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DNET
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Dysembryoplastic neuroepithelial tumor (DNET)
            • Synonyms

              • Mixed glial-neuronal neoplasm
            • Definitions

              • Benign, highly epileptogenic tumor frequently associated with cortical dysplasia

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Onishi S et al: T2-FLAIR mismatch sign in dysembryoplasticneuroepithelial tumor. Eur J Radiol. 126:108924, 2020
                                            2. Slegers RJ et al: Low-grade developmental and epilepsy associated brain tumors: a critical update 2020. Acta Neuropathol Commun. 8(1):27, 2020
                                            3. Yuan F et al: Dysembryoplastic neuroepithelial tumour in the cerebellum: case report and literature review. Br J Neurosurg. 1-3, 2020
                                            4. Chiang JCH et al: Septal dysembryoplastic neuroepithelial tumor: a comprehensive clinical, imaging, histopathologic, and molecular analysis. Neuro Oncol. 21(6):800-8, 2019
                                            5. Ko A et al: Epilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function. Pediatr Neurol. 91:50-6, 2019
                                            6. Luzzi S et al: Dysembryoplastic neuroepithelial tumors: what you need to know. World Neurosurg. 127:255-65, 2019
                                            7. Rau A et al: Discrimination of epileptogenic lesions and perilesional white matter using diffusion tensor magnetic resonance imaging. Neuroradiol J. 32(1):10-6, 2019
                                            8. Trisolini E et al: Actionable FGFR1 and BRAF mutations in adult circumscribed gliomas. J Neurooncol. 145(2):241-245, 2019
                                            9. Yang J et al: Satellite lesions of DNET: implications for seizure and tumor control after resection. J Neurooncol. 143(3):437-445, 2019
                                            10. Isler C et al: Dysembryoplastic neuroepithelial tumours: clinical, radiological, pathological features and outcome. Br J Neurosurg. 32(4):436-441, 2018
                                            11. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Dysembryoplastic Neuroepithelial Tumor. IARC Press. 132-5, 2016
                                            12. Alexander H et al: Progressive dysembryoplastic neuroepithelial tumour. J Clin Neurosci. 22(1):221-4, 2015
                                            13. Ranger A et al: Seizures in children with dysembryoplastic neuroepithelial tumors of the brain-A review of surgical outcomes across several studies. Childs Nerv Syst. ePub, 2015
                                            14. Kim AH et al: Recurrence after gross-total resection of low-grade pediatric brain tumors: the frequency and timing of postoperative imaging. J Neurosurg Pediatr. 14(4):356-64, 2014
                                            15. Yang AI et al: Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy. Epileptic Disord. 16(3):328-32, 2014
                                            16. Chuang NA et al: Glioblastoma multiforme arising from dysembryoplastic neuroepithelial tumor in a child in the absence of therapy. J Pediatr Hematol Oncol. Epub ahead of print, 2013
                                            17. Daghistani R et al: Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 55(2):217-24, 2013
                                            18. Englot DJ et al: Factors associated with seizure freedom in the surgical resection of glioneuronal tumors. Epilepsia. 53(1):51-7, 2012
                                            19. Kim SK et al: Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. Korean J Radiol. 13(4):492-5, 2012
                                            20. Bird-Lieberman G et al: Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. J Neurosurg Pediatr. 7(4):416-20, 2011
                                            21. Alexiou GA et al: Benign lesions accompanied by intractable epilepsy in children. J Child Neurol. 24(6):697-700, 2009
                                            22. Bilginer B et al: Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology. Childs Nerv Syst. 25(4):485-91, 2009
                                            23. Lee J et al: Dysembryoplastic neuroepithelial tumors in pediatric patients. Brain Dev. 31(9):671-81, 2009
                                            24. Ray WZ et al: Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. J Neurooncol. 94(2):283-92, 2009
                                            25. Rutka JT: Dysembryoplastic neuroepithelial tumor. J Neurosurg Pediatr. 3(6):455, 2009
                                            26. Bulakbasi N et al: Dysembryoplastic neuroepithelial tumors: proton MR spectroscopy, diffusion and perfusion characteristics. Neuroradiology. 49(10):805-12, 2007
                                            27. Parmar HA et al: Fluid-attenuated inversion recovery ring sign as a marker of dysembryoplastic neuroepithelial tumors. J Comput Assist Tomogr. 31(3):348-53, 2007
                                            28. Maehara T et al: Usefulness of [11C]methionine PET in the diagnosis of dysembryoplastic neuroepithelial tumor with temporal lobe epilepsy. Epilepsia. 45(1):41-5, 2004
                                            29. Vuori K et al: Low-grade gliomas and focal cortical developmental malformations: differentiation with proton MR spectroscopy. Radiology. 230(3):703-8, 2004
                                            30. Fernandez C et al: The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol. 24(5):829-34, 2003
                                            31. Adamek D et al: Dysembryoplastic neuroepithelial tumour (DNT). Is the mechanism of seizures related to glutamate? An immunohistochemical study. Folia Neuropathol. 39(2):111-7, 2001
                                            32. Stanescu Cosson R et al: Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow-up: a study of 53 cases. J Neuroradiol. 28(4):230-40, 2001
                                            33. Lee DY et al: Dysembryoplastic neuroepithelial tumor: radiological findings (including PET, SPECT, and MRS) and surgical strategy. J Neurooncol. 47(2):167-74, 2000
                                            34. Honavar M et al: Histological heterogeneity of dysembryoplastic neuroepithelial tumour: identification and differential diagnosis in a series of 74 cases. Histopathology. 34(4):342-56, 1999
                                            35. Thom M et al: Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases. J Neurol Neurosurg Psychiatry. 67(1):97-101, 1999
                                            36. Ostertun B et al: Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J Neuroradiol. 17(3):419-30, 1996
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Dysembryoplastic neuroepithelial tumor (DNET)
                                                      • Synonyms

