Primary malignant tumors of the aorta: clinical presentation, treatment, and course of different entities.

Abstract

The objective of this study was to analyze possible correlations between the clinical presentation and the course of patients with different types of primary malignant aortic tumors. A single academic center's experience was reviewed retrospectively. Four patients with primary malignant tumors of the aorta were treated in an 11-year period. Three different histologic entities were found: malignant fibrous histiocytoma, epitheloid angiosarcoma, and unclassified sarcoma. Two female patients presenting with clinical symptoms of vasculitis proved to have epitheloid aortic sarcoma. Both developed diffuse metastasis to bone and skin with initial lymphatic disease in the groin. The other patients developed local recurrence and pulmonary metastasis. Survival of the 4 patients was 11, 20, and 51 months, 1 patient with metastatic disease is still alive 6 months after surgery. Different types of malignant aortic tumors seem to have different clinical presentation and course.