma 2 Malignant endothelioma 2 Aortic intimal sarcoma 0 Myxosarcoma 0 Endotheliosarcoma 1 Fibromyxoma 0 Fibroxanthosarcoma 1
1 1 0 2 0 1 1 0 0 0 2 1 0 1 0
Adapted from Meis-Kindblom and Kindblom.16
for confirmation of the vascular origin of these tumors, of which inmunohistochemical analysis (IHC) is currently the most frequently utilized.15,16 Based on IHC analysis, Thalheimer et al. proposed in 2004 a new classification system for PMTAs, which divides them into two main groups: (1) a mural origin, histologically originating from the media or adventitia and expressing mesenchymally specific antigens on IHC; or (2) an intimal origin, which can be subdivided into intimal angiosarcomas and intimal myofibroblastic sarcomas.17,18 Due to the absence of a large series addressing this pathologic entity, the optimal treatment of these tumors remains undefined. Long-term anticoagulation has been recommended to prevent ischemic events. When the mass persists in the aorta, however, recurrent embolization or aortic occlusion occurs and anticoagulation has not been demonstrated to avoid further embolic episodes. Based on the limited data, the current ‘‘gold standard’’ treatment is surgical resection and aortic reconstruction with graft interposition. However, it is difficult to determine survival improvement after surgery, mostly because of the disparity of results in the cases reported. The mortality of these interventions is high (16.7% within the first month) and median survival has been estimated to be extended by only 3 months (from 7 to 10).14 Furthermore, the risk for surgery-related complications is enhanced, because, to perform a curative surgery, it is necessary to resect all intercostals and lumbar arteries rising from the involved segment of the aorta. Further, risk for embolic events is high because of
the particular thrombogenic predisposition of this disease.14 The poor results obtained by radical surgery have prompted a search for alternative treatments. Few reports of endovascular treatment for PMTAs have been published worldwide,14,19,20 but only one study reported a diagnosis of PMTA established before treatment. Herein we have presented a second case in which the endovascular treatment was decided upon with a confirmed diagnosis of PMTA, followed by embolic prophylaxis. This was intended to be a temporary treatment to avoid recurrent embolic events. Unfortunately, we were unable to perform radical treatment because early metastatic complications made the patient unfit for surgery. Nevertheless, survival (8 months after diagnosis) was slightly longer than the median survival described in the literature, and the objective of avoiding new embolic events was successfully achieved. This study has provided new data regarding endovascular treatment of PMTAs, which should be considered as an alternative, particularly in metastatic disseminated disease and in inoperable patients with a high risk for embolization. The avoidance of embolic events could improve survival and quality of life in these patients who, regrettably, have a poor prognosis in the short and medium term. For cases in which endovascular treatment is used, exhaustive planning should be performed, focusing all efforts on avoiding embolization during and after surgery. REFERENCES 1. Fueglistaler P, Wolff T, Guerke L, et al. Endovascular stent graft for symptomatic mobile thrombus of the thoracic aorta. J Vasc Surg 2005;42:781e3. 2. Lopez S, Tarmiz A, Rousseau H, et al. Floating aortic thrombus: aortic trauma treated by heparin and delayed covered stent. Ann Vasc Surg 2011;25:984.e1e3. 3. Lozano P, Gomez F, Jaume J, et al. Recurrent embolism caused by floating thrombus in the thoracic aorta. Ann Vasc Surg 1998;12:609e11. 4. Mohammadi S, Trahan S, Miro S, et al. Large freefloating intra-aortic thrombus. Circulation 2007;116: e142e3. 5. Daniel L, Figarella-Branger D, Tournigard P, et al. Aortic sarcomas with peripheral emboli: apropos of 2 cases. Rev Med Intern 1997;18:799e805. 6. Mohsen NA, Haber M, Urrutia VC, et al. Intimal sarcoma of the aorta. AJR Am J Roentgenol 2000;175:1289e90. 7. Szekely E, Kulka J, Miklos I, et al. Leiomyosarcomas of great vessels. Pathol Oncol Res 2000;6:233e6. 8. Shuster TA, Dall’Olmo CA, Spandone D, et al. Abdominal aortic sarcoma: report of a case with long-term survival and review of the literature. Ann Vasc Surg 2002;16: 545e9.
Vol. 28, No. 2, February 2014
9. Pimiento JM, Teso D, Malkan A, et al. Cancer of unknown primary origin: a decade of experience in a communitybased hospital. Am J Surg 2007;194:833e7. 10. Ozcan T, Horoz M, Abali H, et al. Unusual aortic localization of a malignant epithelial tumor metastasis of unknown origin. J Cardiovasc Med 2009;10:78e80. 11. Schr€ oder C, Sch€ onhofer B, Vogel B. Transesophageal echographic determination of aortic invasion by lung cancer. Chest 2005;127:438e42. 12. Oldenburg WA. Primary Tumors of Major Blood Vessels. 6th ed. Philadelphia: Elsevier Saunders, 2005. 13. Rusthoven C, Shames LM, Marilyn MB, et al. High-grade undifferentiated pleomorphic sarcoma of the aortic arch: a case of endovascular therapy for embolic prophylaxis and review of the literature. Vasc Endovasc Surg 2010;44:385e91. 14. Chiche L, Mongredien B, Brocheriou I, et al. Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature.
Case reports 492.e9
15. Lin JS. Diagnostic application of immunohistochemistry in pleomorphic sarcomas. Zhonghua Bing Li Xue Za Zhi 1990;19:67e70. 16. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 1998;22: 683e97. 17. Thalheimer A, Fein M, Geissinger E, et al. Intimal angiosarcoma of the aorta: report of a case and review of the literature. J Vasc Surg 2004;40:548e53. 18. Muhelsted S, Mallery S, Joyce L, et al. Myxoid leiomyosarcoma of the descending thoracic aorta. J Thorac Cardiovasc Surg 2001;121:590e2. 19. Utsunomiya D, Ikeda O, Ideta I, et al. Malignant fibrous histiocytoma arising from the aortic wall mimicking a pseudoaneurism with ulceration. Circ J 2007;71:1659e61. 20. Ramjee V, Ellozy S. Aortic angiosarcoma masquerading as a thoracic aortic aneurysm: a case report and literature. J Vasc Surg 2009;50:1477e80.