A case report of a paratesticular SMARCB1 deficient myoepithelioma like tumor in an unusual location

Myoepithelioma-like tumor of the vulvar region is a relatively recently described mesenchymal neoplasm with myxoid features that typically arises in the subcutaneous tissue of the genital region of adult women. It consistently exhibits loss of nuclear expression of SMARCB1/INI1 and lacks gene rearrangements characteristic of myoepithelial neoplasms of soft tissue. These tumors are well circumscribed and typically don’t display markedly atypical histologic features. Cases with analogous morphology have been sporadically reported in the genital region of male patients. Herein, we present a rare case of a SMARCB1/INI1-deficient myoepithelioma-like tumor occurring in the paratesticular region of a 47-year-old man. To our knowledge, only three cases of this entity have been previously reported in this anatomical location.