JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
alterations in seven patients with foramen of Monro and septum pellucidum DNETs. They noted that septum pellucidum DNETs did not show KIAA549-BRAF fusions or FGFR1 mutations, alterations commonly noted in pilocytic astrocytomas. Further, BRAF V600E mutations, the most common alteration in cortical DNETs, were absent in foramenofMonro and septumpellucidum DNETs. Of note, malignant transformation to high grade gliomas have been infrequently reported from cortical DNETs, 9,10,31 but such occurrence has never been found in DNETs at extracortical sites. These distinctions indicate that perhaps periventricular DNETs are a distinct clinical entity from supratentorial cortical DNETs.
Rimal H. Dossani, MD, Deva Patra, MD, Nimer Adeeb, MD Elizabeth Wild, MD, and Christina Notarianni, MD, are affiliated with the Department of Neurosurgery at the Louisiana State University Health Schiences Center, Shreveport, LA. Abhilasha Ghildyal andMDMarjorie Fowler, MD, are affiliated with the Department of Pathology at the Louisiana State University Health Sciences Center, Shreveport, LA. 13. Spalice A, Ruggieri M, Grosso S, et al. Dysembryoplastic neuroepithelial tumors: a prospective clinicopathologic and outcome study of 13 children. Pediatr Neurol. 2010;43(6):395-402. 14. Kirkpatrick PJ, Honavar M, Janota I, Polkey CE. Control of temporal lobe epilepsy following en bloc resection of low-grade tumors. J Neurosurg. 1993;78(1):19-25. 15. Minkin K, Klein O, Mancini J, Lena G. Surgical strategies and seizure control in pediatric patients with dysembryoplastic neuroepithelial tumors: a single-institution experience. J Neurosurg Pediatr. 2008;1(3):206-210. 16. Nolan MA, Sakuta R, Chuang N, et al. Dysembryoplastic neuroepithelial tumors in childhood: long-term outcome and prognostic features. Neurology . 2004;62(12):2270-2276. 17. Altinors N, Calisaneller T, Gulsen S, Ozen O, Onguru O. Intraventricular dysembryoplastic neuroepithelial tumor: case report. Neurosurgery . 2007;61(6):E1332-1333; discussion E1333. 18. Yamasaki F, Kurisu K, Satoh K, et al. Apparent diffusion coefficient of human brain tumors at MR imaging. Radiology . 2005;235(3):985-991. 19. Tatke M, Sharma A, Malhotra V. Dysembryoplastic neuroepithelial tumour. Childs Nerv Syst. 1998;14(7):293-296. 20. Daumas-Duport C, Pietsch T, Hawkins C, Shankar S. Dysembryoplastic neuroepithelial tumour. In: Louis D, Ohgaki H, Wiestler O, (eds) ea, eds. WHO Classification of Tumours of the Central Nervous System, ed 4. Lyon: IARC; 2007:99-102. 21. Komori T, Scheithauer BW, Hirose T. A rosette-forming glioneuronal tumor of the fourth ventricle: infratentorial form of dysembryoplastic neuroepithelial tumor? Am J Surg Pathol. 2002;26(5):582-591. 22. Leung SY, Gwi E, Ng HK, Fung CF, Yam KY. Dysembryoplastic neuroepithelial tumor. A tumor with small neuronal cells resembling oligodendroglioma. Am J Surg Pathol. 1994;18(6):604-614. 23. Gessi M, Hattingen E, Dorner E, et al. Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases. Am J Surg Pathol . 2016;40(6):806-811. 24. Saito T, Sugiyama K, Yamasaki F, et al. Familial occurrence of dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: case report. Neurosurgery . 2008;63(2):E370-372; discussion E372. 25. Fedi M, Anne Mitchell L, Kalnins RM, et al. Glioneuronal tumours in neurofibromatosis type 1: MRI-pathological study. J Clin Neurosci. 2004;11(7):745-747. 26. Fujisawa H, Marukawa K, Hasegawa M, et al. Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors. J Neurosurg . 2002;97(6):1350-1355. 27. 27. FontebassoAM, Bechet D, JabadoN. Molecular biomarkers inpediatric glial tumors: a needed wind of change. Curr Opin Oncol. 2013;25(6):665- 673. 28. Chappe C, Padovani L, Scavarda D, et al. Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression. Brain Pathol . 2013;23(5):574-583. 29. Gessi M, MoneimYA, Hammes J, et al. FGFR1 mutations in Rosette-forming glioneuronal tumors of the fourth ventricle. J Neuropathol Exp Neurol. 2014;73(6):580-584. 30. Cataltepe O, Marshall P, Smith TW. Dysembryoplastic neuroepithelial tumor located in pericallosal and intraventricular area in a child. Case report. J Neurosurg Pediatr . 2009;3(6):456-460. 31. Hammond RR, Duggal N, Woulfe JM, Girvin JP. Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg. 2000;92(4):722-725.
CONCLUSION
DNETs of the septum pellucidum are a rare location for DNETs and only a few case reports have been reported to date. A gross total excision of septum pellucidum lesions is sufficient to cure the lesion without need for adjuvant chemoradiation. In light of recent evidence, DNETs of the septumpellucidummay represent a distinct clinical entity within the larger category of DNETs as they lack BRAF V600E mutations, which are the most common genetic alteration found in cortical DNETs. Future studies highlighting the unique genetic profiles of septum pellucidum and other periventricular DNETs will serve to highlight a novel pathogenesis for these lesions.
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14 J La State Med Soc VOL 170 JANUARY/FEBRUARY 2018
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