Intimal angiosarcoma of the aorta: Report of a case and review of the literature

Abstract

Although extremely rare, the group of primary malignant tumors of the aorta (PMTA) exhibits enormous histologic heterogeneity. In most cases, diagnosis is established late in the course of the disease; the median survival time is only a few months. We present the case of a 75-year-old patient with an intimal angiosarcoma of the infrarenal abdominal aorta and discuss the clinical presentation, histopathologic diagnosis, and classification of primary aortic sarcomas. A critical review of the diagnostic and therapeutic management in this case revealed that the atypical aortic thrombus should have prompted a comprehensive preoperative diagnostic work-up, specifically with magnetic resonance tomography of the aorta and bone scintigraphy.

18F-FDG PET/CT for Detecting Sarcoma of the Aorta in a Patient with Takayasu Arteritis

“…Primary malignancies such as angiosarcoma of the aorta are extremely rare. They occur more frequently in men with a male-tofemale ratio of 2:1 [1][2][3]. The average age of those affected is in their 60s [1][2][3].…”

“…They occur more frequently in men with a male-tofemale ratio of 2:1 [1][2][3]. The average age of those affected is in their 60s [1][2][3]. At the time of initial presentation, >50 % of patients have metastatic disease [3][4][5].…”

“…The average age of those affected is in their 60s [1][2][3]. At the time of initial presentation, >50 % of patients have metastatic disease [3][4][5]. Sarcomas of the aorta are classified into intimal angiosarcomas of endothelial origin, intimal myofibroblastic sarcomas of mesenchymal origin, and mural sarcomas of medial or adventitial origin of the aorta, depending on the immunohistochemical pattern of the tumors [3].…”

“…At the time of initial presentation, >50 % of patients have metastatic disease [3][4][5]. Sarcomas of the aorta are classified into intimal angiosarcomas of endothelial origin, intimal myofibroblastic sarcomas of mesenchymal origin, and mural sarcomas of medial or adventitial origin of the aorta, depending on the immunohistochemical pattern of the tumors [3]. The majority of cases are believed to arise in the intima as an intraluminal growth, which forms a plaque-like structure along the intima or presents with a polypoid intraluminal growth [5,6].…”

“…The majority of cases are believed to arise in the intima as an intraluminal growth, which forms a plaque-like structure along the intima or presents with a polypoid intraluminal growth [5,6]. Mural sarcomas are defined as extra-aortic soft tissue sarcomas [3]. Mural sarcomas behave less aggressively than intimal sarcomas, despite the very similar overall and average survival (mean range, 8-14 months) [3,6,7].…”