Primary peripheral primitive neuroectodermal tumor of the mandible

Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement of the mandible is however rare. A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible. Given their insidious clinical symptoms, variable locations and rarity, the accurate diagnosis of peripheral PNETs poses a challenge for clinicians. PNETs exhibits characteristic immunophenotypical and genetic features that distinguish them from other small round cell tumors. A multidisciplinary approach is necessary to manage patients affected by PNET. There is however no consensus about the best therapeutic strategy. We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.