Abstract
Introduction
Dysembryoplastic neuroepithelial tumors (DNET) are classically considered as benign, cortically based tumors that are stable. However, there were case reports that suggested DNET may not be as benign as previously thought. The purpose of our study was to identify atypical location, characteristics, and behavior of DNET in children.
Methods
The MRI images of 51 patients with pathologically proven DNET were retrospectively reviewed. The following were assessed: tumor location, neuroimaging appearances including hemorrhage, calcification and edema, tumor growth preoperatively and after subtotal resection, tumor recurrence, malignant transformation, and metastatic seeding.
Results
Two (3.9 %) patients had intraventricular lesions, three (5.9 %) had associated edema, three (5.9 %) had calcifications, and one (2 %) had hemorrhage on preoperative CT and MRI. Sixteen of 51 (31.4 %) lesions exhibited enhancement post gadolinium administration. Six of 29 (20.7 %) preoperative lesions that had follow-up imaging were enlarging prior to surgery. In 6 of 18 (33.3 %) with subtotal resection, there was an increase in size of the residual tumor. Tumor recurrence at the surgical bed occurred in 3 of 30 (10 %) patients who had gross total resection. Two of 51 (3.9 %) cases developed secondary lesions distant to the primary tumor; the secondary lesions were within the lateral ventricles.
Conclusion
In children, DNET may have atypical location, characteristics and behavior, including growth of primary or residual lesions and multifocal tumor. These findings emphasize the need for follow-up of patients with DNET post-resection.
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References
Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER Jr, Vedrenne C (1988) Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 23:545–556
Lee J, Lee BL, Joo EY, Seo DW, Hong SB, Hong SC, Suh YL, Lee M (2009) Dysembryoplastic neuroepithelial tumors in pediatric patients. Brain Dev 31:671–681. doi:10.1016/j.braindev.2008.10.002
Aronica E, Leenstra S, van Veelen CW, van Rijen PC, Hulsebos TJ, Tersmette AC, Yankaya B, Troost D (2001) Glioneuronal tumors and medically intractable epilepsy: a clinical study with long-term follow-up of seizure outcome after surgery. Epilepsy Res 43:179–191
Giulioni M, Rubboli G, Marucci G, Martinoni M, Volpi L, Michelucci R, Marliani AF, Bisulli F, Tinuper P, Castana L, Sartori I, Calbucci F (2009) Seizure outcome of epilepsy surgery in focal epilepsies associated with temporomesial glioneuronal tumors: lesionectomy compared with tailored resection. J Neurosurg 111:1275–1282. doi:10.3171/2009.3.JNS081350
Garcia-Fernandez M, Fournier-Del Castillo C, Ugalde-Canitrot A, Perez-Jimenez A, Alvarez-Linera J, De Prada-Vicente I, Suarez-Rodriguez J, Bernabeu-Verdu J, Villarejo-Ortega F (2011) Epilepsy surgery in children with developmental tumours. Seizure 20:616–627. doi:10.1016/j.seizure.2011.06.003
Khajavi K, Comair YG, Wyllie E, Palmer J, Morris HH, Hahn JF (1999) Surgical management of pediatric tumor-associated epilepsy. J Child Neurol 14:15–25
Kawataki T, Sato E, Kato T, Sato T, Horikoshi T, Kinouchi H (2010) A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter. J Neurosurg Pediatr 6:600–603. doi:10.3171/2010.10.PEDS10391
Cataltepe O, Marshall P, Smith TW (2009) Dysembryoplastic neuroepithelial tumor located in pericallosal and intraventricular area in a child. Case report. J Neurosurg Pediatr 3:456–460. doi:10.3171/2009.1.PEDS0823
