Mandibular peripheral primitive neuroectodermal tumor: a rare case report with review of literature

Primitive neuroectodermal tumor (PNET) is a high grade malignant neoplasm of small round cell tumor family, commonly affecting children and young adults. Peripheral primitive neuroectodermal tumor (pPNET) is a predominately neural, nonepithelial malignancy seen outside the nervous system that can arise in any place throughout the body including the diverse tissues of the head and neck. The diagnosis of PNET is confounded by its clinical and histopathological similarity to Ewing's sarcoma of the bone and has seldom been reported in the literature. The paucity of literature pertaining to the successful diagnosis and management of this lesion mandates its documentation and discussion. This article describes a case of an 11-year-old boy with an aggressive pPNET of the mandible. The clinical and radiographic presentations of this rare entity along with a detailed review on the current management modalities have been discussed.