{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T16:47:39Z","timestamp":1787071659237,"version":"build-2736575974"},"reference-count":68,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T00:00:00Z","timestamp":1741824000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous mesenchymal locally aggressive sarcoma with high recurrence. We report a case of recurrent vulvar DFSP, and a literature review was performed with a PubMed search using the terms \u201cDermatofibrosarcoma protuberans\u201d and \u201cvulva.\u201d Total 85 cases with vulvar DFSP were analyzed for their clinicopathological features among fibrosarcomatous DFSP (FS-DFSP) and non-FS-DFSP. Listless asymptomatic growth had resulted in delay in presentation of 2.3 years. Tumor size was significantly more in FS-DFSP (\n                    <jats:italic toggle=\"yes\">P<\/jats:italic>\n                    0.005). Higher mitotic rate is found to have strong association with FS-DFSP (\n                    <jats:italic toggle=\"yes\">P<\/jats:italic>\n                    0.0001). FS-DFSP has more recurrences compared with non-FS-DFSP (\n                    <jats:italic toggle=\"yes\">P<\/jats:italic>\n                    0.0001). DFSP with FS transformation was more aggressive as there was significant difference in metastasis (\n                    <jats:italic toggle=\"yes\">P<\/jats:italic>\n                    0.073, OR 0.18) and mortality (\n                    <jats:italic toggle=\"yes\">P<\/jats:italic>\n                    0.210, OR 0.19). To investigate the risk of outcome, 59 cases were included following the exclusion of &lt;6 months follow-up. Age (&gt;50 years) was found to be a risk for metastasis and death from the disease. Tumor size (&gt;5 cm) and mitosis (\u22655\/10-HPF among non-FS-DFSP) were found to be the risk for the LR. Different histological diagnosis was made initially in 32%, and local excision was performed without margins in 65% of vulvar DFSP because of initial incorrect diagnosis. Therefore, pre-treatment biopsy or re-biopsy should be performed with immunohistochemistry for definitive diagnosis to exclude histologic mimics. The optimal treatment is wide local excision with 3-cm margin or with margin assessment for both primary and recurrent DFSP. As most recurrences occur within the first three years of wide excision in DFSP, regular follow-up visits are advocated.\n                  <\/jats:p>","DOI":"10.4103\/ijpm.ijpm_695_21","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T12:00:08Z","timestamp":1741867208000},"page":"806-814","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Vulvar dermatofibrosarcoma protuberans masquerading as angiomyxoma: A case report and review of 85 cases"],"prefix":"10.4103","volume":"68","author":[{"given":"Niraj N.","family":"Mahajan","sequence":"first","affiliation":[{"name":"Department of Obstetrics and Gynecology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vartika","family":"Srivastava","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kusum","family":"Jashnani","sequence":"additional","affiliation":[{"name":"Department of Pathology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhiram M.","family":"Kasbe","sequence":"additional","affiliation":[{"name":"Department of Community Medicine, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nandita","family":"Kaushal","sequence":"additional","affiliation":[{"name":"Department of Pathology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akshaya","family":"Bansode","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anushka","family":"Papney","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,3,13]]},"reference":[{"key":"R1-20251208","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/0090-8258(76)90009-3","article-title":"Soft tissue sarcomas of vulva","volume":"4","author":"Davos","year":"1976","journal-title":"Gynecol Oncol"},{"key":"R2-20251208","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1111\/j.1471-0528.1981.tb00968.x","article-title":"Dermatofibrosarcoma protuberans of the vulva. 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