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Genomic alterations Epithelioid sarcoma is characterized by inactivation of the SMARCB1 gene, which is present in both conventional and proximal types of epithelioid sarcoma.[55] This abnormality leads to increased dependence on EZH2 and tumor formation.[56] Treatment of epithelioid sarcoma Treatment options for epithelioid sarcoma include the following: Surgery with or without chemotherapy and/or radiation therapy Surgical removal of primary and recurrent tumor(s) is the most effective treatment.[57][Level of evidence C1] Because of the propensity of this disease to have occult metastasis to the lymph nodes, sentinel lymph node biopsy is recommended for epithelioid sarcoma of the extremities or buttocks in the absence of clinically (by imaging or physical examination) enlarged lymph nodes.[58] Evidence (surgery with or without chemotherapy and/or radiation therapy): In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections

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