The diagnostic challenge of aortic intimal sarcomas, from vasculitis-like syndrome to mesenteric ischemia: Report of two cases and individual patient data literature review

Abstract

Primary aortic intimal sarcomas (AIS) are rare but often fatal. They are highly malignant and often recur despite aggressive therapy. We report two cases of AIS presenting with nonspecific clinical symptomatology leading to initial delayed diagnosis and poor prognosis. The first patient, a 79-year-old female, presented with vasculitis-like symptoms and tumoral peripheral emboli, while the second, a 63-year-old male, with new-onset hypertension and mesenteric ischemia. Both recurred after radical resection and died 16 months after surgery. 140 additional cases were identified in the literature, and their demographic and clinical characteristics individually reviewed, further reaffirming the wide clinical spectrum dilemma leading to AIS poor outcome. Radical resection seemed beneficial and associated with increased patient survival. Extending the initial aortic resection should be favored over choosing the smallest graft to avoid early recurrence.

Keywords

• Aortic intimal sarcoma
• Angiosarcoma
• Delayed diagnosis
• Vasculitis
• Mesenteric ischemia