Primary Vulvar Ewing Sarcoma in a 30-Year-Old Woman: A Case Report

Fatoumata Matokoma Sidibe,Samia Arifi,Hajar Ouahbi,Hind El Fatemi,Fatim Zhara El Mrabet,Nawfel Mellas,Rajae Kanab,Layla Tahiri

doi:10.4236/crcm.2018.73022

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.

Highlights

Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered Ewing sarcoma family of tumors (ESFT), characterized by chromosomal translocation t(11; 22) (q24; q12) leading to a chimeric transcript EWSFLI1 in 85% of cases [1]
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
In 5% - 10% of cases EWS gene is fused with other members of Erythroblast Transformation-Specific (ETS) gene (ERG, ETV 1, ETV 4 and FEV)

Summary

Introduction

Primary Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are considered Ewing sarcoma family of tumors (ESFT), characterized by chromosomal translocation t(11; 22) (q24; q12) leading to a chimeric transcript EWSFLI1 (a member of ETS gene) in 85% of cases [1]. ES/PNET is an aggressive malignant round cell tumor yet chemosensitive [2]. They commonly affect bones and especially diaphysis of longs bones. We present a case of 30-year-old woman with a confirmed ES arising in the vulva and we report a systematic review of the published literature regarding primary vulvar ES

Case Report

Discussion

Findings

Conclusions