Maher CO, White JB, Scheithauer BW, Raffel C (2008) Recurrence of dysembryoplastic neuroepithelial tumor following resection. Pediatr Neurosurg 44:333–336. doi:10.1159/000138372
Whittle IR, Dow GR, Lammie GA, Wardlaw J (1999) Dsyembryoplastic neuroepithelial tumour with discrete bilateral multifocality: further evidence for a germinal origin. Br J Neurosurg 13:508–511
Schittenhelm J, Mittelbronn M, Wolff M, Truebenbach J, Will BE, Meyermann R, Beschorner R (2007) Multifocal dysembryoplastic neuroepithelial tumor with signs of atypia after regrowth. Neuropathology 27:383–389
Ray WZ, Blackburn SL, Casavilca-Zambrano S, Barrionuevo C, Orrego JE, Heinicke H, Dowling JL, Perry A (2009) Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. J Neurooncol 94:283–292. doi:10.1007/s11060-009-9849-9
Hammond RR, Duggal N, Woulfe JM, Girvin JP (2000) Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 92:722–725. doi:10.3171/jns.2000.92.4.0722
Rushing EJ, Thompson LD, Mena H (2003) Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. Ann Diagn Pathol 7:240–244
Stanescu Cosson R, Varlet P, Beuvon F, Daumas Duport C, Devaux B, Chassoux F, Fredy D, Meder JF (2001) Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow-up: a study of 53 cases. J Neuroradiol 28:230–240
Campos AR, Clusmann H, von Lehe M, Niehusmann P, Becker AJ, Schramm J, Urbach H (2009) Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology 51:433–443. doi:10.1007/s00234-009-0511-1
Thom M, Toma A, An S, Martinian L, Hadjivassiliou G, Ratilal B, Dean A, McEvoy A, Sisodiya SM, Brandner S (2011) One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetic, and clinical correlations and a review of the literature. J Neuropathol Exp Neurol 70:859–878. doi:10.1097/NEN.0b013e3182302475
Yu AH, Chen L, Li YJ, Zhang GJ, Li KC, Wang YP (2009) Dysembryoplastic neuroepithelial tumors: magnetic resonance imaging and magnetic resonance spectroscopy evaluation. Chin Med J (Engl) 122:2433–2437
Fernandez C, Girard N, Paz Paredes A, Bouvier-Labit C, Lena G, Figarella-Branger D (2003) The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol 24:829–834
Ostertun B, Wolf HK, Campos MG, Matus C, Solymosi L, Elger CE, Schramm J, Schild HH (1996) Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J Neuroradiol 17:419–430
Thom M, Gomez-Anson B, Revesz T, Harkness W, O'Brien CJ, Kett-White R, Jones EW, Stevens J, Scaravilli F (1999) Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases. J Neurol Neurosurg Psychiatry 67:97–101
Lee DY, Chung CK, Hwang YS, Choe G, Chi JG, Kim HJ, Cho BK (2000) Dysembryoplastic neuroepithelial tumor: radiological findings (including PET, SPECT, and MRS) and surgical strategy. J Neurooncol 47:167–174
Minkin K, Klein O, Mancini J, Lena G (2008) Surgical strategies and seizure control in pediatric patients with dysembryoplastic neuroepithelial tumors: a single-institution experience. J Neurosurg Pediatr 1:206–210. doi:10.3171/PED/2008/1/3/206
Qaddoumi I, Ellison DW, Morris EB, Broniscer A, Boop F, Merchant T, Palmer SL, Gajjar A (2010) Dysembryoplastic neuroepithelial tumors and cognitive outcome: cure at a price? Cancer 116:5461–5469. doi:10.1002/cncr.25528
Nolan MA, Sakuta R, Chuang N, Otsubo H, Rutka JT, Snead OC 3rd, Hawkins CE, Weiss SK (2004) Dysembryoplastic neuroepithelial tumors in childhood: long-term outcome and prognostic features. Neurology 62:2270–2276
Sakuta R, Otsubo H, Nolan MA, Weiss SK, Hawkins C, Rutka JT, Chuang NA, Chuang SH, Snead OC 3rd (2005) Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor. J Child Neurol 20:377–384
