title: Vulvar epithelioid sarcoma in pregnancy.
abstract: BACKGROUND
  Epithelioid sarcoma is a soft tissue tumor rarely found centrally and even less commonly on the vulva. Vulvar sarcoma in pregnancy is also exceedingly rare with only five cases reported to date, none of which have been an epithelioid sarcoma.
  
  
  METHODS
  We report a case of a 29-year-old woman presenting with a vulvar epithelioid sarcoma at 36 weeks of gestation. The patient underwent a radical resection 6 weeks postpartum followed by chemotherapy. Despite a radical hemivulvectomy and doxorubicin and ifosfamide chemotherapy, she developed pulmonary metastasis and died of tumor-related pulmonary failure secondary to her disease 612 months after diagnosis. To our knowledge this is the first case of a vulvar epithelioid sarcoma presenting during pregnancy. The English literature is reviewed and a total of 18 previous cases of vulvar epithelioid sarcoma have been reported outside of pregnancy. Insight into the biological behavior and therapeutic management of this disease is discussed.
  
  
  CONCLUSIONS
  The optimal management of vulvar epithelioid sarcoma remains to be determined. However, it would seem that early and aggressive surgical resection provides the best possibility for cure. The role of radiation and/or chemotherapy remains to be determined.
authors.affiliation: Department of Obstetrics and Gynecology, Program in Women's Oncology, Brown University, Providence, Rhode Island 02905, USA. rmoore@wihri.org
authors.forename: Richard G
authors.initials: RG
authors.lastname: Moore
authors.forename: Margaret M
authors.initials: MM
authors.lastname: Steinhoff
authors.forename: Cornelius O
authors.initials: CO
authors.lastname: Granai
authors.forename: Leslie R
authors.initials: LR
authors.lastname: DeMars
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D012509:Sarcoma / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D014846:Vulvar Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery