Zusammenfassung
GRUNDLAGEN: Die seltenen malignen Dünndarmtumore stellen eine heterogene Gruppe von Neubildungen dar, wobei es sich am häufigsten um Adenokarzinome, gefolgt von neuroendokrinen Tumoren, Lymphomen, gastrointestinalen Stromatumoren und Leiomyosarkomen handelt. Aufgrund ihrer Seltenheit sind die Erfahrungen mit dem diagnostischen wie auch mit dem therapeutischen Management dieser Tumorgruppe in Einzelinstitutionen oft gering. METHODIK: Auf Basis der Literatur werden Inzidenz, Risikofaktoren, Klassifikation und Symptome dieser Tumoren dargestellt. Weiters werden der diagnostische Allgorithmus und die derzeitige Therapie von malignen Tumoren des Dünndarms aufgezeigt. ERGEBNISSE: Aufgrund der unspezifischen Symptomatik werden diese Tumore meist sehr spät diagnostiziert. Neben Endoskopie und Dünndarmkontrastmitteldarstellung kommt heute der Kapselendoskopie ein hoher Stellenwert in der Diagnose dieser Tumore zu. Gute Langzeitüberlebensergebnisse bei Patienten mit Dünndarmkarzinom lassen sich nur durch radikale chirurgische Sanierung mit regionaler Lymphadenektomie erreichen. Für das chirurgische Vorgehen bei neuroendokrinen Dünndarmtumoren sind Tumorgröße- und Lokalisation ausschlaggebend. Lymphome des Dünndarms werden multimodal behandelt. Bei Vorliegen von Multilokalität bzw. präoperativ nachgewiesenem Lymphknotenbefall stellt das primär systemische Vorgehen die Therapie der Wahl dar. Die sehr seltenen Dünndarmsarkome werden chirurgisch behandelt. Für gastrointestinale Stromatumoren des Dünndarms hat sich zusätzlich die medikamentöse Therapie mit STI-571 (Imatinib, Glivec®) etabliert. SCHLUSSFOLGERUNGEN: Eine präoperative Diagnose ist nur in etwa 50 % aller malignen Dünndarmtumoren möglich. Dementsprechend erfolgt die Diagnose in vielen Fällen erst durch die Laparotomie und nachfolgende histologische Untersuchung des entfernten Tumors. In der Behandlung dieser Tumoren kommt dem radikalen chirurgischen Vorgehen eine entscheidende Bedeutung zu. Eine hoffnungsvolle neue sehr erfolgreiche Therapieoption konnte in die Behandlung der gastrointestinalen Stromatumore eingeführt werden.
Summary
BACKGROUND: The rare malignant tumors of the small intestine comprise a heterogeneous group. Adenocarcinomas are the most frequent, followed by neuroendocrine tumors, lymphomas, gastrointestinal stromal tumors (GIST) and leiomyosarcomas. Because of their rarity, individual institutions often have little experience with diagnostic and therapeutic management of this tumor group. METHODS: On the basis of the literature, incidence, classification, risk factors and symptoms of these tumors are presented. Furthermore, diagnostic algorithms for malignant small-intestinal tumors, as well as recent therapeutic modalities, are shown. RESULTS: Due to their unspecific symptoms, these tumors are usually diagnosed late. Along with endoscopy and contrast studies, capsule endoscopy has become an important tool in the diagnosis of these tumors. Good long-term survival can only be achieved with radical surgery including regional lymphadenectomy. With neuroendocrine tumors, tumor size and location will determine the surgical procedure. Therapy of small-intestinal lymphomas is multimodal. When there are multiple tumors, or lymph-node involvement has been shown preoperatively, as is usually the case, primary systemic treatment is preferred. Small-intestinal sarcomas are very rare and are treated surgically. For gastrointestinal stromal tumors involving the small intestine, STI-571 (Imatinib, Glivec®) has additionally become established as pharmacotherapy. CONCLUSIONS: Preoperative diagnosis is established in only 50 % of malignant small intestinal tumors. So in many cases, these tumors are diagnosed by laparotomy and following histological examination of the resected tumor. In most of these tumors radical surgery is the decisive treatment option. A promising new treatment modality with very good results was introduced for the therapy of gastrointestinal stromal tumors.
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Hauser, H. Guidelines – malignant tumors of the small intestine. Eur Surg 38, 118–123 (2006). https://doi.org/10.1007/s10353-006-0231-2
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DOI: https://doi.org/10.1007/s10353-006-0231-2