Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review

Abstract

106rimitive neuroectodermal tumor (PNET) is a term used to describe a group of highly rare and malignant neoplasms composed of undifferentiated round cell tumors of neuroectodermal origin with variable cell differentiation. PNET generally occurs in the central nervous system (CNS); peripheral PNET occurring outside the CNS has also been reported. Peripheral PNET is commonly related to Ewing's sarcoma (ES). However, it exhibits a more aggressive clinical behavior than ES, with extremely rapid metastasis. PNET has a poor prognosis and is commonly seen in children and young adults.1 Because this tumor presents aggressive biological behavior and tends toward loco-regional and systemic metastases, it is a tumor with an extremely poor survival rate.2 The incidence of head and neck PNET Imaging Features of Primitive Neuroectodermal Tumor in the Maxilla: A Case Report and Literature Review A AB BS S T TR RA AC CT T Primitive neuroectodermal tumor (PNET) is a term used to describe a group of highly malignant neoplasms composed of undifferentiated round cell tumors of neuroectodermal origin with variable cell differentiation. PNET generally occurs in the central nervous system (CNS), but peripheral PNET occurring outside the CNS has also been reported. The most common location of PNET in head and neck region is the orbit, followed by the neck and the parotid gland. PNET of the maxilla is rare. The aims of this report are to present the case of a 41-year-old female diagnosed with PNET tumor of the maxilla after detailed clinical, radiological, and histopathological examinations and to review the previously reported cases of PNET of the maxilla in the literature.K Ke ey y W Wo or rd ds s: : Neuroectodermal tumors, primitive, peripheral; maxilla; diagnosis; cone-beam computed tomography Ö ÖZ ZE ET T Primitif nöroektodermal tümör (PNET), nöroektodermal kökenli farklılaşmamış yuvarlak hücreli tümörlerden oluşan son derece malign tümörlerin bir grubunu tanımlamak için kullanılan bir terimdir. PNET genellikle santral sinir sisteminde meydana gelir fakat santral sinir sistemi dışında ortaya çıkan periferal PNET olguları da rapor edilmiştir. Baş-boyun bölgesinde en fazla gözde görülür. Gözden sonra en sık boyun ve parotis bezinde görülür. Maksilladaki PNET nadirdir. Bu raporun amacı detaylı klinik, radyolojik ve histopatolojik incelemeden sonra maksillada PNET tanısı koyulan 41 yaşında kadın hastayı sunmak ve literatürde daha önce rapor edilen maksilladaki PNET vakalarını incelemektir.A An na ah h t ta ar r K Ke e l li i m me e l le er r: : Nöroektodermal tümörler, primitif, periferik; maksilla; tanı; konik ışınlı bilgisayarlı tomografi T Tu ur rk ki iy ye e K Kl li in ni ik kl le er ri i J J D De en nt ta al l S Sc ci i C Ca as se es s 2 20 01 16 6; ;2 2( (3 3) ): :1 10 06 6--1 12 2