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 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. | * 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. | ||
* Epithelioid sarcoma most commonly strikes young adults, yet no age group is immune. The disease has a tendency to develop local recurrences and [[metastasis]] thereafter to regional lymph nodes, lung, bone, brain, and other locations, including the scalp.<ref name=pmid19415960/> Generally speaking, epithelioid sarcoma has a high rate of relapse after initial treatment and tends to recur locally (at or near the original tumor site). Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases).<ref name=pmid24046109>{{cite journal |last1=Levy |first1=Antonin |last2=Le Péchoux |first2=Cécile |last3=Terrier |first3=Philippe |last4=Bouaita |first4=Ryan |last5=Domont |first5=Julien |last6=Mir |first6=Olivier |last7=Coppola |first7=Sarah |last8=Honoré |first8=Charles |last9=Le Cesne |first9=Axel |last10=Bonvalot |first10=Sylvie |title=Epithelioid Sarcoma: Need for a Multimodal Approach to Maximize the Chances of Curative Conservative Treatment |journal=Annals of Surgical Oncology |volume=21 |issue=1 |pages=269–76 |year=2014 |pmid=24046109 |doi=10.1245/s10434-013-3247-4 }}</ref> These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. The overall five-year survival rate for epithelioid sarcoma is anywhere from 25 to 78%.<ref name=pmid24046109/> Importantly, the 10-year and 15-year survival rate drops off significantly.<ref name=LevDina>{{cite web |last1=Lev |first1=Dina |title=Epigenetic reprogramming of epitheliold sarcoma: a role for INI1-HDAC crosstalk |url=https://ofur.rice.edu/content.aspx?id=4294967475 }}{{dead link|date=October 2015}}</ref><ref name=pmid16353216>{{cite journal |last1=Casanova |first1=Michela |last2=Ferrari |first2=Andrea |last3=Collini |first3=Paola |last4=Bisogno |first4=Gianni |last5=Alaggio |first5=Rita |last6=Cecchetto |first6=Giovanni |last7=Gronchi |first7=Alessandro |last8=Meazza |first8=Cristina |last9=Garaventa |first9=Alberto |last10=Di Cataldo |first10=Andrea |last11=Carli |first11=Modesto |title=Epithelioid sarcoma in children and adolescents |journal=Cancer |volume=106 |issue=3 |pages=708–17 |year=2006 |pmid=16353216 |doi=10.1002/cncr.21630 }}</ref> Associated with a more positive outcome are younger age, female vs. male sex, distal vs. proximal location, smaller tumor size, and negative margins upon tumor resection.<ref name=pmid5476785/><ref>{{cite journal |last1=Jawad |first1=Muhammad Umar |last2=Extein |first2=Jason |last3=Min |first3=Elijah S. |last4=Scully |first4=Sean P. |title=Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database |journal=Clinical Orthopaedics and Related Research |volume=467 |issue=11 |pages=2939–48 |year=2009 |pmid=19224301 |pmc=2758965 |doi=10.1007/s11999-009-0749-2 }}</ref><ref name=pmid16353216/> | |||
==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 22:05, 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.
- Epithelioid sarcoma most commonly strikes young adults, yet no age group is immune. The disease has a tendency to develop local recurrences and metastasis thereafter to regional lymph nodes, lung, bone, brain, and other locations, including the scalp.[3] Generally speaking, epithelioid sarcoma has a high rate of relapse after initial treatment and tends to recur locally (at or near the original tumor site). Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases).[4] These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. The overall five-year survival rate for epithelioid sarcoma is anywhere from 25 to 78%.[4] Importantly, the 10-year and 15-year survival rate drops off significantly.[5][6] Associated with a more positive outcome are younger age, female vs. male sex, distal vs. proximal location, smaller tumor size, and negative margins upon tumor resection.[1][7][6]
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.[8]
References
- ↑ 1.0 1.1 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 3.2 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.
- ↑ 4.0 4.1 Levy, Antonin; Le Péchoux, Cécile; Terrier, Philippe; Bouaita, Ryan; Domont, Julien; Mir, Olivier; Coppola, Sarah; Honoré, Charles; Le Cesne, Axel; Bonvalot, Sylvie (2014). "Epithelioid Sarcoma: Need for a Multimodal Approach to Maximize the Chances of Curative Conservative Treatment". Annals of Surgical Oncology. 21 (1): 269–76. doi:10.1245/s10434-013-3247-4. PMID 24046109.
- ↑ Lev, Dina. "Epigenetic reprogramming of epitheliold sarcoma: a role for INI1-HDAC crosstalk".[dead link]
- ↑ 6.0 6.1 Casanova, Michela; Ferrari, Andrea; Collini, Paola; Bisogno, Gianni; Alaggio, Rita; Cecchetto, Giovanni; Gronchi, Alessandro; Meazza, Cristina; Garaventa, Alberto; Di Cataldo, Andrea; Carli, Modesto (2006). "Epithelioid sarcoma in children and adolescents". Cancer. 106 (3): 708–17. doi:10.1002/cncr.21630. PMID 16353216.
- ↑ Jawad, Muhammad Umar; Extein, Jason; Min, Elijah S.; Scully, Sean P. (2009). "Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database". Clinical Orthopaedics and Related Research. 467 (11): 2939–48. doi:10.1007/s11999-009-0749-2. PMC 2758965. PMID 19224301.
- ↑ 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.