Zusammenfassung
„Low-grade“ muzinöse Neoplasien der Appendix (LAMN) sind selten und werden bei mindestens 0,13 % der Appendektomien in Deutschland diagnostiziert, wobei hier von einer deutlichen Untererfassung auszugehen ist. Bei Perforation der Tumoren können abdominelle Muzinansammlungen entstehen, das Pseudomyxoma peritonei (PMP). Die Herausforderung in der Therapie der LAMN ist das adäquate Vorgehen bei dem Zufallsbefund der Tumoren. Bei präoperativem Verdacht auf eine muzinöse Neoplasie muss abgewogen werden, ob bei dem Vorliegen einer Akutpathologie, meist einer Appendizitis, ein konservatives Vorgehen vertretbar ist oder ob eine unmittelbare Appendektomie notwendig ist. Ist dies der Fall, muss intraoperativ eine Perforation der Appendix vermieden werden und die Inspektion der gesamten Abdominalhöhle auf Muzinablagerungen erfolgen. Ist eine konservative Therapie möglich, sollte die weitere Therapie an einem Zentrum erfolgen. Wird erst intraoperativ der Verdacht geäußert, sollte auch hier die Perforation der Appendix vermieden werden und die gesamte Abdominalhöhle auf ein PMP untersucht werden. Liegt ein PMP vor, ist die Zytoreduktion und hypertherme intraperitoneale Chemoperfusion (HIPEC) an einem Zentrum indiziert. Wird die Diagnose im Rahmen der postoperativen histologischen Aufarbeitung gestellt, sollte evaluiert werden, ob eine Perforation vorlag und Muzinansammlungen im Operationsbericht vermerkt sind. Bei einer LAMN ohne den Nachweis eines PMP ist die Appendektomie die adäquate Therapie. Bei intraabdominellen Muzinansammlungen sollten Proben entnommen werden und die weitere Therapie an einem Zentrum mit ausreichender Expertise erfolgen. Eine Ileozökalresektion oder eine onkologische Hemikolektomie sind nicht indiziert. Nach adäquater Therapie sollten alle Patienten die Nachsorge mittels Schnittbildgebungen (präferenziell Magnetresonanztomographie) erhalten sowie die Bestimmung der Tumormarker karzinoembryonales Antigen (CEA), Karbohydratantigen (CA) 19–9 und CA 125.
Abstract
Low-grade appendiceal mucinous neoplasms (LAMN) are rare and are diagnosed in at least 0.13% of appendectomies in Germany, although significant underreporting is likely. Perforation of the tumors can result in abdominal mucinous collections, so-called pseudomyxoma peritonei (PMP). The challenge in the treatment of LAMN is the adequate approach to the incidental finding of these tumors. If a mucinous neoplasm is preoperatively suspected in cases of an acute condition, usually appendicitis, it must be weighed up whether a conservative approach is justifiable or whether immediate appendectomy is necessary. If this is the case, an intraoperative perforation of the appendix must be avoided and the complete abdominal cavity must be inspected for mucin deposits. If conservative treatment is possible, further treatment should take place at a specialized center. If the neoplasm is first found incidentally during surgery, perforation of the appendix should also be avoided and the entire abdominal cavity should be inspected for a PMP. If a PMP is present cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) should be performed in a specialized center. If LAMN are found during the postoperative histological work-up, it should be evaluated whether a perforation was present and mucin collections are noted in the surgical report. In the case of LAMN without evidence of a PMP, appendectomy is the adequate treatment. In cases of intra-abdominal mucinous collections, samples should be taken and further treatment should be performed at a center with sufficient expertise. An ileocecal resection or oncological hemicolectomy is not indicated. After adequate treatment, all patients should receive a follow-up using cross-sectional imaging (preferably magnetic resonance imaging, MRI) and determination of the tumor markers CEA, CA 19–9 and CA 125.
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F. Köhler, N. Matthes, J.F. Lock, C.-T. Germer und A. Wiegering geben an, dass kein Interessenkonflikt besteht.
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Köhler, F., Matthes, N., Lock, J.F. et al. Zufallsbefund muzinöse Neoplasie der Appendix. Chirurgie 94, 832–839 (2023). https://doi.org/10.1007/s00104-023-01910-0
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DOI: https://doi.org/10.1007/s00104-023-01910-0
Schlüsselwörter
- Muzinöse Appendixneoplasie
- Mukozele
- Pseudomyxoma peritonei
- Hypertherme intraperitoneale Chemoperfusion
- Appendektomie
- Zytoreduktion