Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

Arch Gynecol Obstet. 2012 Mar;285(3):785-9. doi: 10.1007/s00404-011-2011-x. Epub 2011 Jul 30.


Purpose: 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.

Conclusion: 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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Antineoplastic Agents / therapeutic use
  • Bone Neoplasms / diagnosis
  • Bone Neoplasms / pathology*
  • Bone Neoplasms / therapy
  • Combined Modality Therapy
  • Female
  • Humans
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / secondary
  • Lung Neoplasms / therapy
  • Sarcoma, Ewing / diagnosis
  • Sarcoma, Ewing / secondary*
  • Sarcoma, Ewing / therapy
  • Treatment Outcome
  • Vulvar Neoplasms / diagnosis
  • Vulvar Neoplasms / secondary*
  • Vulvar Neoplasms / therapy


  • Antineoplastic Agents