Akute Extremitätenischämie als Erstsymptom eines gedeckt rupturierten, thrombosierten Pseudoaneurysmas eines undifferenziertem pleomorphen high grade Sarkoms der Arteria iliaca externa

Abstract

Primary malignant vascular tumors represent a rare cause of acute extremity ischemia. Due to unspecific symptoms the correct diagnosis is often delayed and confirmed in many cases as late as post mortem. Differential diagnosis of malignant vascular tumors should be considered in patients with acute ischemia, atypical history and absence of typical risk factors for vascular diseases. The overall prognosis of such tumors is poor. If possible, complete curative resection in combination with arterial reconstruction should be performed. Multimodal therapy has to be considered and discussed in appropriate tumor boards. We report a case of a 70-year-old male patient with acute ischemia and contained rupture of a pseudoaneurysm of the external iliac artery due to an undifferentiated high grade pleomorphic sarcoma. At the time of the primary operation, diffuse skeletal metastases were present but even detected postoperatively during staging. Therefore, no adjuvant or palliative therapy was initiated. In the postoperative course, recurrent non reversible ischemia was present followed by amputation of the right leg. The patient died 5 months after first operation. In the autopsy further metastases of lung and liver were found.