{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T22:59:46Z","timestamp":1782601186183,"version":"3.54.5"},"reference-count":24,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,1,27]],"date-time":"2023-01-27T00:00:00Z","timestamp":1674777600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Cervico-vaginal (CV) localization of extra-mammary Paget\u2019s disease (EMPD) of the vulva is extremely rare. In order to investigate the incidence risk and the pathognomonic clinical and pathological features of this condition, a retrospective analysis was conducted including 94 women treated for vulvar EMPD at the European Institute of Oncology, Milan, Italy, from October 1997 to May 2020. Overall nine patients developed CV involvement from EMPD, with a cumulative incidence of 2.5% (95% CI: 0.5\u20138.0%) at 5 years, 6.5% (95% CI: 1.9\u201315.1%) at 10 years and 14.0% (95% CI: 4.8\u201327.8%) at 15 years, respectively. All cases except one were firstly detected by abnormal glandular cytology. None reported vaginal bleeding or other suspicious symptoms. The colposcopic findings were heterogeneous and could sometimes be misdiagnosed. Cervical and\/or vaginal biopsies were always performed for histopathological diagnosis by identification of Paget cells in the epithelium or stroma. Most patients developed invasive EMPD (5\/9) of the cervix and\/or vagina and underwent hysterectomy with partial or total colpectomy. CV involvement from EMPD should not be underestimated in women with a long-standing history of vulvar Paget\u2019s disease. Liquid-based cytology with immunocytochemistry represents a valuable tool for early diagnosis and should be routinely performed during the required lifelong follow-up.<\/jats:p>","DOI":"10.3390\/diagnostics13030464","type":"journal-article","created":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T08:28:23Z","timestamp":1675067303000},"page":"464","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Tips and Tricks for Early Diagnosis of Cervico-Vaginal Involvement from Extramammary Paget\u2019s Disease of the Vulva: A Referral Center Experience"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6909-1228","authenticated-orcid":false,"given":"Anna Daniela","family":"Iacobone","sequence":"first","affiliation":[{"name":"Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"},{"name":"Department of Biomedical Sciences, University of Sassari, 07100 Sassari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Elena","family":"Guerrieri","sequence":"additional","affiliation":[{"name":"Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora Petra","family":"Preti","sequence":"additional","affiliation":[{"name":"Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noemi","family":"Spolti","sequence":"additional","affiliation":[{"name":"Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluigi","family":"Radici","sequence":"additional","affiliation":[{"name":"Preventive Gynecology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Peveri","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences and Community Health, University of Milan, 20122 Milan, Italy"},{"name":"Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, 17177 Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Bagnardi","sequence":"additional","affiliation":[{"name":"Department of Statistics and Quantitative Methods, University of Milan-Bicocca, 20126 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8231-2814","authenticated-orcid":false,"given":"Giulio","family":"Tosti","sequence":"additional","affiliation":[{"name":"Dermato-Oncology Unit, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo","family":"Maggioni","sequence":"additional","affiliation":[{"name":"Department of Gynecology, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1838-0163","authenticated-orcid":false,"given":"Fabio","family":"Bottari","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, University of Sassari, 07100 Sassari, Italy"},{"name":"Division of Laboratory Medicine, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Scacchi","sequence":"additional","affiliation":[{"name":"Division of Diagnostic Cytology, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6283-2019","authenticated-orcid":false,"given":"Mariacristina","family":"Ghioni","sequence":"additional","affiliation":[{"name":"Division of Pathology, European Institute of Oncology IRCCS, 20141 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1097\/DSS.0000000000002064","article-title":"Extramammary Paget disease: A review of the literature\u2014Part I: History, epidemiology, pathogenesis, presentation, histopathology, and diagnostic work-up","volume":"46","author":"Morris","year":"2020","journal-title":"Dermatol. 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