Turkish Neurosurgery 2001 , Vol 11 , Num 1-2
Şahsine TOLUNAY1, Gülaydan FİLİZ1, Soner ŞAHİN2, Kaya AKSOY2
1Uludağ Üniversity School of Medicine, Department of Patholoji, Bursa, Turkey
2Uludağ Üniversity School of Medicine, Department of Neurosurgery, Bursa, Turkey
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, immunohistoechemical staining revealed widespread GFAP positivity, and focal positivity for NSE and synaptophysine. The diagnosis was DINI. 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. Keywords : Desmoplasia, embryonal neuroepithelial tumors, infantile tumors
Corresponding author : Şahsine Tolunay, olunay@uludag-edu.tr