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Pediatrics
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Brain
Cysts and Neoplasms
DNET
DNET
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Dysembryoplastic neuroepithelial tumor (DNET)

            • Definitions

              • Benign, mixed glial-neuronal neoplasm
              • Epileptogenic tumor often 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. Luzzi S et al: Dysembryoplastic neuroepithelial tumors: what you need to know. World Neurosurg. 127:255-65, 2019
                                            3. Isler C et al: Dysembryoplastic neuroepithelial tumours: clinical, radiological, pathological features and outcome. Br J Neurosurg. 32(4):436-41, 2018
                                            4. Zamora C et al: Supratentorial tumors in pediatric patients. Neuroimaging Clin N Am. 27(1):39-67, 2017
                                            5. Lee D et al: BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas. J Surg Oncol. 111(3):359-64, 2015
                                            6. 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. 31(6):847-55, 2015
                                            7. Chuang NA et al: Glioblastoma multiforme arising from dysembryoplastic neuroepithelial tumor in a child in the absence of therapy. J Pediatr Hematol Oncol. 36(8):e536-9, 2014
                                            8. 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
                                            9. Daghistani R et al: Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 55(2):217-24, 2013
                                            10. Chassoux F et al: Dysembryoplastic neuroepithelial tumors: an MRI-based scheme for epilepsy surgery. Neurology. 79(16):1699-707, 2012
                                            11. Thom M et al: 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(10):859-78, 2011
                                            12. 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
                                            13. Campos AR et al: Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology. 51(7):433-43, 2009
                                            14. 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
                                            15. Bulakbasi N et al: Dysembryoplastic neuroepithelial tumors: proton MR spectroscopy, diffusion and perfusion characteristics. Neuroradiology. 49(10):805-12, 2007
                                            16. 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
                                            17. 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
                                            18. 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
                                            19. 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
                                            20. 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
                                            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)

                                                      • Definitions

                                                        • Benign, mixed glial-neuronal neoplasm
                                                        • Epileptogenic tumor often 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. Luzzi S et al: Dysembryoplastic neuroepithelial tumors: what you need to know. World Neurosurg. 127:255-65, 2019
                                                                                      3. Isler C et al: Dysembryoplastic neuroepithelial tumours: clinical, radiological, pathological features and outcome. Br J Neurosurg. 32(4):436-41, 2018
                                                                                      4. Zamora C et al: Supratentorial tumors in pediatric patients. Neuroimaging Clin N Am. 27(1):39-67, 2017
                                                                                      5. Lee D et al: BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas. J Surg Oncol. 111(3):359-64, 2015
                                                                                      6. 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. 31(6):847-55, 2015
                                                                                      7. Chuang NA et al: Glioblastoma multiforme arising from dysembryoplastic neuroepithelial tumor in a child in the absence of therapy. J Pediatr Hematol Oncol. 36(8):e536-9, 2014
                                                                                      8. 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
                                                                                      9. Daghistani R et al: Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 55(2):217-24, 2013
                                                                                      10. Chassoux F et al: Dysembryoplastic neuroepithelial tumors: an MRI-based scheme for epilepsy surgery. Neurology. 79(16):1699-707, 2012
                                                                                      11. Thom M et al: 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(10):859-78, 2011
                                                                                      12. 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
                                                                                      13. Campos AR et al: Simple and complex dysembryoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology. Neuroradiology. 51(7):433-43, 2009
                                                                                      14. 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
                                                                                      15. Bulakbasi N et al: Dysembryoplastic neuroepithelial tumors: proton MR spectroscopy, diffusion and perfusion characteristics. Neuroradiology. 49(10):805-12, 2007
                                                                                      16. 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
                                                                                      17. 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
                                                                                      18. 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
                                                                                      19. 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
                                                                                      20. 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