Vulval epithelioid sarcoma - query bank. May 2009

Question: 35 year old with a right 15 mm epithelioid sarcoma of the vulva. Is a right vulvectomy and bilateral groin node dissection adequate surgical treatment?

Please note: the search for this response was carried out over 1 year ago. Eligible users may request an update of the evidence by submitting a new Clinical Query here.

Answer: No systematic reviews or randomised controlled trials of the management of epithelioid sarcoma of the vulva were identified. Two case reports (Kasamatsu and Moore) have summarised other reported cases of vulvar epithelioid sarcoma, and concluded that:

“Radical vulvectomy or extensive local excision with inguinal lymphadenectomy at the time of diagnosis is recommended as the treatment of choice.” (Kasamatsu)

“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.” (Moore)

(Evidence level III)

A more recent review has also been published (Altundag) although this is not available in the Library collection and so has not been assessed further.

The RCOG have also published a working party report on the management of vulval cancer.

Date of search: May 2009


  • Altundag K. Dikbas O. Oyan B. Usubutun A. Turker A. Epithelioid sarcoma of vulva: a case report and review of the literature. Medical Oncology. 21(4):367-72, 2004. Abstract
  • Kasamatsu T. Hasegawa T. Tsuda H. Okada S. Sawada M. Yamada T. Tsunematsu R. Ohmi K. Mizuguchi K. Kawana T. Primary epithelioid sarcoma of the vulva. International Journal of Gynecological Cancer. 11(4):316-20, 2001 Jul-Aug. Abstract
  • Moore RG. Steinhoff MM. Granai CO. DeMars LR. Vulvar epithelioid sarcoma in pregnancy. Gynecologic Oncology. 85(1):218-22, 2002 Apr. Abstract
  • Royal College of Obstetricians and Gynaecologists. Management of vulval cancer. 2006.


Classification of evidence levels

Ia Evidence obtained from meta-analysis of randomised controlled trials.

Ib Evidence obtained from at least one randomised controlled trial.

IIa Evidence obtained from at least one well-designed controlled study without randomisation.

IIb Evidence obtained from at least one other type of well-designed quasi-experimental study.

III Evidence obtained from well-designed non-experimental descriptive studies, such as comparative studies, correlation studies and case studies.

IV Evidence obtained from expert committee reports or opinions and/or clinical experience of respected authorities.

This clinical query answer was produced following the clinical query protocol as described here.

The RCOG will not be liable for any special or consequential damages arising from the use or reliance on information contained within the Query Bank.

Date published: 11/05/2009


main menu