A unique case of epithelioid sarcoma involving the mandibular alveolar mucosa.

Epithelioid sarcoma (ES) is an infrequent, malignant, mesenchymal, soft-tissue neoplasm of uncertain histogenesis characterized by epithelioid cytomorphology. The lack of SMARCB1/INI1 (Integrase interactor 1) expression typifies this pathology. It usually presents as a painless, indolent, slowly enlarging lesion or rarely as an aggressive growth. Epithelioid sarcoma affects mostly pediatric patients or young adults. It recurs quite frequently and possesses a high degree of metastatic potential. Two predominant histomorphologic patterns include classic/conventional type and proximal types. Epithelioid sarcoma immunohistochemically shows vimentin, cytokeratin, epithelial membrane antigen, CD34 positivity, and negative staining with S100 and desmin. Management is generally multimodal, including wide surgical resection, radiotherapy, and chemotherapy. To date, to our knowledge, only 11 instances of head and neck ES have been reported in the literature. Our case deals with the diagnosis of intraoral ES in a 55-year-old female patient, probably the first one to involve the mandibular mucosa, based on relevant clinical-radiologic-pathologic-immunohistochemical findings.