Epithelioid sarcoma

Case 242 May 2011  (P.McKee) 

Pallisaded granuloma-like tumour with large area of necrosis. 

Case 691  7.2.13 (H. Diwan)  F 43 gluteal mass (proximal type) 

Irregular nodules of small blue-staining cells in the dermis.  Central necrosis.  Fibrous septae.  HP shows polygonal cells with eos cytoplasm and relatively uniform nuclei. Pancytokeratin and CD34+ve.  Rare soft tissue sarcoma presents with deep mass.  Aggressive.  Described 1997 – more common form of ES described 1970 is peripheral. 

Case 1283   25.5.15 (IH Chaudhry)  M 45 abd wall mass (proximal type) 

Sheets of epithelioid cells showing infiltrative pattern.  Vascular component made epithelioid angiosarcoma a possibility but IH confirmed D.  Proximal type shows more cellular pleomorphism and necrosis, and is more aggressive than the distal type. 

Case 1468 9.2.16  (U. Sundram)  M 38 numerous nodules incl. scalp 

Very difficult diagnosis.  Storiform growth pattern.  Fusiform cells dissecting collagen and infiltrating fat.  Moderate atypia only.  EMA++; CD34+; PanK faint +; CK5/6 –ve.  Example of advanced metastasising disease (lung mets present). 

Case 2121  26.7.18  (R. Perret)  M 19 polypoid mass perineum  (proximal type) 

Apparently typical clinical and histological presentation.  ES are highly aggressive tumours which frequently metastasize, usually to the lungs.  Massive necrosis (tumour cells were hard to find on H+E) and neoplastic cells with vesicular nuclei and prominent eosinophilic cytoplasm (rhabdoid like) are an almost constant finding in this variant of ES. CD34, EMA and CK AE1/AE3 +ve; S100, CD31, ERG and desmin all –ve. 

DD: Malignant extrarenal rhabdoid tumour – usually occurs in young children. 

Case 3062  1.4.22 (R. Carr) F 60 papule, vulva 

Small, fibrotic dermal nodule containing darkly-staining, angulated tumour cells, looking vaguely granulomatous.  P63 -ve/epithelial markers +ve.  Doesn’t have the typical features of a “proximal type” ES. 

Case 4113  26.10.22 (H. Diwan) F 70 multiple posterior neck masses 

Expansile dermal tumour composed of atypical pleomorphic epithelioid cells dissecting collagen; acantholysis in places; tissue necrosis. Proximal type.