VASCULAR IMAGES
Aortic fibrosarcoma presenting as rupture of an abdominal aortic aneurysm Kaori Kato, MD, PhD,a Kazuo Yamamoto, MD, PhD,a Shinpei Yoshii, MD, PhD,a and Toshimitsu Suzuki, MD, PhD,b Nagaoka, Niigata, Japan A 48-year-old man was admitted to our emergency department with a sudden onset of severe abdominal pain. He reported a 2-week history of back pain, with complaints of a mass on his left thigh, and was amidst further evaluation by a local physician. On arrival, he was tachycardic with profound diaphoresis. Abdominal examination revealed tenderness elicited on palpitation and a large pulsating mass in his left upper quadrant. His initial laboratory results were unremarkable other than a slight increase in white blood cell count. Computed tomography imaging revealed an intraperitoneal hematoma and an extensively dilated infrarenal abdominal aorta with a maximal diameter of 84 mm (Cover). The aneurysmal wall was irregular but well defined and appeared to contain intramural thrombus that showed subtle late-phase enhancement (A). An urgent midline laparotomy was performed. The surface of the aneurysm was irregular but covered by retroperitoneal membrane. It showed little adhesion to the omentum and no adhesion to the intestines (B). The abdominal aorta was clamped proximally and distally to the aneurysm, and the aneurysm was opened. Atherosclerotic findings inside were minimal, but there was a defect of the intimal layer of the anterior wall. The aneurysm was resected, and the abdominal aorta was reconstructed with a woven polyester tube graft. Histopathologic analysis revealed massive proliferation of fibroblastic tumor cells invading the entire layer of the aorta and destroying the intima. The adventitia remained patent, suggesting that the tumor originated from the aorta and was not a metastatic entity from adjacent organs. Furthermore, the tumor cells formed fascicles with vague herringbone pattern (C), consistently stained positive for vimentin, and showed Ki-67 labeling index of 50%. Taken together, these findings suggested that a high-grade adult fibrosarcoma (World Health Organization classification) arising from the aortic wall led to a ruptured pseudoaneurysm. Postoperative magnetic resonance imaging revealed multiple metastasis to the vertebral bodies and adductor muscles, and the patient was promptly introduced to ifosfamide-based chemotherapy combined with radiotherapy. Primary aortic sarcomas are extremely rare, but may cause aortic rupture,1 dissection, or ischemia,2 and attention is required when diagnosing and treating aortic emergencies. Written consent was obtained from the patient for publication of the study. REFERENCES 1. 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. 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. Submitted May 21, 2013; accepted May 5, 2014. From the Department of Cardiovascular Surgerya and the Department of Pathology,b Tachikawa Medical Center. Author conflict of interest: none. E-mail: [email protected]. 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. J Vasc Surg 2015;61:1066 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2014.05.004
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Aortic fibrosarcoma presenting as rupture of an abdominal aortic aneurysm Kaori Kato, MD, PhD,a Kazuo Yamamoto, MD, PhD,a Shinpei Yoshii, MD, PhD,a and Toshimitsu Suzuki, MD, PhD,b Nagaoka, Niigata, Japan A 48-year-old man was admitted to our emergency department with a sudden onset of severe abdominal pain. He reported a 2-week history of back pain, with complaints of a mass on his left thigh, and was amidst further evaluation by a local physician. On arrival, he was tachycardic with profound diaphoresis. Abdominal examination revealed tenderness elicited on palpitation and a large pulsating mass in his left upper quadrant. His initial laboratory results were unremarkable other than a slight increase in white blood cell count. Computed tomography imaging revealed an intraperitoneal hematoma and an extensively dilated infrarenal abdominal aorta with a maximal diameter of 84 mm (Cover). The aneurysmal wall was irregular but well defined and appeared to contain intramural thrombus that showed subtle late-phase enhancement (A). An urgent midline laparotomy was performed. The surface of the aneurysm was irregular but covered by retroperitoneal membrane. It showed little adhesion to the omentum and no adhesion to the intestines (B). The abdominal aorta was clamped proximally and distally to the aneurysm, and the aneurysm was opened. Atherosclerotic findings inside were minimal, but there was a defect of the intimal layer of the anterior wall. The aneurysm was resected, and the abdominal aorta was reconstructed with a woven polyester tube graft. Histopathologic analysis revealed massive proliferation of fibroblastic tumor cells invading the entire layer of the aorta and destroying the intima. The adventitia remained patent, suggesting that the tumor originated from the aorta and was not a metastatic entity from adjacent organs. Furthermore, the tumor cells formed fascicles with vague herringbone pattern (C), consistently stained positive for vimentin, and showed Ki-67 labeling index of 50%. Taken together, these findings suggested that a high-grade adult fibrosarcoma (World Health Organization classification) arising from the aortic wall led to a ruptured pseudoaneurysm. Postoperative magnetic resonance imaging revealed multiple metastasis to the vertebral bodies and adductor muscles, and the patient was promptly introduced to ifosfamide-based chemotherapy combined with radiotherapy. Primary aortic sarcomas are extremely rare, but may cause aortic rupture,1 dissection, or ischemia,2 and attention is required when diagnosing and treating aortic emergencies. Written consent was obtained from the patient for publication of the study. REFERENCES 1. 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. 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. Submitted May 21, 2013; accepted May 5, 2014. From the Department of Cardiovascular Surgerya and the Department of Pathology,b Tachikawa Medical Center. Author conflict of interest: none. E-mail: [email protected]. 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. J Vasc Surg 2015;61:1066 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2014.05.004
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