Maligne Tumoren der Aorta

Zusammenfassung

Aortale Malignome sind seltene Erkrankungen von denen bisher nur knapp 200 Fälle in der Literatur beschrieben sind. Am häufigsten sind High-Grade-Sarkome in über 80%, die überwiegend intimal wachsen und an jeder Lokalisation der Aorta vorkommen können. Neben unspezifischen Symptomen wie Fieber, Hypertonie und allgemeiner Schwäche, manifestieren sich Aortensarkome in einem Drittel als Embolie oder im Falle von muralen Sarkomen als Aneurysma oder Ruptur. In der Diagnostik gilt die MRT als Goldstandard, da CT-Angiografie oder PET-CT nur unspezifische Zeichen aufweisen können. Da sich aortale Malignome häufig als andere Erkrankungen maskieren, sind Diagnostik und Therapie oft verzögert. In der Therapie steht, wenn noch möglich, die radikale Tumorentfernung im Vordergrund, auf die ggf. eine adjuvante medikamentöse Therapie folgt. Das 1-, 3- und 5-Jahres-Überleben liegt nach Literaturlage bei 26%, 7,6% und 3,5%.

Abstract

Aortic malignancies are rare diseases, of which only about 200 cases have been described in the literature. Most common forms are high-grade sarcomas, in over 80% of patients, which predominantly grow intimately and which can occur at any location in the aorta. In addition to non-specific symptoms, such as fever, hypertension and general weakness, aortic sarcomas in one third of patients manifest as peripheral embolism or, in the case of mural sarcoma, as an aneurysm or aortic rupture. In diagnostic testing, MRI is considered the gold standard, as CT angiography or PET CT can only show non-specific signs. Since aortic malignancies often manifest themselves as other diseases, diagnosis and therapy are often delayed. In therapy, the focus is on radial tumour resection with aortic replacement, possibly followed by adjuvant medical therapy. The 1-, 3- and 5-year survival rates are 26%, 7.6% and 3.5%, respectively.

Schlüsselwörter

aortale Tumoren - Angiosarkom - aortale Malignome

Keywords

aortic tumors - angiosarcoma - aortic malignancies

Publication History

Received: 01 June 2024

Accepted after revision: 15 April 2025

Article published online:
19 May 2025

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