TITLE:
Primary Vulvar Ewing Sarcoma in a 30-Year-Old Woman: A Case Report
AUTHORS:
Fatoumata Matokoma Sidibe, Hajar Ouahbi, Rajae Kanab, Layla Tahiri, Fatim Zhara El Mrabet, Samia Arifi, Hind El Fatemi, Nawfel Mellas
KEYWORDS:
Ewing Sarcoma/Primitive Neuroectodermal Tumor, Vulva, Advanced Disease, Multimodal Treatment
JOURNAL NAME:
Case Reports in Clinical Medicine,
Vol.7 No.3,
March
21,
2018
ABSTRACT: Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered as Ewing sarcoma family of tumors (ESFT), characterized by chromosomal translocation t(11; 22) (q24; q12) leading to a chimeric transcript EWS-FLI1 in 85% of cases. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities in children, adolescents and young adults. It rarely occurs in the female genital tract. We report an extremely rare case of advanced vulvar Ewing sarcoma/PNET of the vulva confirmed by Fluorescence In Situ Hybridization (FISH) in a 30-year-old woman. The patient was treated by 6 cycles of chemotherapy followed by radiotherapy with favourable outcome.