Primary epithelioid sarcoma of the vulva in pregnancy

Primary epithelioid sarcoma (ES) of the vulva is an extremely rare tumor, and to date, only 20 cases have been reported. We report the second case of vulvar ES presenting in pregnancy. A 25-year-old woman, at 6 weeks of gestation, was diagnosed as having vulvar sarcoma and was referred to our hospital. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA), and wimentin. The tumor cells did not stain for S-100, SMA, HMB-45, desmin, LCA, CD-34, or estrogen and progesterone receptors. The tumor showed diffuse and strong staining for CD-117 (c-kit). After the curettage, she underwent local wide excision and left inguinal lymph node dissection and received external-beam radiation therapy. At 16 months of follow-up the patient was well without any disease recurrence. The appropriate treatment of vulvar ES is not clear, and the role of chemotherapy is disappointing; however, it might be worth investigating the role of Imatinib in c-kit overexpressing vulvar ES.