AGGRESSIVE PRIMITIVE TUMOR IN A PEDIATRIC PATIENT WITH LETHAL EVOLUTION

Abstract

Primitive tumors are considered rare, malignant, small and round-cell tumors that represent a disease of childhood and categorized into the Ewing family of tumors due its similarities. Herein we report a case of primitive tumor in a pediatric patient with lethal evolution. A 1-year-old black male patient was showing an expansive lesion in the left hemimandible, 8 cm in diameter, exhibiting superficial telangiectasia, crackling consistency, similar to a mucous membrane. The radiographic findings revealed irregular lytic radiolucency. An incisional biopsy was performed and the histopathologic report suggested a mucoepidermoid carcinoma. A histochemical and immunohistochemical panel was performed with the following markers: PAS-stain (+), DAK-p63 (+), AE1 ⁄AE3 (+), S-100 (+), and calponin (focaly +). The patient died 2 months after the biopsy procedure, before the immunohistochemical report was finished. We discuss the aggressive nature of such a rare tumor.