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Their incidence has increased dramatically during recent years, probably due to the different sexual habits and changes in the prevalence of HIV\/ AIDS and HPV virus carriers, among other factors. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety eight percent of all vulvar tumors are benign and only 2% are malignant. The overall incidence of tumors with vulvar location is between two and seven cases per 100,000 women, and it increases with age, while the death rate is estimated at 0.7 per 100,000 women. Sarcomas of the vulva comprise approximately 1\u20133% of all vulvar cancers, with leiomyosarcomas, epithelioid sarcomas, and rhabdomyosarcomas being the most common among them. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In this paper, we present the most common forms of sarcomas of the vulva (leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, rhabdomyosarcoma) in order to emphasize the broad differential diagnosis, rare appearance, non-specific clinical picture, aggressive course, and high mortality.<\/jats:p>","DOI":"10.1177\/0394632015576029","type":"journal-article","created":{"date-parts":[[2015,3,27]],"date-time":"2015-03-27T15:16:51Z","timestamp":1427469411000},"page":"168-177","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":32,"title":["Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1"],"prefix":"10.1177","volume":"28","author":[{"given":"AA","family":"Chokoeva","sequence":"first","affiliation":[{"name":"Onkoderma-Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Tchernev","sequence":"additional","affiliation":[{"name":"Policlinic for Dermatology and Venereology, University Hospital Lozenetz, Sofia, Bulgaria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JC","family":"Cardoso","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University Hospital of Coimbra, Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JW","family":"Patterson","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of Virginia Health System, Charlottesville, VA, USA"},{"name":"Department of Pathology, University of Virginia Health System, Charlottesville, VA, 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