Primary extramammary Paget disease of the anogenital area: morphological features of the tumor and their importance for the disease recurrence

Abstract

Extramammary Paget’s disease (EMPD) is a rather rare variant of adenocarcinoma, most often localized in the anogenital area or perineum. The aim of our study was to determine the prognostic significance of histological variants of primary EMPD in relation to the risk of tumor recurrence. Material and methods. 202 samples of primary EMPD were taken from 158 patients. Sections stained with hematoxylin and eosin were examined. Results and discussion. 23 patients (14.6 %) had recurrent EMPD. The study revealed that «glandular», «pigmented», «signet ring», «acantholytic» morphological variants of EMPD, as well as syringocystadenocarcinoma papilliferum in situ–like changes and the presence in the dermis of structures similar to eccrine syringoma, are associated with the development of disease recurrence. Conclusion. The «signet-ring» and «pigment» morphological variants of EMPD have the highest predictor value in relation to the risk of disease recurrence. The «signet ring-cell» variant of primary EMPD is the only one that influences the time of the first recurrence of the disease.

Keywords

• extramammary paget’s disease
• vulva
• perineum
• anogenital region
• relapse
• morphology
• signet ring cell variant