Abstract

Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge

Highlights

  • Classification of some pediatric primary central nervous system tumors can be challenging on the basis of routine histology and immunohistochemistry

  • All patients were alive with disease with a mean follow-up of 112 months. These four cases illustrate the morphological diversity of well-differentiated, oligodendroglioma-like glial neoplasms and the uncertainty in their classification among pediatric tumors

  • The diagnosis is often aided by the presence of secondary histological structures of Scherer, combined losses in chromosome 1p and 19q, and mutations in the either IDH-1 or the IDH-2 gene

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Summary

Introduction

Classification of some pediatric primary central nervous system tumors can be challenging on the basis of routine histology and immunohistochemistry. While OD is considered a tumor of adults, rare reports identify tumors with similar features in the pediatric population. Many studies on OD included patients younger than 15 years, but only a few studies analyzed pediatric OD exclusively. Such studies present some evidence that the biology of these tumors may be different from their adult counterparts. Another study demonstrated a much higher survival probability of pediatric oligodendrogliomas compared to adults [3]. Pediatric ODs reported to date seem to lack the typical molecular and clinical features of adult ODs, and the distinction from other well-differentiated tumors such as dysembryoplastic neuroepithelial tumor (DNT) may be extremely difficult [4]

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