Tumores neuroectodérmicos primitivos periféricos de localización en el área orocervical: presentación de dos casos clínicos

Abstract

Introduction: Peripheral primitive neuroectodermal tumors (PNET) are a family of smallround cell tumors of presumed neuroectodermal origin. This broad family can be subdivided into three major groups: PNET from the central nervous system, PNET from the autonomic nervous system or peripheral PNET. Ewing's sarcoma and peripheral neuroepitelioma, the two most frequently encountered members of the peripheral PNET family, are considered to represent a spectrum according to the extent of neuroectodermal differentiation, ranging from the least differentiated (Ewing's sarcoma) to the most differentiated (peripheral neuroepithelioma). Case report: We present a patient with a peripheral neuroectodermal tumor located in the neck and another one with a peripheral neuroectodermal tumor of the mandibular condyle. Discussion: Peripheral neuroectodermal tumors are a very rare and aggressive tumors. They characteristically reveal the presence of small round cells and a translocation of the gene EWS. The prognosis in overall is very poor. Due to the small numbers of cases published the best treatment is not well defined.