Epithelioid Sarcoma

Epithelioid sarcoma was first described by Enzinger (1970 Epithelioid sarcoma. A sarcoma simulating a granuloma or a carcinoma.Cancer 26:1029-1041).

This rare malignant tumour occurs in adolescents and young adults.

Epithelioid sarcoma is usually located on the distal extremities (hands, fingers and forearm). This tumour rarely occurs on the pelvis, perineum and vulva.

The tumour spreads along fascial and neurovascular structures with formation of satellite nodules.

Multiple recurrences are known to occur in this tumour.

Metastasis to the scalp, lymphnode and lungs occur frequently.

Adequate treatment requires early radical excision or amputation if the tumour is located in the fingers or toes. Regional lymphnode dissection should be included as lymphnode metastasis is common in epithelioid sarcoma. Surgical excision is combined with radiotherapy.

http://www.histopathology-india.net/EpithelioidSarcoma.htm