[Mucinous ovarian neoplasms. Prognostically mostly excellent, infrequently a wolf in sheep's clothing]

Zusammenfassung Muzinöse Ovarialtumoren stellen die zweithäufigste Gruppe neben den serösen Tumoren dar, wobei die Zystadenome mehr als 80 % ausmachen. Muzinöse Zystadenome, Borderlinetumoren/atypisch proliferierte muzinöse Tumoren und Karzinome unterscheiden sich dabei weder durch die klinische Symptomatik noch durch das durchschnittliche Erkrankungsalter. Borderlinetumoren/atypisch proliferierte muzinöse Tumoren und Karzinome treten typischerweise unilateral im Stadium I auf und haben eine exzellente Prognose. Sie sind häufig Zufallsbefunde oder zeigen eine unspezifische abdominelle Symptomatik. Der Erkrankungsgipfel liegt um das 50. Lebensjahr. Implantate bzw. peritoneale Metastasen sowie Bilateralität eines muzinösen Ovarialtumors sollten den dringenden Verdacht auf eine Metastase eines gastrointestinalen Malignoms nach sich ziehen. Weder eine Mikroinvasion, definiert durch eine Invasion bis max. 5 mm, noch ein intraepitheliales Karzinom, charakterisiert durch hochgradige Atypien, beeinflussen die Prognose von Borderlinetumoren. Muzinöse Karzinome zeigen meistens ein konfluentes, drüsiges Wachstumsmuster mit labyrinthartigen Strukturen, während ein destruktives, infiltratives oder gar knotiges histologisches Wachstumsmuster an die Möglichkeit einer Metastase denken lassen sollte. Die seltenen Wandknoten in Borderlinetumoren können einer sarkomartigen spindelzelligen Proliferation, aber auch anaplastischen Karzinomen ähneln, beeinflussen aber meist nicht die Prognose. Immunhistochemisch kann die Abgrenzung insbesondere zu Metastasen aus dem oberen Gastrointestinaltrakt schwierig sein (CK7+ und variable Expression von CK20 sowie CDX2). Pax8-Positivität spricht für ein Primum aus dem Ovar, muss aber nicht gegeben sein. Muzinöse Tumoren in Assoziation mit Teratomen des Ovars ähneln immunhistochemisch Kolontumoren (CK7−/CK20+/CDX2+). Die seltenen endozervikal differenzierten muzinösen Tumoren werden nun als seromuzinöse Tumoren bezeichnet. Sie enthalten 2 und mehr unterschiedliche Zelltypen, sind häufig mit Endometriose assoziiert und scheinen molekulargenetisch den endometrioiden Tumoren verwandt zu sein. Abstract Mucinous ovarian neoplasms represent the second largest group of epithelial ovarian tumors after serous neoplasms, of which benign cystadenomas constitute more than 80 %. Mucinous cystadenomas and carcinomas cannot be distinguished by the clinical features or the mean age of onset of the disease. They typically occur unilaterally, are confined to the adnexae (FIGO stage I) and clinically present with non-specific abdominal symptoms or are diagnosed by chance. The mean age of disease onset is around 50 years old. The prognosis is excellent. Implants, peritoneal metastases and bilateral occurrence of ovarian mucinous neoplasms should lead to the suspicion of metastasis particularly from a gastrointestinal tumor. Neither microinvasion defined as a maximum extent of invasion of 5 mm, nor intraepithelial carcinoma characterized by high grade atypia without invasion, affect the prognosis of mucinous borderline tumors. Mucinous carcinomas typically show confluent glandular, expansile growth that leads to a labyrinth-like pattern. A destructive infiltrative or nodular growth pattern, however, should lead to the consideration of metastasis. Mural nodules that may reveal a spindle cell sarcomatous or anaplastic carcinomatous pattern occur infrequently in mucinous and do not affect the prognosis. Pax8 positivity is indicative of a primary ovarian neoplasm. In this case, however, mucinous tumors associated with teratomas may show the colonic immunoreaction pattern (CK7−/CK20+/CDX2+). 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Author information Authors and Affiliations Corresponding author Rights and permissions About this article Cite this article Lax, S., Staebler, A. Muzinöse Ovarialtumoren. Pathologe 35, 327–335 (2014). https://doi.org/10.1007/s00292-014-1912-4 Published: Issue date: DOI: https://doi.org/10.1007/s00292-014-1912-4