Epithelioid sarcoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Pathogenesis
- Epithelioid sarcoma is the second most common soft tissue sarcoma in hand. Epithelioid sarcoma is also sixth most common soft tissue sarcoma in upper extremity.
- Primary site of epithelioid sarcoma is upper distal extremities. Other rare sites of epithelioid sarcoma are:
- Vulva
- Penis
- Spine
- Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioid-like features.
- Epithelioid sarcoma accounts for less than 1% of all soft tissue sarcomas. It was first clearly characterized by F.M. Enzinger in 1970.[1] It commonly presents itself in the distal limbs (fingers, hands, forearms, or feet) of young adults as a small, soft mass or a series of bumps. A proximal version has also been described, frequently occurring in the upper extremities.[2] Rare cases have been reported in the pelvis, vulva, penis, and spine.
Genetics
SMARCB1 gene is involved in the pathogenesis of epithelioid sarcoma.
Associated Conditions
Gross Pathology
Microscopic Pathology
References
- ↑ Enzinger, F. M. (1970). "Epithelioid sarcoma.A sarcoma simulating a granuloma or a carcinoma". Cancer. 26 (5): 1029–41. doi:10.1002/1097-0142(197011)26:5<1029::AID-CNCR2820260510>3.0.CO;2-R. PMID 5476785.
- ↑ Guillou, L; Wadden, C; Coindre, JM; Krausz, T; Fletcher, CD (1997). "'Proximal-type' epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series". The American Journal of Surgical Pathology. 21 (2): 130–46. doi:10.1097/00000478-199702000-00002. PMID 9042279.