Primary extramammary Paget disease of the anogenital area: morphological features of the tumor and their importance for the disease recurrence
https://doi.org/10.18699/SSMJ20210105
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.
About the Authors
A. M. Konstantinova
Saint-Petersburg Clinical Scientific and Practical Center for Specialized Types of Medical Care (Oncological); Saint-Petersburg State University; Saint-Petersburg Medico-Social Institute
Russian Federation
Russian Federation
Anastasia M. Konstantinova, candidate of medical sciences
197758, Saint-Petersburg, Leningradskaja str., 68a, Lit A
199004, Saint-Petersburg; 21 linea VO, 8a
195271, Saint-Petersburg; Kondratievsky av., Lit 72A
K. V. Shelekhova
Saint-Petersburg Clinical Scientific and Practical Center for Specialized Types of Medical Care (Oncological); Saint-Petersburg Medico-Social Institute
Russian Federation
Russian Federation
Kseniya V. Shelekhova, doctor of medical sciences
197758, Saint-Petersburg, Leningradskaja str., 68a, Lit A
195271, Saint-Petersburg; Kondratievsky av., Lit 72A
M. Michal
Charles University; Bioptical Laboratory
Czech Republic
Czech Republic
Michal Michal, MD, professor
30460, Pilsen, Svobody aly., 80
32600, Pilsen, Mikulashske sq., 4
D. V. Kazakov
Charles University; Bioptical Laboratory
Czech Republic
Czech Republic
Dmitry V. Kazakov, candidate of medical sciences, professor
30460, Pilsen, Svobody aly., 80
32600, Pilsen, Mikulashske sq., 4
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Review
For citations:
Konstantinova A.M., Shelekhova K.V., Michal M., Kazakov D.V. Primary extramammary Paget disease of the anogenital area: morphological features of the tumor and their importance for the disease recurrence. Сибирский научный медицинский журнал. 2021;41(1):50-59. (In Russ.) https://doi.org/10.18699/SSMJ20210105
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