Difference between revisions of "Epithelioid sarcoma"

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Epithelioid sarcoma (view source)

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#redirect [[Soft_tissue_lesions#Epithelioid_sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Epithelioid_sarcoma_-_high_mag.jpg
| Width      =
| Caption    = Epithelioid sarcoma. [[H&E stain]].
| Micro      = epithelioid morphology and spindle morphology (predominant pattern dependent on location), +/-prominent nucleolus, zonal necrosis with an irregular border
| Subtypes  = proximal type, distal type
| LMDDx      = carcinoma, [[rheumatoid nodule]], [[granuloma annulare]]
| Stains    =
| IHC        = INI1 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - usu. extremities
| Assdx      =
| Syndromes  =
| Clinicalhx = usu. young adults
| Signs      =
| Symptoms  = mass lesion
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Epithelioid sarcoma''' is a rare malignant [[soft tissue lesions|soft tissue lesion]].


:Sarcomas with an epithelioid morphology are covered in ''[[epithelioid sarcomas]]''.
===General===
*Rare.
*Adolescents, young adults.
*Serum CA-125 may be useful for following clinically.<ref name=pmid19756736>{{Cite journal  | last1 = Hoshino | first1 = M. | last2 = Kawashima | first2 = H. | last3 = Ogose | first3 = A. | last4 = Kudo | first4 = N. | last5 = Ariizumi | first5 = T. | last6 = Hotta | first6 = T. | last7 = Umezu | first7 = H. | last8 = Hatano | first8 = H. | last9 = Morita | first9 = T. | title = Serum CA 125 expression as a tumor marker for diagnosis and monitoring the clinical course of epithelioid sarcoma. | journal = J Cancer Res Clin Oncol | volume = 136 | issue = 3 | pages = 457-64 | month = Mar | year = 2010 | doi = 10.1007/s00432-009-0678-1 | PMID = 19756736 }}</ref>
Subclassification:<ref name=pmid9042279>{{cite journal |author=Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD |title="Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series |journal=Am. J. Surg. Pathol. |volume=21 |issue=2 |pages=130–46 |year=1997 |month=February |pmid=9042279 |doi= |url=}}</ref>
*Proximal type:
**More aggressive.
*Distal type:
===Microscopic===
Features:<ref>{{Ref WHOSTAB|205}}</ref>
*Epithelioid morphology and spindle morphology - which predominates is dependent on location (see subclassification).
*+/-Prominent nucleolus - '''distinctive feature'''.
*Zonal necrosis with irregular border.
**Descriptors: "Garland necrosis", necrosis with "scalloped border" = necrotic regions with irregular border.
Subclassification:<ref name=pmid9042279>{{cite journal |author=Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD |title="Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series |journal=Am. J. Surg. Pathol. |volume=21 |issue=2 |pages=130–46 |year=1997 |month=February |pmid=9042279 |doi= |url=}}</ref>
*Proximal-type (proximal location):
**More epithelioid.
*Distal-type (distal location):
**More spindled.
**Granuloma-like pattern.
DDx:
*Carcinoma.
*[[Rheumatoid nodule]].
*[[Granuloma annulare]].
====Images====
<gallery>
Image:Epithelioid_sarcoma_-_intermed_mag.jpg | ES - intermed. mag. (WC/Nephron)
Image:Epithelioid_sarcoma_-_high_mag.jpg | ES - high mag. (WC/Nephron)
Image:Epithelioid_sarcoma_-_smarcb1_-_high_mag.jpg | ES - SMARCB1/INI1 - high mag. (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case594.html ES - several images (upmc.edu)].
===IHC===
Features:<ref name=pmid10452506>{{cite journal |author=Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF |title=Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis |journal=Hum. Pathol. |volume=30 |issue=8 |pages=934–42 |year=1999 |month=August |pmid=10452506 |doi= |url=}}</ref>
*INI1 (SMARCB1<ref>{{OMIM|601607}}</ref>) -ve.<ref name=pmid19997734>{{cite journal |author=Mentzel T |title=[Epithelioid sarcoma: morphologic variants and differential diagnosis] |language=German |journal=Pathologe |volume=31 |issue=2 |pages=135–41 |year=2010 |month=March |pmid=19997734 |doi=10.1007/s00292-009-1250-0 |url=}}</ref>
*Vimentin +ve.
*Various keratins +ve.
**Keratin 8, Keratin 19 +ve.
**34betaE12 +ve/-ve.
*CD34 +ve.
**Malignant rhabdoid tumour -ve.
Others:
*S100 -ve (r/o melanoma).
*CK7 +ve / CK20 -ve.<ref name=pmid12680954>{{Cite journal  | last1 = Humble | first1 = SD. | last2 = Prieto | first2 = VG. | last3 = Horenstein | first3 = MG. | title = Cytokeratin 7 and 20 expression in epithelioid sarcoma. | journal = J Cutan Pathol | volume = 30 | issue = 4 | pages = 242-6 | month = Apr | year = 2003 | doi =  | PMID = 12680954 }}</ref>
*CA-125 +ve.<ref name=pmid19756736/><ref name=pmid16740043>{{Cite journal  | last1 = Lee | first1 = HI. | last2 = Kang | first2 = KH. | last3 = Cho | first3 = YM. | last4 = Lee | first4 = OJ. | last5 = Ro | first5 = JY. | title = Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity. | journal = Arch Pathol Lab Med | volume = 130 | issue = 6 | pages = 871-4 | month = Jun | year = 2006 | doi = 10.1043/1543-2165(2006)130[871:PESWES]2.0.CO;2 | PMID = 16740043 }}</ref>
==See also==
*[[Soft tissue lesions]].
*[[Epithelioid sarcomas]].
==References==
{{Reflist|2}}
[[Category:Soft tissue lesions]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
Michael
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