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We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: \u201cvulval leiomyoma,\u201d \u201cvulvar leiomyoma,\u201d \u201cvulval smooth muscle tumor,\u201d and \u201cexternal genitalia smooth muscle tumor.\u201d Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.<\/jats:p>","DOI":"10.1177\/0300060517721796","type":"journal-article","created":{"date-parts":[[2017,9,6]],"date-time":"2017-09-06T17:56:11Z","timestamp":1504720571000},"page":"663-674","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":38,"title":["Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor"],"prefix":"10.1177","volume":"46","author":[{"given":"Chao","family":"Sun","sequence":"first","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junkai","family":"Zou","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nasra","family":"Batchu","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qurat","family":"Ulain","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiang","family":"Du","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shulan","family":"Lv","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qing","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"},{"name":"Center of Big Data and Bioinformatics, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"},{"name":"Cardiovascular Research Institute, Morehouse School of Medicine, Atlanta, GA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiling","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"},{"name":"Center of Big Data and Bioinformatics, the First Affiliated Hospital of Xi\u2019an Jiaotong University, Xi\u2019an, Shaanxi, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2017,9,6]]},"reference":[{"key":"bibr1-0300060517721796","unstructured":"Hoffman BL, Williams JW. 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