Difference between revisions of "Epithelioid sarcoma"

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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]], [[malignant rhabdoid tumour]]
| Stains    =
| IHC        = INI1 -ve, CK7 +ve, vimentin +ve, S-100 -ve, CD34 +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  = CA-125 elevated
| 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]].
*[[Malignant rhabdoid tumour]].
===Images===
<gallery>
Image:Epithelioid sarcoma - low mag.jpg | ES - low mag. (WC/Nephron)
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 - very high mag.jpg | ES - very high mag. (WC/Nephron)
Image:Epithelioid sarcoma - cropped - very high mag.jpg | ES - cropped - very high mag. (WC/Nephron)
Image:Epithelioid sarcoma -2- very high mag.jpg | ES - very high mag. (WC/Nephron)
Image:Epithelioid sarcoma - smarcb1 - intermed mag.jpg | ES - SMARCB1/INI1 - intermed. 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]]

Latest revision as of 22:08, 3 August 2016

Epithelioid sarcoma
Diagnosis in short

Epithelioid sarcoma. H&E stain.
LM epithelioid morphology and spindle morphology (predominant pattern dependent on location), +/-prominent nucleolus, zonal necrosis with an irregular border
Subtypes proximal type, distal type
LM DDx carcinoma, rheumatoid nodule, granuloma annulare, malignant rhabdoid tumour
IHC INI1 -ve, CK7 +ve, vimentin +ve, S-100 -ve, CD34 +ve
Site soft tissue - usu. extremities
Clinical history usu. young adults
Symptoms mass lesion
Prevalence rare
Blood work CA-125 elevated

Epithelioid sarcoma is a rare malignant 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.[1]

Subclassification:[2]

  • Proximal type:
    • More aggressive.
  • Distal type:

Microscopic

Features:[3]

  • 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:[2]

  • Proximal-type (proximal location):
    • More epithelioid.
  • Distal-type (distal location):
    • More spindled.
    • Granuloma-like pattern.

DDx:

Images

  • ES - low mag. (WC/Nephron)

  • ES - intermed. mag. (WC/Nephron)

  • ES - high mag. (WC/Nephron)

  • ES - very high mag. (WC/Nephron)

  • ES - cropped - very high mag. (WC/Nephron)

  • ES - very high mag. (WC/Nephron)

  • ES - SMARCB1/INI1 - intermed. mag. (WC/Nephron)

  • ES - SMARCB1/INI1 - high mag. (WC/Nephron)

www:

IHC

Features:[4]

  • INI1 (SMARCB1[5]) -ve.[6]
  • Vimentin +ve.
  • Various keratins +ve.
    • Keratin 8, Keratin 19 +ve.
    • 34betaE12 +ve/-ve.
  • CD34 +ve.
    • Malignant rhabdoid tumour -ve.

Others:

See also

References

  1. ↑ 1.0 1.1 Hoshino, M.; Kawashima, H.; Ogose, A.; Kudo, N.; Ariizumi, T.; Hotta, T.; Umezu, H.; Hatano, H. et al. (Mar 2010). "Serum CA 125 expression as a tumor marker for diagnosis and monitoring the clinical course of epithelioid sarcoma.". J Cancer Res Clin Oncol 136 (3): 457-64. doi:10.1007/s00432-009-0678-1. PMID 19756736.
  2. ↑ 2.0 2.1 Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD (February 1997). ""Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series". Am. J. Surg. Pathol. 21 (2): 130–46. PMID 9042279.
  3. ↑ The International Agency for Research on Cancer (Editors: Fletcher, C.D.M.; Unni, K. Krishnan; Mertens, F.) (2006). Pathology and Genetics of Tumours of Soft Tissue and Bone (IARC WHO Classification of Tumours) (3rd ed.). World Health Organization. pp. 205. ISBN 978-9283224136.
  4. ↑ Miettinen M, Fanburg-Smith JC, Virolainen M, Shmookler BM, Fetsch JF (August 1999). "Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis". Hum. Pathol. 30 (8): 934–42. PMID 10452506.
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601607
  6. ↑ Mentzel T (March 2010). "[Epithelioid sarcoma: morphologic variants and differential diagnosis]" (in German). Pathologe 31 (2): 135–41. doi:10.1007/s00292-009-1250-0. PMID 19997734.
  7. ↑ Humble, SD.; Prieto, VG.; Horenstein, MG. (Apr 2003). "Cytokeratin 7 and 20 expression in epithelioid sarcoma.". J Cutan Pathol 30 (4): 242-6. PMID 12680954.
  8. ↑ Lee, HI.; Kang, KH.; Cho, YM.; Lee, OJ.; Ro, JY. (Jun 2006). "Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity.". Arch Pathol Lab Med 130 (6): 871-4. doi:10.1043/1543-2165(2006)130[871:PESWES]2.0.CO;2. PMID 16740043.
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