Primary Ewing Sarcoma of Vulva: A Case Report and a Review of Literature

Kakoti, Lopa Mudra; Sharma, Jagannath Dev; Kataki, Amal Chandra; Barmon, Debabrata

doi:10.1007/s40944-017-0103-7

Cited by 4 publications

(1 citation statement)

References 8 publications

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“…Molecular testing provides a more accurate diagnosis for ES. For instance, chromosomal translocation t(11;22)(q24;q12) (EWS‐FLI1 gene rearrangement), which was demonstrated in our patient, is highly specific for ES/PNET and is detected in more than 90% of tumors 18 …”

Section: Discussionmentioning

confidence: 55%

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

Housheimi

Khalil

Khalifeh

et al. 2020

J of Obstet and Gynaecol

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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.

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Section: Discussionmentioning

confidence: 55%

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

Housheimi

Khalil

Khalifeh

et al. 2020

J of Obstet and Gynaecol

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Primitive Neuroectodermal Tumor of the Vulva: A Rare Case Report with Review of Literature

Majumdar

Das

Barik

et al. 2023

Indian J Gynecol Oncolog

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Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

Xu¹,

Xing²,

Chen³

et al. 2019

WJCC

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BACKGROUNDPrimitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva.CASE SUMMARYCase 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm. The patient underwent radical resection of the tumor. After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide (IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression. Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm. The tumor was partially resected by surgery. The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy, and the patient died at home six months after surgery.CONCLUSIONPNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis.

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Primary Ewing Sarcoma of Vulva: A Case Report and a Review of Literature

Cited by 4 publications

References 8 publications

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

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

Primitive Neuroectodermal Tumor of the Vulva: A Rare Case Report with Review of Literature

Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

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