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December 23, 2005
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Joseph J. Ricotta, II, M.D., Audra A. Noel, M.D., Dylan V. Miller, M.D., Peter Gloviczki, M.D., Charles J. Mullany, M.B.M.S., Sanjeev Kumar, M.B.B.S., Thomas C. Bower, M.D..
Mayo Clinic, Rochester, MN, USA.
Objective: With only 24 cases of primary angiosarcoma of the aorta (PASA) reported, standard diagnosis and treatment is unknown. To identify optimal management, we reviewed our single-institution experience.
Methods: PASA patients treated at our institution between 1985-2005 were reviewed. Other aortic malignancies and other arterial angiosarcomas were excluded.
Results: Nine patients were diagnosed with PASA (mean 65 years, range 54 to 82). Symptoms were claudication (n=4), abdominal pain and weight loss (n=3), back pain (n=3), and renovascular hypertension (n=2). Computed tomography (CT) scan demonstrated an irregular, eccentric, lobulated thrombus with peripheral enhancement in all patients. Tumors were located in the descending thoracic (5), infrarenal (3) and ascending (1) aorta. Metastases were present in 8/9 patients in visceral (n=5) or lower extremity arteries (n=3), and bone (n=2). Four patients underwent resection of all gross disease including metastases, two had thrombectomy alone and three were unresectable. All biopsies demonstrated high-grade pleomorphic sarcoma with expression of endothelial antigens (CD31, FLI-1, CD34). Five patients had postoperative treatment with chemotherapy (n=3) and radiation therapy (n=2). Mean survival was 23.5 months from presentation and 17.5 months from diagnosis. Survival for patients with resection for cure (37.5 months) was better than aortic thrombectomy (3.5 months), or no resection (0.5 months). Resection plus chemotherapy resulted in better survival (66.5 months) than resection alone (5 months) or chemotherapy alone (0.5 months).
Conclusion: PASA is a rare disease with a poor prognosis. Diagnosis with either pre-operative CT or intraoperative biopsy, and subsequent aggressive resection with adjuvant chemotherapy may improve survival.
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Primary Angiosarcoma Of The Aorta: A 20-Year Experience
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Joseph J. Ricotta, II, M.D., Audra A. Noel, M.D., Dylan V. Miller, M.D., Peter Gloviczki, M.D., Charles J. Mullany, M.B.M.S., Sanjeev Kumar, M.B.B.S., Thomas C. Bower, M.D..
Mayo Clinic, Rochester, MN, USA.
Objective: With only 24 cases of primary angiosarcoma of the aorta (PASA) reported, standard diagnosis and treatment is unknown. To identify optimal management, we reviewed our single-institution experience.
Methods: PASA patients treated at our institution between 1985-2005 were reviewed. Other aortic malignancies and other arterial angiosarcomas were excluded.
Results: Nine patients were diagnosed with PASA (mean 65 years, range 54 to 82). Symptoms were claudication (n=4), abdominal pain and weight loss (n=3), back pain (n=3), and renovascular hypertension (n=2). Computed tomography (CT) scan demonstrated an irregular, eccentric, lobulated thrombus with peripheral enhancement in all patients. Tumors were located in the descending thoracic (5), infrarenal (3) and ascending (1) aorta. Metastases were present in 8/9 patients in visceral (n=5) or lower extremity arteries (n=3), and bone (n=2). Four patients underwent resection of all gross disease including metastases, two had thrombectomy alone and three were unresectable. All biopsies demonstrated high-grade pleomorphic sarcoma with expression of endothelial antigens (CD31, FLI-1, CD34). Five patients had postoperative treatment with chemotherapy (n=3) and radiation therapy (n=2). Mean survival was 23.5 months from presentation and 17.5 months from diagnosis. Survival for patients with resection for cure (37.5 months) was better than aortic thrombectomy (3.5 months), or no resection (0.5 months). Resection plus chemotherapy resulted in better survival (66.5 months) than resection alone (5 months) or chemotherapy alone (0.5 months).
Conclusion: PASA is a rare disease with a poor prognosis. Diagnosis with either pre-operative CT or intraoperative biopsy, and subsequent aggressive resection with adjuvant chemotherapy may improve survival.
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