Highlights • Myoepithelial carcinoma of the vulva and epithelioid sarcoma has overlapping histological, immunohistochemical and genetic features.• Similarities between two diagnoses in aggressive vulvar cancer should be noted as a… Click to show full abstract
Highlights • Myoepithelial carcinoma of the vulva and epithelioid sarcoma has overlapping histological, immunohistochemical and genetic features.• Similarities between two diagnoses in aggressive vulvar cancer should be noted as a sarcoma-based chemotherapy regimen should be considered.• We recommend immediate surgical resection with bilateral superficial and deep inguinal lymph node dissections in these aggressive cancers.• Identification of SMARCB1-deficiency trials of targeted therapies such as EZH2 inhibitors which show promise in halting further progression.
               
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