Primary vulvar Ewing sarcoma/peripheral primitive neuroectodermal tumor with pelvic lymph nodes metastasis: A case report and review of literature
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Blackwell Publishing
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. © 2020 Japan Society of Obstetrics and Gynecology
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Keywords
Extraskeletal ewing sarcoma, Fluorescence in situ hybridization test, Radical local excision, Cd99 antigen, Cyclophosphamide, Doxorubicin, Etoposide, Ifosfamide, Vimentin, Vincristine, Adult, Article, Cancer radiotherapy, Cancer staging, Case report, Chromosome translocation, Clinical article, Clinical examination, Disease free interval, Drug response, Ewing sarcoma, External beam radiotherapy, Female, Fluorescence in situ hybridization, Human, Human tissue, Immunohistochemistry, Inguinal lymph node, Labia majora, Local excision, Lymph node metastasis, Lymphadenopathy, Minimal residual disease, Multiple cycle treatment, Neoadjuvant chemotherapy, Neuroectoderm tumor, Neutropenia, Nuclear magnetic resonance imaging, Pelvis lymph node, Positron emission tomography-computed tomography, Primary tumor, Thrombocytopenia, Treatment duration, Vulva cancer, Vulvodynia, Vulvovaginal discomfort