title: Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature.
abstract: OBJECTIVE
  Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related malignant neoplasms, usually affecting the skeletal system. Extraosseous ES/PNETs are uncommon, with occasional reports of tumors affecting the genitourinary tract. Only few cases of primary vulvar Ewing's sarcoma/PNET have previously been reported.
  
  
  METHODS
  We present a patient with primary vulvar Ewing's sarcoma with pulmonary metastasis who presented at the Department of Gynecology and Obstetrics, University of Schleswig-Holstein, Campus Luebeck.
  
  
  RESULTS
  The present report is the third case of a primary vulvar Ewing's sarcoma and the first constellation with pulmonary metastasis at diagnosis in the current literature. We present the diagnostic and therapeutic management including surgery, chemotherapy and radiation.
  
  
  CONCLUSIONS
  The treatment of the Ewing's sarcomas and PNETs requires a multidisciplinary systemic approach. Despite its rarity, the differential diagnosis of vulvar sarcoma must be considered in young women.
authors.affiliation: Department of Obstetrics and Gynecology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, Germany.
authors.forename: Katharina
authors.initials: K
authors.lastname: Kelling
authors.forename: Frank
authors.initials: F
authors.lastname: Noack
authors.forename: Christopher
authors.initials: C
authors.lastname: Altgassen
authors.forename: Peter
authors.initials: P
authors.lastname: Kujath
authors.forename: Michael K
authors.initials: MK
authors.lastname: Bohlmann
authors.forename: Friederike
authors.initials: F
authors.lastname: Hoellen
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000556:secondary / Q000628:therapy; D012512:Sarcoma, Ewing / Q000175:diagnosis / Q000556:secondary* / Q000628:therapy; D016896:Treatment Outcome; D014846:Vulvar Neoplasms / Q000175:diagnosis / Q000556:secondary* / Q000628:therapy
chemical_list: D000970:Antineoplastic Agents