Journals / Turkish Neurosurgery / 2001 / Cilt: 11 - Sayı: 1
9 aylık infantil erkekte desmoplastik supratentoriyal nöroepitelyal tümör: Olgu sunumu
- Journal
- Turkish Neurosurgery
- Pages
- 69–72
- DOI
- —
Abstract
Desmoplastic infantile neuroepithelial tumors (DINTs) are superficially located supratentorial neoplasms that include masses known as "desmoplastic infantile gangliomas" (DIGs) and "desmoplastic infantile astrocytomas" (DIAs). Apart from the presence of ganglion cells in DIGs, these tumors and DIAs share the same features. Traditionally, tumors have been designated as DIAs only when intensive histological examination has revealed no ganglion cells. Some DINTs show no ganglion cells on routine staining with hematoxylin and eosin (HE), but are positive for glial fibrillary acidic protein (GFAP), neuronspecific enolases (NSE), neurofilament protein (NF), and synaptophysine on immunohistochemical staining. Currently, it is generally agreed that DIGs and DIAs are variants of one type of tumor, and that both these forms of neoplasia should be labeled DINTs. We describe the case of a 9-month-old male infant who presented with progressive head enlargement. The baby had a cystic, 8 cm-diameter, space-occupying lesion in his left parietal lobe that was surrounded by edema. The mass was identified on cranial computerized tomography, and the patient underwent total tumor excision. Microscopic examination of the mass revealed extensive desmoplasia and focal aggregations of primitive cells. Sections stained with HE showed no ganglion cells; however, immunohistochemical staining revealed widespread GFAP positivity, and focal positivity for NSE and synaptophysine. The diagnosis was DINT. This report discusses the clinicopathologic features of the case, and compares our findings to those documented in previous reports of DIA and DIG attached to the dura.
Özet
Desmoplastik İnfantil Nöroepitelyal Tümörler (DİNT), Desmoplastik İnfantil Gangliogliomalar (DİG) ve Desmoplastik İnfantil Astrositomalar (DİA) olarakta bilinen yüzeyel yerleşimli supratentorial tümörlerdir. DİG'larda ganglion hücrelerinin bulunmasının dışında bu iki tip neoplazi aynı özellikleri taşır. Bu tömürler dikkatli histolojik incelemelere rağmen ganglion hücresi bulunamaz ise DİA olark isimlendirilir. Bazı DİNT'ler Hematoksilen Eozin (H.E) ile rutin boyamalarda ganglion hücresi göstermez fakat Glial fibriler Acidic Protein (GFAP), Nöron Spesifik Enolaz (NSE), Nöroflament Protein (NF) ve Synaptofizin ile immunohistokimyasal boyamalarda pozitiflik gösterirler. Genel olarak kabul edilen görüş, DİG ve DİA'nın bir tip tümörün varyantları olduğu ve bunların hepsinin DİNT terimi altında toplanması gerektiğidir.