Epithelioid Sarcoma in a child presenting as a submandibular mass

S Al-Salam, M Al Ashari

Abstract

Epithelioid sarcoma (ES) is a clinical curiosity with uncertain histogenesis. Histologically ,two variants have been described: the ìclassic or distalî variant, which usually affects distal extremities of adolescents and young adults between 15 and 35 years of age, particularly in the hand and foot, and consists of a subcutaneous or deeper nodular proliferation of rounded to plump cells with abundant eosinophilic
cytoplasm palisading around areas of necrosi2,while the ìproximalî variant , first described in 1997 by Guillou et al. is found mostly in the pelvic, perineal, and genital tracts of young to middle-aged adults and characterized by a proliferation of epithelioidlike cells with rhabdoid features in the absence of a granuloma-like pattern

Key words: Soft tissue sarcoma; Epithelioid sarcoma; Paediatric; Submandibular