| Summary: | Introduction
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.
Case presentation
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, tumor cells displayed CD99, NIKX2.
Conclusions
In all cells and proliferative capacity of KI-67 in 40% of tumor cells, but other markers were negative.
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