Immunohistochemical and ultrastructural studies of a primary aortic intimal sarcoma

An intimal sarcoma of the abdominal aorta in a 63-year-old woman is reported. The clinical symptoms consisted of chronic arterial hypertension, vomiting and epigastric pain. Treatment was operative, but the patient died 20 hours after surgery. The studies were performed on a surgical specimen and on autopsy material. The aortic tumour consisted of pleomorphic spindle-shaped and giant cells. In the vertebral metastases a storiform pattern of the tumour cells was found. No specific features characteristic for leiomyogenic, lipogenic or an endothelial nature of the tumour giant cells was disclosed in electron microscopy and the picture rather indicated their histiocytic character. Of the 18 cellular markers studied, the immunostainings for vimentin and alpha-1-antichymotrypsin were evidently positive. The tumour was classified as a pleomorphic intimal aortic sarcoma probably a malignant fibrous histiocytoma (MFH). The literature on 26 previously published aortal tumours is reviewed with emphasis on their topographical distribution and histological classification. In only 4 previous cases was the final diagnosis supported by electron microscopical or immunopathological findings. The role of marker studies in the classification of aortal tumours is discussed.