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We review the literature and briefly discuss the epidemiology, treatment approaches, prognostic factors and expected outcomes of this rare disease.<\/jats:p>","DOI":"10.1136\/bcr-2021-242271","type":"journal-article","created":{"date-parts":[[2021,6,3]],"date-time":"2021-06-03T00:16:10Z","timestamp":1622679370000},"page":"e242271","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Extrarenal rhabdoid tumour of the vulva: diagnostic and management challenges"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4687-1058","authenticated-orcid":false,"given":"Sarita","family":"Kumari","sequence":"first","affiliation":[{"name":"Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jayashree","family":"Natarajan","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lalit","family":"Kumar","sequence":"additional","affiliation":[{"name":"Medical Oncology, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neerja","family":"Bhatla","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,6,2]]},"reference":[{"key":"2025082708294930000_14.6.e242271.1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197805)41:5<1937::AID-CNCR2820410538>3.0.CO;2-U"},{"key":"2025082708294930000_14.6.e242271.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ygyno.2007.05.045"},{"key":"2025082708294930000_14.6.e242271.3","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199702000-00002"},{"key":"2025082708294930000_14.6.e242271.4","doi-asserted-by":"crossref","DOI":"10.1097\/00000478-198910000-00004","article-title":"Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? 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