Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

Abstract

Primitive neuroectodermal tumor (PNET) is a rare, highly malignant small round cell neoplasm that has poor prognosis. PNET belongs to the Ewing's sarcoma (ES) family of tumors. Both PNET and ES share a common neuroectodermal origin. 1,2 However, PNET exhibits a more aggressive clinical behavior than ES and metastasizes very rapidly. The term "PNET" was first introduced by Hart and Earle 5 to describe medulloblastoma-like lesions in the cerebral hemisphere. In 1986, Dehner 6 suggested differentiation between central and peripheral PNETs. Subsequently, PNET arising from the central nervous system was termed "central PNET (cPNET)", while tumors developing outside the central and autonomic nervous systems were called "peripheral PNETs (pPNETs)". PNET accounts for 1%-4% of all soft tissue neoplasms. It is more common in children and young adults. 11 PNET mostly affects the thoracopulmonary region, pelvis, abdominal region, and extremities; 2,8,10,11 less frequently, it occurs in the head and neck areas. PNET of the maxilla is exceedingly rare among elderly people. Only 10 cases Here, we report a case of a 67-year-old male diagnosed with PNET of the maxillary sinus after detailed clinical, radiological, and histopathological examinations.