Key result
Primary sarcomas of the aorta are rare tumors that can present with metastatic peripheral emboli and universally behave as malignant tumors despite occasional benign histologic descriptions.
Case Report (n=30)
Highlights the rare presentation of primary aortic sarcoma diagnosed premortem via peripheral emboli and emphasizes their malignant clinical behavior.
Consider aortic sarcoma in unexplained peripheral emboli; leaves open optimal management strategies for this rare entity.
Primary sarcomas of the aorta are rare vascular tumors. About 26 cases have been reported between 1973 and 1989. They are seldom diagnosed antemortem. The present report describes a patient with a myxoid malignant fibrous histiocytoma arising in the intima of the lower thoracic aorta, which was diagnosed premortem in metastatic peripheral emboli. The clinical and pathologic features of this patient along with a summary of the 29 primary aortic tumors previously reported (26 malignant and 3 benign) are discussed. Although few of these tumors have been described histologically as benign, all of them behave as malignant tumors.
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Tejada et al. (1991) conducted a case report in Primary sarcoma of the aorta (n=30). Primary sarcoma of the aorta was evaluated on Clinical and pathologic features. Primary sarcomas of the aorta are rare tumors that can present with metastatic peripheral emboli and universally behave as malignant tumors despite occasional benign histologic descriptions.
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