                                                        • Mixed glial-neuronal neoplasm
                                                      • Definitions

                                                        • Benign, highly epileptogenic tumor frequently associated with cortical dysplasia

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Onishi S et al: T2-FLAIR mismatch sign in dysembryoplasticneuroepithelial tumor. Eur J Radiol. 126:108924, 2020
                                                                                      2. Slegers RJ et al: Low-grade developmental and epilepsy associated brain tumors: a critical update 2020. Acta Neuropathol Commun. 8(1):27, 2020
                                                                                      3. Yuan F et al: Dysembryoplastic neuroepithelial tumour in the cerebellum: case report and literature review. Br J Neurosurg. 1-3, 2020
                                                                                      4. Chiang JCH et al: Septal dysembryoplastic neuroepithelial tumor: a comprehensive clinical, imaging, histopathologic, and molecular analysis. Neuro Oncol. 21(6):800-8, 2019
                                                                                      5. Ko A et al: Epilepsy Surgery for Children With Low-Grade Epilepsy-Associated Tumors: Factors Associated With Seizure Recurrence and Cognitive Function. Pediatr Neurol. 91:50-6, 2019
                                                                                      6. Luzzi S et al: Dysembryoplastic neuroepithelial tumors: what you need to know. World Neurosurg. 127:255-65, 2019
                                                                                      7. Rau A et al: Discrimination of epileptogenic lesions and perilesional white matter using diffusion tensor magnetic resonance imaging. Neuroradiol J. 32(1):10-6, 2019
                                                                                      8. Trisolini E et al: Actionable FGFR1 and BRAF mutations in adult circumscribed gliomas. J Neurooncol. 145(2):241-245, 2019
                                                                                      9. Yang J et al: Satellite lesions of DNET: implications for seizure and tumor control after resection. J Neurooncol. 143(3):437-445, 2019
                                                                                      10. Isler C et al: Dysembryoplastic neuroepithelial tumours: clinical, radiological, pathological features and outcome. Br J Neurosurg. 32(4):436-441, 2018
                                                                                      11. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Dysembryoplastic Neuroepithelial Tumor. IARC Press. 132-5, 2016
                                                                                      12. Alexander H et al: Progressive dysembryoplastic neuroepithelial tumour. J Clin Neurosci. 22(1):221-4, 2015
                                                                                      13. Ranger A et al: Seizures in children with dysembryoplastic neuroepithelial tumors of the brain-A review of surgical outcomes across several studies. Childs Nerv Syst. ePub, 2015
                                                                                      14. Kim AH et al: Recurrence after gross-total resection of low-grade pediatric brain tumors: the frequency and timing of postoperative imaging. J Neurosurg Pediatr. 14(4):356-64, 2014
                                                                                      15. Yang AI et al: Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy. Epileptic Disord. 16(3):328-32, 2014
                                                                                      16. Chuang NA et al: Glioblastoma multiforme arising from dysembryoplastic neuroepithelial tumor in a child in the absence of therapy. J Pediatr Hematol Oncol. Epub ahead of print, 2013
                                                                                      17. Daghistani R et al: Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 55(2):217-24, 2013
