Epithelioid Sarcoma: A Hand Surgeon's Dilemma

Proximal and classic-type epithelioid sarcoma (ES) comprise less than 1% of adult soft tissue sarcomas. ES shows a male predominance and most commonly involves the forearms and fingers, with a median age at presentation of 25 to 33 years. The 5-year local recurrence rate is 35%, and the metastatic rate is 40% to 50%; the 5- and 10-year survival rates are 60% to 85% and 42% to 62%, respectively (all ES). We present the case of a 27-year-old right-handed man with classic-type ES of the dominant hand. Case notes were reviewed following consent from the patient, a diesel fitter from a cattle station in the Northern Territory, Australia. Key moments in his diagnostic and management journey were summarized in this Case Report. This young gentleman went on to develop locoregional and distant metastases, for which he has undergone multiple resections. At the time of writing, he is being fitted with a forearm prosthesis and undergoing rehabilitation and surveillance. Living remotely limits access to timely surgical care. Further research into adjuvant therapies is required to determine a clear approach to treatment. Clinicians must think "sarcoma" in an otherwise benign lump presentation. Close and regular follow-up is imperative.