Zusammenfassung
Das Ziel der vorliegenden Übersicht besteht darin, basierend auf einer repräsentativen
Auswahl relevanter Literaturverweise, den Paradigmenwechsel im chirurgischen Management
von Desmoidtumoren (DT) in Koinzidenz mit familiärer adenomatöser Polyposis (FAP)
darzustellen. Schwerpunkt liegt auf der Differenzialtherapie, die durch epidemiologische
Daten sowie Aussagen zur Diagnostik und Nachsorge ergänzt wird. DT sind seltene benigne
Weichteiltumoren, die nicht metastasieren, jedoch zu lokal infiltrativem Wachstum
neigen. Im Gegensatz zur Normalbevölkerung treten DT bei Patienten mit FAP häufiger
auf, zeigen ein anderes Verteilungsmuster und sind in besonderem Maße hinsichtlich
Morbidität und Mortalität verantwortlich. Intraabdominale DT machen hierbei den größten
Teil der Manifestationen aus und bereiten die meisten Probleme bei der Behandlung.
Als Ursache werden genetische und hormonelle Faktoren sowie ein vorangegangenes chirurgisches
Trauma diskutiert. Typische Symptome sind abdominale Schmerzen, Übelkeit und Erbrechen.
DT können sich jedoch auch als „Akutes Abdomen“ präsentieren. Diagnostisch ist die
Computertomografie richtungsweisend. Das Behandlungsspektrum umfasst verschiedene
medikamentöse Ansätze, eine Tumorresektion sowie die Strahlentherapie. Aufgrund der
geringen Häufigkeit der Erkrankung basieren Daten zur Diagnostik und Therapie auf Studien
mit kleinen Fallzahlen oder Fallberichten. Die Verbesserung der Prognose der Erkrankung
sowie die Entwicklung klarer Leitlinien sind daher auf die Durchführung internationaler
multizentrischer Studien angewiesen.
Abstract
Based on a representative selection of relevant references, the aim of this study
was to reflect the change of the algorithm in the surgical management of desmoid tumours
(DT) in cases of accompanying familial adenomatous polyposis (FAP). Main focus is
concerned with the basics of differential treatment, including additional considerations
on epidemiology, diagnosis, outcome and follow-up. DT are rare benign tumours that
do not metastasise but tend to invade locally. In contrast to the general population,
DT in patients with FAP are more common, show a different pattern of tumour sites
and cause considerable morbidity and mortality. Most DT occur in the abdominal cavity
and account for the majority of serious problems. Genetic disposition and hormonal
factors as well as prior surgical trauma are considered causative for the development
of DT. Characteristic symptoms are abdominal pain, nausea and vomiting but DT may
also present as acute abdomen. CT scan determines localisation and extension of the
tumour. Treatment includes various strategies of medication, surgical resection and
radiation. Data concerning diagnostic and therapeutic procedures are based on studies
with small case series or case reports only. Therefore data from international multicentre
studies are necessary for improving the prognosis and developing reliable and stringent
guidelines.
Schlüsselwörter
Desmoidtumoren - familiäre adenomatöse Polyposis - chirurgisches Vorgehen - Differenzialtherapie
Key words
desmoid tumours - familial adenomatous polyposis - surgical approach - differential
therapy
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Dr. med. Olof Jannasch
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