Epitheloid sarcoma is a rare soft tissue sarcoma classified under tumor of uncertain histogenesis. Incidence being 0.4/million/year, it is divided into proximal variant-where in the proximal parts of the body are more involved and distal variant which is commoner and less aggressive of the two predominantly involving distal parts of upper limb followed by distal lower extremities and proximal upper limb. It has a male preponderance (1.8:1), although females have better prognosis comparatively, also it has a tendency of lymphatic spread with 13% metastasis. Recurrence of 77% is reported. Definitive diagnosis is made only on tissue biopsy. Most common variant is pseudo granulomatous type. This case report is based on a case which presented with a single right anterior shoulder swelling and was found to be of epitheloid sarcoma on biopsy. A 60 years old female patient came to OPD of D. Y. Patil Medical College, Pune, with complaints of swelling over her right shoulder since 1 month, initially small and gradually increasing in size of swelling. No history of pain and loss of appetite or weight. On examination, a 4 3 cms firm to hard swelling present over anterior part of right shoulder. It was observed to be non-tender, well defined and mobile. Patient has been investigated with x-ray, FNAC, and ultrasound of the swelling. FNAC was significant other giant cell lesion of soft tissue hence taken up for excision biopsy which showed epitheloid sarcoma on histopathology with faint focal positivity for pancytokeratin and EMA and negative for LCA, CK-3 and S-100.
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