Intimal sarcomas of the aorta and iliofemoral arteries: a clinicopathological study of 26 cases

Intimal Sarcoma of the Descending Aorta Mimicking Aortitis

“…Intimal aortic sarcomas are usually localized in the abdominal aorta between the celiac artery and the iliac bifurcation, and in 30% of cases the descending aorta is involved as seen in the present case [1,2,4,6]. The most common clinical presentation is related to tumor embolization with peripheral or mesenteric ischemia and necrotic skin lesions, or to metastasis, which occurs mainly to bones, liver, and lungs.…”

“…Indeed, most cases have been diagnosed either at necropsy or at examination of aortic surgical specimens [1,2]. An intimal sarcoma must be differentiated from a more common angiosarcoma, which shows definite and diffuse endothelial differentiation, and from a ley- omiosarcoma, which is characterized by smooth muscle marker immunoreactivity [4][5][6]. The pathogenesis of intimal sarcoma is still unknown, but genetic factors, including changes in the MDM2-p53pathway, seem to play important roles [7].…”

Treatment of intimal sarcoma of peripheral veins

“…The latter are predominantly located in the left atrium. Regarding sarcomas arising in large vessels, the sites most frequently involved are the pulmonary arteries and the aorta, followed by venous sarcomas, especially of the inferior vena cava [3] .…”

“…History and physical examination are an essential element of the workup, contrary to other STS, this type of tumor must never been biopsied. Diagnostic approach should have a high degree of suspicious, the clinical manifestations are unspecific, symptomatic thoracic aneurysm, dyspnea, distal embolization or sudden death for tumors at the aorta, this mistaken diagnosis can lead to inappropriate therapy, such as anticoagulation or thrombolysis [2] , [3] , [4] or local pain, claudication, motor or sensory loss, or as a mass in peripheral localization [5] , [8] . Imaging should include an adequate and high-quality imaging studies, an MRI preoperative of the extremity in compromise to determine resectability as well an IV contrast CT scan of the chest, abdomen and pelvis to exclude secondary tumors or metastatic disease.…”

“…The differential diagnosis of Intimal sarcomas should include benign lesions as well other soft tissue sarcoma (STS), diagnostic approach should have a high degree of suspicious, clinical manifestations are unspecific. Imaging should include an adequate and high-quality studies such as a Magnetic resonance imaging (MRI) preoperative to determine resectability as well as a CT scan of the chest, abdomen and pelvis to exclude metastatic disease [2] , [3] , [4] , [5] , [6] , [7] , [8] .…”