                                                                                      18. Englot DJ et al: Factors associated with seizure freedom in the surgical resection of glioneuronal tumors. Epilepsia. 53(1):51-7, 2012
                                                                                      19. Kim SK et al: Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. Korean J Radiol. 13(4):492-5, 2012
                                                                                      20. Bird-Lieberman G et al: Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. J Neurosurg Pediatr. 7(4):416-20, 2011
                                                                                      21. Alexiou GA et al: Benign lesions accompanied by intractable epilepsy in children. J Child Neurol. 24(6):697-700, 2009
                                                                                      22. Bilginer B et al: Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology. Childs Nerv Syst. 25(4):485-91, 2009
                                                                                      23. Lee J et al: Dysembryoplastic neuroepithelial tumors in pediatric patients. Brain Dev. 31(9):671-81, 2009
                                                                                      24. Ray WZ et al: Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. J Neurooncol. 94(2):283-92, 2009
                                                                                      25. Rutka JT: Dysembryoplastic neuroepithelial tumor. J Neurosurg Pediatr. 3(6):455, 2009
                                                                                      26. Bulakbasi N et al: Dysembryoplastic neuroepithelial tumors: proton MR spectroscopy, diffusion and perfusion characteristics. Neuroradiology. 49(10):805-12, 2007
                                                                                      27. Parmar HA et al: Fluid-attenuated inversion recovery ring sign as a marker of dysembryoplastic neuroepithelial tumors. J Comput Assist Tomogr. 31(3):348-53, 2007
                                                                                      28. Maehara T et al: Usefulness of [11C]methionine PET in the diagnosis of dysembryoplastic neuroepithelial tumor with temporal lobe epilepsy. Epilepsia. 45(1):41-5, 2004
                                                                                      29. Vuori K et al: Low-grade gliomas and focal cortical developmental malformations: differentiation with proton MR spectroscopy. Radiology. 230(3):703-8, 2004
                                                                                      30. Fernandez C et al: The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol. 24(5):829-34, 2003
                                                                                      31. Adamek D et al: Dysembryoplastic neuroepithelial tumour (DNT). Is the mechanism of seizures related to glutamate? An immunohistochemical study. Folia Neuropathol. 39(2):111-7, 2001
                                                                                      32. Stanescu Cosson R et al: Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow-up: a study of 53 cases. J Neuroradiol. 28(4):230-40, 2001
                                                                                      33. Lee DY et al: Dysembryoplastic neuroepithelial tumor: radiological findings (including PET, SPECT, and MRS) and surgical strategy. J Neurooncol. 47(2):167-74, 2000
                                                                                      34. Honavar M et al: Histological heterogeneity of dysembryoplastic neuroepithelial tumour: identification and differential diagnosis in a series of 74 cases. Histopathology. 34(4):342-56, 1999
                                                                                      35. Thom M et al: Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases. J Neurol Neurosurg Psychiatry. 67(1):97-101, 1999
                                                                                      36. Ostertun B et al: Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J Neuroradiol. 17(3):419-30, 1996