“…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.…”