title: Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
abstract: Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. 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. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.
authors: Onkoderma-Policlinic for Dermatology and Dermatologic Surgery, Sofia, Bulgaria.
authors: A A
authors: AA
authors: Chokoeva
authors: Policlinic for Dermatology and Venereology, University Hospital Lozenetz, Sofia, Bulgaria georgi_tchernev@yahoo.de.
authors: G
authors: G
authors: Tchernev
authors: Department of Dermatology, University Hospital of Coimbra, Coimbra, Portugal.
authors: J C
authors: JC
authors: Cardoso
authors: Department of Dermatology, University of Virginia Health System, Charlottesville, VA, USA Department of Pathology, University of Virginia Health System, Charlottesville, VA, USA.
authors: J W
authors: JW
authors: Patterson
authors: Department of Urology, Medical University of Plovdiv, Bulgaria; University Clinic of Urology, University Hospital "St. George", Plovdiv, Bulgaria.
authors: I
authors: I
authors: Dechev
authors: Department of Urology, Medical University of Plovdiv, Bulgaria; University Clinic of Urology, University Hospital "St. George", Plovdiv, Bulgaria.
authors: S
authors: S
authors: Valkanov
authors: Department of Neuroscience, Psychology, Drug Research and Child Health - Neurofarba - Pharmacology and Toxicology Section, University of Florence, Florence, Italy.
authors: M
authors: M
authors: Zanardelli
authors: University of Rome "G.Marconi", Rome, Italy.
authors: T
authors: T
authors: Lotti
authors: Department of Dermatology and Allergology, Hospital Dresden-Friedrichstadt, Dresden, Germany.
authors: U
authors: U
authors: Wollina
mesh_terms: D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000473:pathology; D012509:Sarcoma / Q000175:diagnosis* / Q000473:pathology*; D014846:Vulvar Neoplasms / Q000175:diagnosis* / Q000473:pathology*
keywords: dermatofibrosarcoma protuberans; liposarcoma; sarcomas; synovial sarcoma; vulva