Tehran University Medical Journal

Background: Epithelioid sarcoma is a malignant soft tissue tumor of uncertain histogenesis, categorized as a morphologically distinct neoplasm that characteristically affects the distal parts of the extremities in young adults. In fact, epithelioid sarcoma is the most common soft tissue sarcoma in the hand and wrist.

Case report: This 32 year-old male presented with an inguinal swelling, for which he underwent surgery with the initial intent of inguinal hernia repair. With this uncommon manifestation and site, among the differential diagnosis were abscess and GI malignancies. However, after a supplementary evaluation that included biopsy of the ulcer margin, the diagnosis focused on undifferentiated high-grade epithelial tumor, highly suggestive of epithelioid sarcoma. Immunohistochemical studies revealed CD34 and cytokeratin positivity, which confirmed this diagnosis.

Conclusion: Due to the malignant nature, invasive behavior and high recurrence rate of this tumor, as well as its unknown response to chemotherapy and radiation, extensive resection and hepatectomy are recommended for treatment.