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
- Histologically, epithelioid sarcoma forms nodules with central necrosis surrounded by bland, polygonal cells with eosinophilic cytoplasm and peripheral spindling.[3] Epithelioid sarcomas typically express vimentin, cytokeratins, epithelial membrane antigen, and CD34, whereas they are usually negative for S100, desmin, and FLI-1.[3] They typically stain positive for CA125.[4]
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.
- ↑ 3.0 3.1 Armah, Henry B. Armah; Parwani, Anil V. (2009). "Epithelioid sarcoma". Archives of Pathology & Laboratory Medicine. 133 (5): 814–9. doi:10.1043/1543-2165-133.5.814 (inactive October 6, 2015). PMID 19415960.
- ↑ Kato, Hiroshi; Hatori, Masahito; Kokubun, Shoichi; Watanabe, Mika; Smith, Richard A; Hotta, Tetsuo; Ogose, Akira; Morita, Tetsuro; Murakami, Takashi; Aiba, Setsuya (2004). "CA125 expression in epithelioid sarcoma". Japanese Journal of Clinical Oncology. 34 (3): 149–54. doi:10.1093/jjco/hyh027. PMID 15078911.