Primary vulvar Ewing sarcoma/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature

Alaa El Housheimi; Ali Khalil; Dalia Khalifeh; Ghina Berjawi; Muhieddine Seoud; Abir Tabbarah; Chirine Khaled; Rami Mahfouz; Reem Abdallah

doi:10.1111/jog.14399

Primary vulvar Ewing sarcoma/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature

J Obstet Gynaecol Res. 2020 Oct;46(10):2185-2192. doi: 10.1111/jog.14399. Epub 2020 Aug 5.

Authors

Alaa El Housheimi¹, Ali Khalil², Dalia Khalifeh¹, Ghina Berjawi³, Muhieddine Seoud², Abir Tabbarah⁴, Chirine Khaled⁴, Rami Mahfouz⁴, Reem Abdallah²

Affiliations

¹ Department of Obstetrics and Gynecology, American University of Beirut Medical Center, Beirut, Lebanon.
² Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, American University of Beirut Medical Center, Beirut, Lebanon.
³ Department of Diagnostic Radiology, American University of Beirut Medical Center, Beirut, Lebanon.
⁴ Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

PMID: 32761741
DOI: 10.1111/jog.14399

Abstract

Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNET) are soft tissue tumors that generally affect the bones. Extraosseous ES/pPNET has been rarely reported. Our patient presented with a 6 × 4 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-cm lesion in the right vulvar region with enlarged bilateral inguinal and right iliac lymph nodes. Fluorescence in situ hybridization test for translocation t(11;22)(q24;q12) was positive, confirming the diagnosis. The patient received three cycles 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 cm with free margins. She received four additional cycles of adjuvant chemotherapy and 30 sessions radiotherapy. She is currently disease free after 37 months. No ES/pPNET cases with pelvic lymph nodes metastasis were ever reported.

Keywords: extraskeletal Ewing sarcoma; fluorescence in situ hybridization test; radical local excision.

Publication types

Case Reports
Review

MeSH terms

Chemotherapy, Adjuvant
Female
Humans
In Situ Hybridization, Fluorescence
Lymph Nodes
Neuroectodermal Tumors, Primitive, Peripheral* / diagnosis
Neuroectodermal Tumors, Primitive, Peripheral* / therapy
Sarcoma, Ewing* / diagnosis
Sarcoma, Ewing* / therapy