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Pathologie der Präkanzerosen und der Karzinome von Vulva und Vagina sowie morphologische Prognosefaktoren

Pathology of precancerous lesions and carcinoma of the vulva and vagina as well as morphologic prognosic factors

Der Onkologe volume 15, pages 15–27 (2009)Cite this article

Zusammenfassung

Das Vaginal- und das Vulvakarzinom sind mit jeweils ca. 1% aller malignen Tumoren des weiblichen Genitales selten. In Analogie zum Dysplasiekonzept bei der Cervix uteri werden die Präkanzerosen der Vagina als vaginale intraepitheliale Neoplasien (VAIN) und die der Vulva als vulväre intraepitheliale Neoplasien (VIN) bezeichnet. Aufgrund einer differenten Pathogenese und Morphologie werden prinzipiell zwei Arten der VIN definiert: Die klassische VIN ist durch eine Assoziation zu High-Risk-HPV charakterisiert, einem Auftreten bei jungen Frauen und einer Multifokalität. Die differenzierte VIN („simplex“ bzw. „differentiated type“) ist deutlich seltener, wird in der Regel bei postmenopausalen Patientinnen diagnostiziert, weist keine Assoziation zu einer HPV-Infektion auf, jedoch eine Alteration von p53. Die meisten der invasiven Vaginal- bzw. Vulvakarzinome sind Plattenepithelkarzinome, gefolgt von den Adenokarzinomen. Die Datenlage zu prognostisch relevanten Faktoren beim Vaginalkarzinom ist eingeschränkt. Gut etabliert ist das Tumorstadium und bei radiotherapierten Karzinomen die Tumorgröße. Das Ausmaß des Befalls der Scheide durch den Tumor (befallene Drittel) wird ebenso kontrovers diskutiert wie die Bedeutung einer proximalen bzw. distalen vaginalen Lokalisation des Karzinoms. Relevante Prognosefaktoren beim Vulvakarzinom sind das Tumorstadium, der Nachweis (inguinaler) Lymphknotenmetastasen (LKM), die Größe der LKM sowie der Nachweis eines Kapseldurchbruchs, die Invasionstiefe des Karzinoms und der Abstand zum Resektionsrand. Kontrovers werden das Grading und der Nachweis von Lymphgefäßeinbrüchen diskutiert. Voraussetzung für die adäquate Erhebung prognostisch und therapeutisch relevanter Faktoren ist eine sorgfältige pathologisch-anatomische Befundung.

Abstract

The popular terms for vaginal and vulvar squamous precancerous lesions are vaginal intraepithelial neoplasia (VAIN) and vulvar intraepithelial neoplasia (VIN). Their grading is similar to the CIN concept. Based on morphology and pathogenetic data, two types of VIN have been defined. The common type is characterized by a strong association with high-risk human papillomavirus infection, occurrence at a younger age, and multifocality. The differentiated (or simplex) type is rare and shows an association with older age and p53 alterations. The International Society for the Study of Vulvovaginal Disease has proposed a new VIN classification that eliminates VIN 1 and combines VIN 2 and VIN 3 to VIN of common or—depending on the histopathology—differentiated type. The majority of invasive carcinomas of the vagina and vulva are squamous cell carcinomas, followed by adenocarcinoma. Data are limited regarding prognostic factors in vaginal carcinoma. Tumor stage is the strongest prognosticator, followed by tumor size in patients treated by radiation therapy. Some controversies exist regarding the impact of the tumor’s location within the vagina and its extent of infiltration of various parts of the vagina. Relevant prognostic factors in vulvar cancer are the stage of the disease, the inguinal lymph node involvement, the size of the metastatic deposits, the presence of extracapsular extension, the depth of invasion, and the distance of the tumor to the resection margins. Other factors in dispute are the tumor grade and the presence of lymphovascular space involvement.

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  1. Abt. Mamma-, Gynäko- & Perinatalpathologie, Institut für Pathologie Universität Leipzig, Liebigstraße 26, 04103, Leipzig, Deutschland

    L.-C. Horn

  2. Institut für Pathologie, Universität Leipzig, Leipzig, Deutschland

    K. Schierle

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Horn, LC., Schierle, K. Pathologie der Präkanzerosen und der Karzinome von Vulva und Vagina sowie morphologische Prognosefaktoren. Onkologe 15, 15–27 (2009). https://doi.org/10.1007/s00761-008-1537-4

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