Abstract
Oligodendrogliomas are rare in the pediatric population, and most oligodendroglioma-like tumors in this age group may belong to other entities. In addition, accurate diagnosis and grading of such lesions using criteria developed for adult oligodendrogliomas prove difficult, and often controversial. During a study of tumors previously diagnosed as pediatric oligodendroglioma, we identified four tumors displayed features of that resembled oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumor but could not be classified as either one of these entities. Ther clinical, histological and immunohistochemical features of these cases were investigated in this study. Two male (both 9 years old) and two female (ages 4 years and 20 months) patients presented with new onset of seizures. All patients were treated surgically, and two required reoperation. Histologically, the tumors were well-differentiated glial neoplasms with focal angiocentric pattern, delicate vascularity, diffuse growth, infiltrative margins, cortical nodules, focal myxoid areas, and leptomeningeal extension. Immunohistochemical studies showed diffuse nuclear positivity with Olig-2 and GFAP antibodies, whereas staining with neuronal markers, EMA, p53, and IDH1 were negative. Fluorescent in-situ hybridization analysis demonstrated intact 1p/19q in all tumors, and there was no ultrastructural evidence of ependymal differentiation. 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.
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
Summary
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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