{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T09:29:06Z","timestamp":1791538146506,"version":"4.3.4"},"reference-count":43,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2024,1,3]],"date-time":"2024-01-03T00:00:00Z","timestamp":1704240000000},"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>Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms \u201cdermatofibrosarcoma protuberans\u201d, \u201cvulva\u201d, and \u201cvulvar\u201d, alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients\u2019 quality of life. Management by a multidisciplinary team is highly recommended.<\/jats:p>","DOI":"10.3390\/cancers16010222","type":"journal-article","created":{"date-parts":[[2024,1,3]],"date-time":"2024-01-03T03:18:07Z","timestamp":1704251887000},"page":"222","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9726-3653","authenticated-orcid":false,"given":"Rosanna","family":"Mancari","sequence":"first","affiliation":[{"name":"Gynecologic Oncology Unit, IRCCS Regina Elena National Cancer Institute, 00144 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4370-8271","authenticated-orcid":false,"given":"Raffaella","family":"Cioffi","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynaecology, San Raffaele Scientific Institute, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francescapaola","family":"Magazzino","sequence":"additional","affiliation":[{"name":"Complex Operating Unit Ginecologia E Ostetricia, Ospedale Civile Di San Dona\u2019 Di Piave (Venezia), Aulss4 Veneto Orientale, 30027 San Don\u00e0 di Piave, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Attademo","sequence":"additional","affiliation":[{"name":"Oncology Unit, Ospedale del Mare, 80147 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6546-4354","authenticated-orcid":false,"given":"Miriam","family":"Sant\u2019angelo","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology, San Raffaele Scientific Institute, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluca","family":"Taccagni","sequence":"additional","affiliation":[{"name":"Department of Surgical Pathology, San Raffaele Scientific Institute, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8451-429X","authenticated-orcid":false,"given":"Giorgia","family":"Mangili","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynaecology, 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 IRCCS \u2018Fondazione G Pascale\u2019, 80144 Napoli, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice","family":"Bergamini","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynaecology, San Raffaele Scientific Institute, 20132 Milan, Italy"},{"name":"Faculty of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,1,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1097\/DSS.0000000000001113","article-title":"Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review","volume":"43","author":"Nguyen","year":"2017","journal-title":"Dermatol. 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