JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
Intracranial Septum Pellucidum Dysembryoplastic Neuroepithelial Tumor: Case Presentation and Review of Pediatric Septum PellucidumTumors
Rimal Dossani, MD, Devi Patra, MD, Nimer Adeeb, MD, Elizabeth Wild, MD, Abhilasha Ghildy- al, MD, Marjorie Fowler, MD, Christina Notarianni, MD
Dysembryoplastic neuroepithelial tumors (DNETs) of the septum pellucidum are a rare location for DNETs, which are most commonly located in the temporal cortex. In this case report, we present the case of a 5-year-oldboywhopresentedwith seizures. Magnetic resonance imaging (MRI) revealed a hyperintensemass onT2-weighted imaging arising from the right septumpellucidum. The patient was placed in right lateral decubitus position andunderwent right interhemispheric craniotomy for resectionofmass. Gross total resectionwas achieved and pathology was consistent with DNET. Patient has been free of seizures since the time of operation and MRI scans have been free of recurrence. 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 pellucidumDNET in a 5-year-old male child 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 5-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 mass arising fromseptumpellucidumandentering 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. side down. A small callosotomy via interhemispheric craniotomy was performed to enter the right lateral ventricle. The tumor appeared to be soft and gelatinous and was removed using bipolar cautery and suction. Finally, the septum pellucidum
The patient was taken to the operating room for resection of mass. He was placed in lateral decubitus position with the right
10 J La State Med Soc VOL 170 JANUARY/FEBRUARY 2018
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