Primary Sarcoma of the Aorta and Lower Extremity Arteries: An Analysis of 26 Cases.
Jonathon E Heath, Lauren F Xu, Allen P Burke. University of Maryland Medical Center, Baltimore
Background: Primary sarcomas of the aorta and lower extremity arteries are rare. The tumors are often initially mistaken for aneurysm or atherosclerotic disease, and are therefore usually diagnosed late in the disease course. We present a series of 26 aortic and lower extremity artery sarcomas with clinicopathologic data.
Design: Data was collected retrospectively by reviewing clinical history, operative reports, histology, and immunohistochemical stains.
Results: Of the 26 cases, there were 16 men (67.7 ± 13 years) and 10 women (58.6 ± 18 years). Tumors occurred in the abdominal aorta (12), descending thoracic aorta (9), femoral artery (4), and ascending aorta (1). Presenting symptoms included claudication (8), pain (7), abdominal aortic aneurysm (AAA) (3), renal artery stenosis (1), aortic rupture (1), vasculitis (1), bowel ischemia (1), recurrence of a previously resected aortic sarcoma (1) and discovered incidentally at surgery or autopsy (3). The diagnosis was not suspected clinically in any case. The tumors were sampled by aortectomy, endoluminal excision, AAA repair, and at autopsy.
Histologically, there were 12 undifferentiated pleomorphic sarcomas (UPS), 11 epithelioid angiosarcomas, 2 angiosarcoma, and 1 osteosarcoma. Typically there was a viable layer of malignant cells facing the lumen, overlying necrotic tumor and fibrin. Immunohistochemically, epithelioid angiosarcomas were cytokeratin and CD34 positive, and UPS were positive for vimentin, and also for actin in fewer than half of the cases.
Conclusions: Aortic sarcomas have a variety of clinical presentations, and are rarely suspected clinically, with the diagnosis first made at surgical resection. Mean age at presentation is in the 7th decade, with a slight male predominance (1.6:1). There are two major histologic types, undifferentiated pleomorphic sarcoma and epithelioid angiosarcoma. The distinction between the latter and carcinoma is facilitated by immunohistochemical stains.
Wednesday, March 2, 2011 1:00 PM
Poster Session VI # 27, Wednesday Afternoon