Epithelioid sarcoma

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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.


  • Rare.
  • Adolescents, young adults.
  • Serum CA-125 may be useful for following clinically.[1]


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



  • 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.


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






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


See also


  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.