Abstract

SANCHEZ ACEDO, C. et al. Peripheral primitive neuroectodermal tumors located in orocervical area: presentation of two clinical cases. Rev Esp Cirug Oral y Maxilofac [online]. 2010, vol.32, n.1, pp.25-30. ISSN 2173-9161.

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.

Keywords : Peripheral primitive neuroectodermal tumor.

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