No age group is exempt, but this tumor occurs most frequently in patients in their 20's and 30's. Therefore, given a patient in such an age group who presents with a tumor resembling a squamous cell carcinoma of skin, the possibility of epithelioid sarcoma should be raised. Although listed as a soft tissue tumor, this type of sarcoma can occur in the dermis or subcutis. These are very rare on the face or trunk, but almost any other site, including buttocks, genitalia, and scalp, may be involved. Most of the cases occur on the extremities, particularly the upper extremity.
Recurrences are very common.
Metastasizes to lymph nodes or by blood stream. May metastasize to skin or produce satellites. Cutaneous tumors are prone to ulcerate. The differential diagnosis includes many cytologically atypical and cytologically malignant tumors of skin. In addition, the d ifferential diagnosis includes necrobiotic palisading granulomatous diseases.