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Peripheral Neuroepithelioma with Ganglion Cells: Report of Two Cases and Review of the Literature
Authors:Susan Williams  David M. Parham  Jesse J. Jenkins III
Affiliation:(1) Department of Pathology, University of Tennessee, 899 Madison Avenue, Memphis, TN 38163, USA , US;(2) Department of Pathology, University of Arkansas for Medical Sciences, 800 Marshall Street, Little Rock, AR 72202, USA , US;(3) Department of Pathology and Laboratory Medicine, St. Jude Children's Research Hospital, 332 North Lauderdale Street, and Department of Pathology, University of Tennessee, 899 Madison Avenue, Memphis, TN 38105-2794, USA , US
Abstract:Peripheral neuroepitheliomas, also known as peripheral primitive neuroectodermal tumors, are by definition primitive embryonal lesions generally composed of poorly differentiated neuroectodermal elements. We have examined two cases that paradoxically contain extensive foci of ganglionic differentiation similar to that of ganglioneuroblastoma, in addition to primitive elements. One tumor arose from the chest wall of an 8-year-old male and the other from the abdominal wall of a 15-year-old male. Differentiation into a mature ganglionic phenotype was confirmed by immunohistochemistry in one case. Rare peripheral neuroepitheliomas have a capacity for maturation that is not generally appreciated. These lesions should not be confused with ganglioneuroblastomas, which are genotypically unrelated neoplasms. Received August 27, 1997; accepted March 27, 1998.
Keywords:: peripheral neuroepithelioma   ganglion cells   neuroblastoma   ganglioneuroblastoma   primitive neuroectodermal tumor
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