JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
staining. A possible genetic association has been suggested by Saito et al who found a familial occurrence of DNETs in the septum pellucidum. 24 Recently, Gessi et al. studied molecular alterations in 7 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, BRAFV600E 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.
12. Rickert CH, Paulus W. Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001;17(9):503-511. 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. Fontebasso AM, Bechet D, Jabado N. Molecular biomarkers in pediatric 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. Rimal H. Dossani, MD, is affiliated with the Department of Neurosurgery at the Louisiana State University Health Sciences Center, Shreveport, Louisiana. Devi Patra, MD, Nimer Adeeb, MD, ElizabethWild, MD, and Christina Notarianni, MD, are affiliated with the Department of Neurosurgery at the Louisiana State University Health Sciences Center, Shreveport, Louisiana. Abhilasha Ghildyal, MD, and Marjorie Fowler, MD are affiliated with the Department of Pathology at the Louisiana State University Health Sciences Center, Shreveport, Louisiana.
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 BRAFV600E 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.
REFERENCES
1. Sarwar M. The septum pellucidum: normal and abnormal. AJNR Am J Neuroradiol . 1989;10(5):989-1005. 2. Rakic P, Yakovlev PI. Development of the corpus callosum and cavum septi in man. J Comp Neurol. 1968;132(1):45-72. 3. Pearce JM. Some observations on the septum pellucidum. Eur Neurol. 2008;59(6):332-334. 4. Scoffings DJ, Kurian KM. Congenital and acquired lesions of the septum pellucidum. Clin Radiol . 2008;63(2):210-219. 5. Russell EJ, Naidich TP. The enhancing septal/alveal wedge: a septal sign of intraaxial mass. Neuroradiology . 1982;23(1):33-40. 6. Baisden BL, Brat DJ, Melhem ER, Rosenblum MK, King AP, Burger PC. Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: a lesion often misdiagnosed as glioma: report of 10 cases. Am J Surg Pathol. 2001;25(4):494-499. 7. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER, Jr., Vedrenne C. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery . 1988;23(5):545-556. 8. Guesmi H, Houtteville JP, Courtheoux P, Derlon JM, Chapon F. [Dysembryoplastic neuroepithelial tumors. Report of 8 cases including two with unusual localization]. Neurochirurgie . 1999;45(3):190-200. 9. Rushing EJ, Thompson LD, Mena H. Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. Ann Diagn Pathol . 2003;7(4):240-244. 10. Yuan J, Sharma N, Choudhri H, Figueroa R, Sharma S. Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it themost common extracortical location for DNT? Childs Nerv Syst. 2011;27(3):485- 490. 11. Suh YL. Dysembryoplastic Neuroepithelial Tumors. J Pathol Transl Med. 2015;49(6):438-449.
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