Gonzales M, Dale S, Susman M, Nolan P, Ng WH, Maixner W, Laidlaw J (2007) Dysembryoplastic neuroepithelial tumor (DNT)-like oligodendrogliomas or Dnts evolving into oligodendrogliomas: two illustrative cases. Neuropathology 27:324–330
Duggal N, Taylor R, Zou GY, Hammond RR (2008) Dysembryoplastic neuroepithelial tumours: clinical, proliferative and apoptotic features. J Clin Pathol 61:127–131. doi:10.1136/jcp.2006.045369
Josan V, Smith P, Kornberg A, Rickert C, Maixner W (2007) Development of a pilocytic astrocytoma in a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 106:509–512. doi:10.3171/ped.2007.106.6.509
Yamaguchi N, Ohnishi H, Tachibana O, Hasegawa M, Emori T (1998) An enlarging dysembryoplastic neuroepithelial tumor during a 6-year period: a case report. No Shinkei Geka 26:1097–1101
Sampetrean O, Maehara T, Arai N, Nemoto T (2006) Rapidly growing dysembryoplastic neuroepithelial tumor: case report. Neurosurgery 59:E1337–E1338. doi:10.1227/01.NEU.0000245621.62721.79, discussion E1338
Preuss M, Nestler U, Zuhlke CJ, Kuchelmeister K, Neubauer BA, Jodicke A (2010) Progressive biological behavior of a dysembryoplastic neuroepithelial tumor. Pediatr Neurosurg 46:294–298. doi:10.1159/000320729
Emmez H, Kale A, Egemen E, Eser P, Kaymaz M, Pasaoglu A (2012) Intraventricular dysembryoplastic neuroepithelial tumour: case report. Neurol Neurochir Pol 46:192–195
Xiong J, Liu Y, Chu SG, Chen H, Chen HX, Mao Y, Wang Y (2012) Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis. Clin Neuropathol 31:31–38
Altinors N, Calisaneller T, Gulsen S, Ozen O, Onguru O (2007) Intraventricular dysembryoplastic neuroepithelial tumor: case report. Neurosurgery 61:E1332–E1333. doi:10.1227/01.neu.0000306114.08540.aa, discussion E1333
Yuan J, Sharma N, Choudhri H, Figueroa R, Sharma S (2011) Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it the most common extracortical location for DNT? Childs Nerv Syst 27:485–490. doi:10.1007/s00381-010-1307-7
Baisden BL, Brat DJ, Melhem ER, Rosenblum MK, King AP, Burger PC (2001) Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: a lesion often misdiagnosed as glioma: report of 10 cases. Am J Surg Pathol 25:494–499
Bilginer B, Soylemezoglu F, Cila A, Akalan N (2009) Intraventricular dysembryoplastic neuroepithelial tumor-like neoplasm with disseminated spinal tumor. Turk Neurosurg 19:69–72
Wang F, Qiao G, Li X, Gui Q (2007) A dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus in a 57-year-old woman: case report. Neurosurgery 61:E420. doi:10.1227/01.NEU.0000255530.46092.AA, discussion E420
Cervera-Pierot P, Varlet P, Chodkiewicz JP, Daumas-Duport C (1997) Dysembryoplastic neuroepithelial tumors located in the caudate nucleus area: report of four cases. Neurosurgery 40:1065–1069, discussion 1069–1070
Fujimoto K, Ohnishi H, Tsujimoto M, Hoshida T, Nakazato Y (2000) Dysembryoplastic neuroepithelial tumor of the cerebellum and brainstem. Case report. J Neurosurg 93:487–489. doi:10.3171/jns.2000.93.3.0487
Kuchelmeister K, Demirel T, Schlorer E, Bergmann M, Gullotta F (1995) Dysembryoplastic neuroepithelial tumour of the cerebellum. Acta Neuropathol 89:385–390
Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP (1999) Dysembryoplastic neuroepithelial tumors: nonspecific histological forms—a study of 40 cases. J Neurooncol 41:267–280
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Daghistani, R., Miller, E., Kulkarni, A.V. et al. Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology 55, 217–224 (2013). https://doi.org/10.1007/s00234-013-1135-z
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DOI: https://doi.org/10.1007/s00234-013-1135-z