{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T14:45:51Z","timestamp":1790779551116,"version":"4.1.0"},"reference-count":120,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2023,3,16]],"date-time":"2023-03-16T00:00:00Z","timestamp":1678924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Vulvar Paget\u2019s disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1\u20132% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis is often difficult and delayed. Although VPD is typically diagnosed at a locally advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. Due to the limited and poor-quality evidence, there is no global consensus on optimal management. Therefore, we performed a systematic review of the literature through the main electronic databases to deepen the current knowledge of this rare disease and discuss the available treatment strategies. Wide surgical excision is recommended as the standard-of-care treatment and should be tailored to the tumor position\/extension and the patient\u2019s performance status. The goal is to completely remove the tumor and achieve clear margins, thus reducing the rate of local recurrences. Non-surgical treatments, such as radiotherapy, chemotherapy, and topical approaches, can be considered, especially in the case of unresectable and recurrent disease. In the absence of clear recommendations, the decision-making process should be individualized, also considering the new emerging molecular targets, such as HER2 and PD-L1, which might pave the way for future targeted therapies. The current review aims to raise awareness of this rare disease and encourage international collaboration to collect larger-scale, high-quality evidence and standardize treatment.<\/jats:p>","DOI":"10.3390\/cancers15061803","type":"journal-article","created":{"date-parts":[[2023,3,17]],"date-time":"2023-03-17T02:29:59Z","timestamp":1679020199000},"page":"1803","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Vulvar Paget\u2019s Disease: A Systematic Review of the MITO Rare Cancer Group"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5501-1936","authenticated-orcid":false,"given":"Giuseppe","family":"Caruso","sequence":"first","affiliation":[{"name":"Department of Maternal and Child Health and Urological Sciences, Sapienza University of Rome, Policlinico Umberto I, 00161 Rome, Italy"},{"name":"Department of Experimental Medicine, Sapienza University of Rome, Policlinico Umberto I, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1595-104X","authenticated-orcid":false,"given":"Amelia","family":"Barcellini","sequence":"additional","affiliation":[{"name":"Radiation Oncology Unit, Clinical Department, CNAO National Center for Oncological Hadrontherapy, 27100 Pavia, Italy"},{"name":"Department of Internal Medicine and Medical Therapy, University of Pavia, 27100 Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Mazzeo","sequence":"additional","affiliation":[{"name":"Department of Medicine (DAME), University of Udine, 33100 Udine, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Gallo","sequence":"additional","affiliation":[{"name":"Department of Maternal and Child Health and Urological Sciences, Sapienza University of Rome, Policlinico Umberto I, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8551-266X","authenticated-orcid":false,"given":"Maria Giuseppa","family":"Vitale","sequence":"additional","affiliation":[{"name":"Department of Oncology and Hematology, University Hospital of Modena, 41100 Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Passarelli","sequence":"additional","affiliation":[{"name":"Department of Urology and Gynecology, Istituto Nazionale Tumori IRCSS, \u201cFondazione G. Pascale\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgia","family":"Mangili","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Unit, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8836-2633","authenticated-orcid":false,"given":"Sandro","family":"Pignata","sequence":"additional","affiliation":[{"name":"Department of Urology and Gynecology, Istituto Nazionale Tumori IRCSS, \u201cFondazione G. Pascale\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Innocenza","family":"Palaia","sequence":"additional","affiliation":[{"name":"Department of Maternal and Child Health and Urological Sciences, Sapienza University of Rome, Policlinico Umberto I, 00161 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1111\/j.1440-0960.2012.00898.x","article-title":"Extramammary Paget\u2019s Disease of the Vulva: An Annotated Review of the Current Literature","volume":"54","author":"Delport","year":"2013","journal-title":"Australas. J. Dermatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/j.det.2010.08.002","article-title":"Extramammary Paget\u2019s Disease: Summary of Current Knowledge","volume":"28","author":"Lam","year":"2010","journal-title":"Dermatol. 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