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Our patient presented with a 6\u2009\u00d7\u20094 cm right subcutaneous solid vulvar lesion causing pain and discomfort. Pathology and immunohistochemistry staining showed strong positivity for CD99 and vimentin, favoring the diagnosis of ES\/pPNET. Magnetic resonance imaging showed a 6\u2010cm lesion in the right vulvar region with enlarged bilateral inguinal and right iliac lymph nodes. Fluorescence <jats:italic>in situ<\/jats:italic> hybridization test for translocation t(11;22)(q24;q12) was positive, confirming the diagnosis. The patient received three\u2009cycles of neoadjuvant chemotherapy with vincristine, adriamycin, cyclophosphamide alternating with ifosfamide and etoposide with complete response. The patient underwent vulvar radical local excision. Residual tumor measured 1.6\u2009cm with free margins. She received four additional cycles of adjuvant chemotherapy and 30 sessions radiotherapy. She is currently disease free after 37\u2009months. No ES\/pPNET cases with pelvic lymph nodes metastasis were ever reported.<\/jats:p>","DOI":"10.1111\/jog.14399","type":"journal-article","created":{"date-parts":[[2020,8,6]],"date-time":"2020-08-06T05:30:05Z","timestamp":1596691805000},"page":"2185-2192","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Primary vulvar Ewing sarcoma\/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature"],"prefix":"10.1111","volume":"46","author":[{"given":"Alaa","family":"El Housheimi","sequence":"first","affiliation":[{"name":"Department of Obstetrics and Gynecology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali","family":"Khalil","sequence":"additional","affiliation":[{"name":"Division of Gynecologic Oncology, Department of Obstetrics and Gynecology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dalia","family":"Khalifeh","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghina","family":"Berjawi","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Radiology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhieddine","family":"Seoud","sequence":"additional","affiliation":[{"name":"Division of Gynecologic Oncology, Department of Obstetrics and Gynecology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abir","family":"Tabbarah","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chirine","family":"Khaled","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rami","family":"Mahfouz","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reem","family":"Abdallah","sequence":"additional","affiliation":[{"name":"Division of Gynecologic Oncology, Department of Obstetrics and Gynecology American University of Beirut Medical Center  Beirut Lebanon"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,8,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clon.2010.02.010"},{"key":"e_1_2_6_3_1","first-page":"132","article-title":"Ewing sarcoma and primitive neuroectodermal tumors in adults: Single center experience in The Netherlands","volume":"65","author":"Smorenburg CH","year":"2007","journal-title":"Neth J Med"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000167056.13614.62"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.25840"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-199907000-00012"},{"key":"e_1_2_6_7_1","first-page":"101","article-title":"Primary extraskeletal Ewing sarcoma originating from chest wall in a child","volume":"7","author":"Avcu S","year":"2010","journal-title":"Eur J Gen Med"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/jjco\/hyq080"},{"key":"e_1_2_6_9_1","first-page":"369","article-title":"Ewing's sarcoma in the spinal nerve root: A case report and review of the literature","volume":"209","author":"Isefuku S","year":"2006","journal-title":"J Exp Med"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197507)36:1<240::AID-CNCR2820360127>3.0.CO;2-H"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0031-3955(05)70541-1"},{"key":"e_1_2_6_12_1","unstructured":"National Comprehensive Cancer Network (NCCN). 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