Difference between revisions of "Epithelioid sarcoma"
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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> | 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> | *[[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. | *[[Vimentin]] +ve. | ||
*Various keratins +ve. | *Various keratins +ve. | ||
**Keratin 8, Keratin 19 +ve. | **Keratin 8, Keratin 19 +ve. | ||
| Line 88: | Line 88: | ||
*S100 -ve (r/o melanoma). | *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> | *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> | *[[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== | ==See also== | ||
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:
- Carcinoma.
- Rheumatoid nodule.
- Granuloma annulare.
- Malignant rhabdoid tumour.
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
- ↑ 1.0 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 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.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601607
- ↑ 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.
- ↑ 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.
- ↑ 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.