Primitive Neuroectodermal Tumor of Maxilla: A Rare Case

Abstract

ABSTRACT Primitive neuroectodermal tumor (PNET) is an aggressive round cell malignancy of presumed neural crest origin belonging to Ewing's sarcoma family of tumors. Peripheral PNET is less common in head and neck region and only eight cases of primary PNET of maxilla have been reported. We report a case of 3-year-old boy diagnosed with PNET of maxilla based on detailed radiologic, histopathologic, and immunohistochemical studies. Though the imaging features of PNET are nonspecific and definitive diagnosis is only by immunohistochemistry, PNET should be included in the differential diagnoses of fast growing soft tissue tumors of children and young adolescents. We emphasize the need for its early diagnosis and prompt management owing to its aggressive nature and high mortality rate.