JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
Rare Location for Dysembryoplastic Neuroepithelial Tumor in the Septum Pellucidum
Rimal H. Dossani, MD, Devi Patra, MD, Nimer Adeeb, MD, Elizabeth Wild, MD, Abhilasha Ghildyal, MD, Marjorie Fowler, MD, Christina Notarianni, MD
The septum pellucidum is a rare location for Dysembryoplastic neuroepithelial tumors (DNETs), which are most commonly located in the temporal cortex. In this case report, we present the case of a five-year-old boy who presented with seizures. T2-weighted magnetic resonance imaging (MRI) revealed a hyperintense mass arising from the right septumpellucidum. The patient was placed in right lateral decubitus position and underwent right interhemispheric craniotomy for resection of mass. Gross total resection was achieved and pathology was consistent with DNET. The patient has been free of seizures since the time of operation, and no recurrence has been found on MRI scans. We review the molecular and histopathological features of DNETs. Most importantly, for the interest of the general medical community, we present a differential diagnosis of pediatric septum pellucidum tumors based on MRI and histopathological findings.
INTRODUCTION
Dysembryoplastic neuroepithelial tumors (DNET) are benign glioneural tumorspresentingwithepilepsy inchildrenandyoung adults. DNETs aremost commonly located in the temporal lobeof the supratentorial cortex, but case reports and small case series have documented DNETs arising from the septum pellucidum and the foramen of Monro. On magnetic resonance imaging (MRI), DNETs appear as hyperintense on T2-weighted and fluid- attenuated inversion recovery (FLAIR) sequences, and do not exhibit contrast enhancement. The best treatment for DNETs is surgical resection. Adjuvant chemotherapy and radiation are not necessary. Pathology demonstrates disorganized microcystic architecture with both glial and neuronal elements without cytological atypia. In this report, we present a septum pellucidum DNET in a five-year-old boy treated with surgical resection. For the interest of the larger medical community, we present a differential diagnosis of pediatric septum pellucidum tumors based on MRI and histopathological findings.
CASE REPORT
The patient is a five-year-old boy who presented with worsening headaches for six months. Three months prior to presentation, he developed seizures well controlled on oxcarbazepine. His past medical history was notable for developmental delay because he was unable to walk until the age of three. His neurological exam was within normal limits. T2-weighted and fluid-attenuated inversion recovery MRI sequences showed a hyperintense mass arising from the right septum pellucidum (Figure 1). T1-weighted MRI showed isointense mass that did not enhance with contrast. The mass appeared to extend down to the foramen of Monro without hydrocephalus.
Figure 1: Preoperative magnetic resonance images showing a mass arising from the septum pellucidum and entering the right lateral ventricle. T1- weighted with contrast axial (A) and coronal (B) images and fluid attenuated inversion recovery (FLAIR) axial (C) and coronal (D) cuts demonstrate the tumor in the right lateral ventricle.
J La State Med Soc VOL 169 SEPTEMBER/OCTOBER 2017 123
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