Epithelioid sarcoma: A retrospective review from a single centre tertiary orthopaedic sarcoma unit

Epithelioid sarcoma often presents as an innocuous lesion, commonly affecting the extremities. Lymph node involvement is common, with patients typically presenting with multiple recurrences of locoregional disease. The aim of our study is to assess the presentation, investigation and management of epithelioid sarcoma within a single tertiary centre.

Retrospective review of our prospectively maintained oncology database identified 39 patients referred and treated for epithelioid soft tissue sarcoma between 1989 and 2025. Median follow-up was 88 months. The primary outcome of interest was overall survival. Secondary outcomes included development of locoregional disease and distant metastases post-primary treatment.

Overall survival at two, five and ten years was 78.3%, 68.4% and 59.9% respectively. Median survival was 139 months (3-178 months). At presentation, 12 patients (31%) had metastases, the majority of which were lymph node and identified on radionuclide imaging with PET CT. Surgical excision was the primary treatment with curative intent in 33 patients. Locoregional recurrence within the ipsilateral limb occurred in 27% patients at a median time of 56 months post-primary treatment, with multiple episodes in over half. Distant metastases developed in 21% patients of which all were in the chest.

Epithelioid sarcoma is a slow growing yet aggressive tumour. Primary excision with clear margins remains the best chance of cure. Early identification of lymph node metastases may better guide prognosis and treatment. Latent, but relentless episodes of locoregional disease post-primary treatment are frequently observed.