Intimal Sarcoma
Cyril Fisher, MD, DSc, FRCPath
Key Facts
Terminology
Malignant neoplasm arising from intimal aspect of large blood vessels
Clinical Issues
Pulmonary trunk or pulmonary artery
Descending thoracic or lower abdominal aorta
Can be found incidentally at autopsy
Poor prognosis
Macroscopic Features
Intravascular, polypoid
Microscopic Pathology
Pleomorphic spindle, polygonal, and multinucleated cells
Focal myxoid stroma is common
Necrosis often a feature
Specific differentiation sometimes identifiable focally (especially in pulmonary arterial tumors)
 This pulmonary artery sarcoma adheres to the vessel wall  and extends along a large part of its circumference. The bulk of the tumor lies within the arterial lumen  . |
 Intimal sarcoma often has a gelatinous appearance  reflecting marked myxoid stromal change within the tumor. Foci of hemorrhage  and necrosis  are also seen. |
TERMINOLOGY
Definitions
Malignant neoplasm arising from intimal aspect of large blood vessels
ETIOLOGY/PATHOGENESIS
Environmental Exposure
Rare association with Dacron prosthetic graft
CLINICAL ISSUES
Epidemiology
