2025 Volume 42 Issue 2 Pages 139-145
We report a case of myoepithelioma-like tumor of the vulvar region （MELTVR）. A woman in her 60s had a 2cm subcutaneous tumor on the perineum. Histologically, the tumor extended from the subcutaneous tissue to the dermis. The tumor consisted of the proliferation of spindle-shaped and epithelioid cells with abundant myxoid stroma. The histology resembled that of soft tissue myoepithelioma;however, immunohistochemically, AE1/AE3 showed very focal and weak positivity, CAM5.2, S-100, and GFAP were negative, and EWSR1 gene rearrangement was absent. Additional immunohistochemical evaluation was performed, and the diagnosis of MELTVR was confirmed based on the expression of Estrogen receptor and complete loss of SMARCB1 expression. The infiltrative proliferation into the dermis was a characteristic feature of this case. In addition, we report on MELTVR from the perspective of tumors that show loss of SMARCB1 expression, along with a discussion of the literature.
