A Proximal Epithelioid Sarcoma of the Vulva During Pregnancy: A Case Report and Review of Literature

We report case of epithelioid sarcoma of the vulva in pregnancy. Epithelioid sarcomas of the vulva are rare tumors with aggressive behavior, fast clinical course and poor prognosis. 40 cases are reported in literature. A 34-years old pregnant patient was diagnosed with vulvar ES, proximal type, in the 27th week of pregnancy. The right labium mass grew from 0.5 to 9 cm in 6 months. After biopsy, Cesarean Section was performed in the 32nd week of pregnancy. Following neoadjuvant chemotherapy, radical excision with dissection of regional, inguinal lymphatic nodes was performed, followed by radiotherapy. The patient relapsed 5 months later and died. No algorithms for the treatment of this condition exist. Poor prognostic factors are locoregional lymphatic node involvement, tumor size larger than 2 cm, deep infiltration, vascular involvement, presence of necrosis, high mitotic index. Due to the rarity of the disease no prospective trials are possible.