Epithelioid Sarcoma in a child presenting as a submandibular mass

Epithelioid sarcoma (ES) is a clinical curiosity with uncertain histogenesis1. Histologically, two variants have been described: the .classic or distal. 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 necrosis 2, while the .proximal. variant , first described in 1997 by Guillou et al. 3, 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. In this monograph we report a case of pediatric epithelioid sarcoma presented as a submandibular mass and to the best of our knowledge it has not been previously documented in this place.