Document Type: Case Report
Department of urology, velayat Hospital, Gazvin university of medical sciences, Gazvin, Iran
Clinical research development center of Amirkabir hospital, Arak University of medical sciences, Arak , Iran
Urology Research Center, Sina Hospital, Tehran University of Medical Sciences, Tehran, Iran
Primary Ewing sarcoma of the vulva is an extremely rare tumor with less than 10 molecular confirmed reported cases. Urinary retention in this case is an unusual manifestation. We describe a 15 years-old female patient with advanced Ewing sarcoma of the vulva and urinary retention during chemotherapy. The technique of catheterization with two catheters could be interesting, in this case as well. When it comes to Immunohistochemistry, tumour cells displayed CD99, NIKX2 In all cells and proliferative capacity of KI-67 in 40% of tumour cells, but other markers were negative.
Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are two members of Ewing sarcoma family of tumors. These tumors are characterized by a t (11;22) (q24 ; q12) chromosomal translocation. Although this malignant round cell tumor is aggressive, fortunately it is sensitive to chemotherapy. Generally, ES/PNET is unusual in the female external genital. Immunohistochemical stains and molecular cytogenetic analysis requires to confirm. Primary ES of the vulva is as extremely rare tumor with only 10 molecular confirmed reported cases up to now.