Epithelioid sarcoma: a clinical and pathological analysis
Objective To study the clinicopathologic features of epithelioid sarcoma (ES),with review of the literature.Methods 22 cases of ES were selected for immunohistochemical staining.Clinical and pathologic features of these cases were also reviewed.The expression of INI1,CKpan,EMA,Vim,CD34,CK8,and CK7 was detected by immunostaining.Results The patients' s ages ranged from 16 to 75 years (mean,40.2 years).The median age of proximal-type ES was 41.1 years,and that of conventional ES was 36.9 years.The male-to-female ratio was 1.2∶ 1.Conventional ES mostly occurred in the distal extremities,proximal-type often in perineal,genital region and trunk.Histologically,granuloma-like architecture with central necrosis often was observed in conventional ES.In proximal-type ES,tumor cells had prominent epithelioid and/or rhabdoid features which had marked cytologic atypia.The immunohistochemical study showed Vim was positive in all cases.CKpan,CK8,EMA,CK7 and CD34 were expressed in 86.3% (19/22),81.8% (17/22),90.9% (20/22),59.1% (13/22) and 4.5% (1/22),respectively.The immunostain for S100 which was focal and weak was found in 22.7% (5/22).CD31 and HMB45 were negative in all cases.The loss of INI1 was found in 83.3% (10/12) and 70.0% (7/10)in the conventional ES and the proximal-type ES,respectively.INI1 was detected in poorly differentiated squamous cell carcinoma,malignant melanoma,clear cell sarcoma of soft tissue,angiosarcoma and epithelioid hemangioendothelioma.INI1 was negative in all cases of malignant rhabdoid tumor and in 1 case (1/15) of malignant peripheral nerve sheath tumor.Conclusions Owing to the histologic variety,immunostaining for INI1 combined with other commonly used markers (CKpan,CK8,EMA and CD34) will be helpful for correct diagnosis of ES.