Epithelioid sarcoma

Epithelioid sarcoma (ES) is a very rare characterized by loss of INI1 . Enzinger first described ES in 1970, but the histopathologic differential diagnosis remains challenging. There are two subtypes, classical type with spindle epithelioid to central pseudogranulomatous cells, and proximal type, which predominantly composed rhabdoid cells. symptoms signs not specific depend on tumor localization. The only treatment for radical excision microscope-radical margin. In general, best extremes resection wide margin or amputation without lymph node dissection. Surgery may be followed adjuvant chemotherapy and/or radiation therapy. Referral patients center that offers hypofractionation RT trials multidisciplinary clinical should considered upfront. Neoadjuvant ifosfamide doxorubicin / therapy must used after team discussion. On 23 January 2020, US Food Drug Administration (FDA) approved tazemetostat – an inhibitor zeste homolog 2 enhancer metastatic locally advanced eligible resection.