epithelioid sarcoma

Epithelioid sarcoma.

Epithelioid sarcoma is a rare mesenchymal soft tissue tumour with an epithelioid pattern.

Epithelioid sarcoma is a rare mesenchymal tumor of uncertain histogenesis which displays multilineage differentiation.

These tumors are so named because their histologic appearance is very similar to that of epithelioid sarcoma, a more malignant tumor with which they are commonly mistaken.

Soft tissue sarcomas that more commonly spread to lymph nodes include synovial cell sarcomas, epithelioid sarcomas, and rhabdomyosarcomas.

Chbani L, Guillou L, Terrier P, et al.: Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group.

Casanova M, Ferrari A, Collini P, et al.: Epithelioid sarcoma in children and adolescents: a report from the Italian Soft Tissue Sarcoma Committee.

Cancerous bone or soft tissue tumors (e.g. osteosarcoma, osteochondroma, fibrosarcoma, epithelioid sarcoma, Ewing's sarcoma, synovial sarcoma, sacrococcygeal teratoma, liposarcoma)

Hornick JL, Dal Cin P, Fletcher CD: Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma.

In a review of 30 pediatric patients with epithelioid sarcoma, the median age at presentation was 12 years, responses to chemotherapy were reported in 40% of patients using sarcoma-based regimens, and 60% of patients were alive at 5 years following initial diagnosis.