Key result
Intimal sarcoma presents as acute mesenteric ischemia with rapid re-thrombosis leading to fatal multiorgan failure.
Why the study?
Primary malignant tumors of the large arteries are exceedingly rare, diagnostically challenging, and tend to mimic thromboembolic disease, often causing delayed diagnosis and poor outcomes.
Case Report (n=1)
No
Intimal sarcoma should be considered in the differential diagnosis for patients presenting with atypical or recurrent arterial thrombosis, particularly when multiple visceral vessels are involved.
Consider intimal sarcoma in thromboembolic mimics to reduce diagnostic delay; hypothesis-generating for early biopsy and targeted therapy trials.
Primary malignant tumors of the large arteries are exceedingly rare and diagnostically challenging. Undifferentiated intimal sarcomas and leiomyosarcomas are the major types encountered. Intimal sarcomas are highly aggressive neoplasms characterized by intraluminal growth and a tendency to mimic thromboembolic disease, often resulting in delayed diagnosis and poor outcomes. We report the case of a 63-year-old man who presented with progressive abdominal pain and weight loss and rapidly developed acute mesenteric ischemia due to extensive intraluminal obstruction of the descending and abdominal aorta with involvement of major visceral branches. Despite emergency surgical interventions, including thrombectomy, vascular bypass, and four re-laparotomies, the clinical course was marked by rapid re-thrombosis, sepsis, and multiorgan failure. Histopathological examination of retrieved intravascular material revealed a high-grade undifferentiated sarcoma that was confirmed at autopsy as an undifferentiated intimal sarcoma of the large arteries. This case highlights the diagnostic pitfalls of malignant vascular tumors and underscores the importance of considering intimal sarcoma in patients with atypical or recurrent arterial thrombosis, particularly when multiple visceral vessels are involved.
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Benignus et al. (2026) conducted a case report in Undifferentiated intimal sarcoma of the large abdominal arteries (n=1). Emergency surgical interventions (thrombectomy, vascular bypass, re-laparotomies) was evaluated. An undifferentiated intimal sarcoma of the large arteries presented as acute mesenteric ischemia with rapid re-thrombosis in a 63-year-old man, leading to fatal septic multiorgan failure.
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