Epithelioid sarcoma pathophysiology: Difference between revisions
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* Epithelioid sarcoma is a rare [[soft tissue sarcoma]] arising from mesenchymal tissue and characterized by [[Epithelioid cell|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.<ref name=pmid5476785>{{cite journal |last1=Enzinger |first1=F. M. |title=Epithelioid sarcoma.A sarcoma simulating a granuloma or a carcinoma |journal=Cancer |volume=26 |issue=5 |pages=1029–41 |year=1970 |pmid=5476785 |doi=10.1002/1097-0142(197011)26:5<1029::AID-CNCR2820260510>3.0.CO;2-R }}</ref> 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.<ref>{{cite journal |last1=Guillou |first1=L |last2=Wadden |first2=C |last3=Coindre |first3=JM |last4=Krausz |first4=T |last5=Fletcher |first5=CD |title='Proximal-type' epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series |journal=The American Journal of Surgical Pathology |volume=21 |issue=2 |pages=130–46 |year=1997 |pmid=9042279 |doi=10.1097/00000478-199702000-00002}}</ref> Rare cases have been reported in the pelvis, vulva, penis, and spine. | |||
==Genetics== | ==Genetics== | ||
''SMARCB1'' gene is involved in the pathogenesis of epithelioid sarcoma. | ''SMARCB1'' gene is involved in the pathogenesis of epithelioid sarcoma. | ||
Revision as of 22:04, 9 February 2016
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Epithelioid sarcoma Microchapters |
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Epithelioid sarcoma pathophysiology On the Web |
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Risk calculators and risk factors for Epithelioid sarcoma pathophysiology |
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.