Zusammenfassung
Aufgrund einer differenten Pathogenese und Morphologie werden prinzipiell 2 Arten der vulvären intraephithelialen Neoplase (VIN) definiert: Die häufigste Form ist mit 98% die klassische VIN (mittleres Erkrankungsalter 30 bis 40 Jahre, Multifokalität, in etwa 90% Nachweis von „High-risk-HPV“). Die differenzierte VIN („simplex type“) ist mit 2–5% deutlich seltener. Sie wird in der Regel postmenopausal diagnostiziert (mittleres Erkrankungsalter etwa 65 Jahre), weist keine HPV-Assoziation, jedoch eine Alteration des p53-Tumorsuppressorgens auf. Sie wird meist im Zusammenhang mit einem (verhornenden) Vulvakarzinom diagnostiziert.
Die derzeit von der WHO favorisierte Unterteilung der VIN in VIN 1 bis 3 soll aufgrund neuerer Daten sowie aufgrund eines Vorschlages der „International Society for the Study of Vulvovaginal Diseases“ (ISSVD) ersetzt werden. Dabei entfällt die Kategorie der VIN 1; die VIN 2 und VIN 3 werden unter dem Begriff VIN subsummiert. An der Unterscheidung zwischen klassischer und differenzierter Form der VIN ändert sich nichts.
Prognosefaktoren beim Vulvakarzinom sind das Tumorstadium, der Nachweis inguinaler Lymphknotenmetastasen, deren Größe sowie der Nachweis eines Kapseldurchbruchs, die Invasionstiefe des Karzinoms sowie der Abstand zum Resektionsrand. Kontrovers werden das Grading und Lymphgefäßeinbrüche diskutiert.
Abstract
On the basis of varying morphology and pathogenesis, two types of vulvar intraepithelial neoplasias (VIN) have been defined: the common type (~98%), classic VIN, is characterised by strong association to high-risk HPV infection (up to 90%), occurrence at younger age (median age 30--40 years) and multifocality. The differentiated (or simplex) type is rare (1%--2%) and is associated with older age (median age 65 years) and p53 alterations. It is usually diagnosed in combination with vulvar (keratinizing) squamous cell carcinoma.
The classification currently preferred by the WHO in which VIN are classified into VIN 1--3 is to be replaced due to new data and according to a proposal by the International Society for the Study of Vulvovaginal Diseases (ISSVD) which eliminates VIN 1 and combines VIN 2 and 3 to VIN of common or, depending on histopathology, differentiated type.
Prognostically relevant factors in vulvar cancer include stage of disease, inguinal lymph node involvement, size of metastatic deposits and presence of extracapsular extension, depth of invasion and distance of the tumor from resection margins. Tumor grade and the presence of lymphovascular space involvement are controversially discussed.
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Horn, LC., Schierle, K., Klostermann, K. et al. Pathologisch-anatomische Aufarbeitung und Befundung von Präparaten bei Präkanzerosen und Karzinomen der Vulva. Pathologe 30, 56–64 (2009). https://doi.org/10.1007/s00292-008-1118-8
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DOI: https://doi.org/10.1007/s00292-008-1118-8