Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET)\nare considered as Ewing sarcoma family of tumors (ESFT), characterized by\nchromosomal translocation t(11; 22) (q24; q12) leading to a chimeric transcript\nEWS-FLI1 in 85% of cases. It typically involves the soft tissues of the\nchest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region\nand extremities in children, adolescents and young adults. It rarely occurs in\nthe female genital tract. We report an extremely rare case of advanced vulvar\nEwing sarcoma/PNET of the vulva confirmed by Fluorescence In Situ Hybridization\n(FISH) in a 30-year-old woman. The patient was treated by 6 cycles\nof chemotherapy followed by radiotherapy with favourable outcome.
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