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Multinodular and Vacuolating Tumor of Cerebrum
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Multinodular and vacuolating neuronal tumor of cerebrum (MVNT)
          • Synonyms

            • Multinodular and vacuolated pattern of ganglion cell tumor
          • Definitions

            • Benign multinodular neuronal/mixed neuronal-glial tumor with conspicuous vacuolation
          • Etiology

            • Initially unknown whether MVNTs were neoplastic or malformative
            • Now identified as true clonal neoplasm
              • MAP kinase signaling pathway alterations (MAP2K1)
              • Missense BRAF mutations in some cases

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • Staging, Grading, & Classification

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Kapucu I et al: Multinodular and vacuolating neuronal tumor of the cerebrum: a benign nonaggressive cerebral lesion. Eur Neurol. 79(1-2):74-75, 2018
                                2. Lecler A et al: Multiparametric imaging improves confidence in the diagnosis of multinodular and vacuolating neuronal tumor of the cerebrum. AJNR Am J Neuroradiol. 39(2):E32-E33, 2018
                                3. Pekmezci M et al: Multinodular and vacuolating neuronal tumor of the cerebrum is a clonal neoplasm defined by genetic alterations that activate the MAP kinase signaling pathway. Acta Neuropathol. 135(3):485-488, 2018
                                4. Thom M et al: Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia? Brain Pathol. 28(2):155-171, 2018
                                5. Alsufayan R et al: Natural history of lesions with the MR imaging appearance of multinodular and vacuolating neuronal tumor. Neuroradiology. 59(9):873-883, 2017
                                6. Nunes RH et al: Multinodular and vacuolating neuronal tumor of the cerebrum: a new "leave me alone" lesion with a characteristic imaging pattern. AJNR Am J Neuroradiol. 38(10):1899-1904, 2017
                                7. Huse JT et al: Multinodular and vacuolating neuronal tumors of the cerebrum: 10 cases of a distinctive seizure-associated lesion. Brain Pathol. 23(5):515-24, 2013
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Clinical Issues

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Multinodular and vacuolating neuronal tumor of cerebrum (MVNT)
                                        • Synonyms

                                          • Multinodular and vacuolated pattern of ganglion cell tumor
                                        • Definitions

                                          • Benign multinodular neuronal/mixed neuronal-glial tumor with conspicuous vacuolation
                                        • Etiology

                                          • Initially unknown whether MVNTs were neoplastic or malformative
                                          • Now identified as true clonal neoplasm
                                            • MAP kinase signaling pathway alterations (MAP2K1)
                                            • Missense BRAF mutations in some cases

                                        IMAGING

                                        • General Features

                                          • CT Findings

                                            • MR Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • Staging, Grading, & Classification

                                                    • Microscopic Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Kapucu I et al: Multinodular and vacuolating neuronal tumor of the cerebrum: a benign nonaggressive cerebral lesion. Eur Neurol. 79(1-2):74-75, 2018
                                                              2. Lecler A et al: Multiparametric imaging improves confidence in the diagnosis of multinodular and vacuolating neuronal tumor of the cerebrum. AJNR Am J Neuroradiol. 39(2):E32-E33, 2018
                                                              3. Pekmezci M et al: Multinodular and vacuolating neuronal tumor of the cerebrum is a clonal neoplasm defined by genetic alterations that activate the MAP kinase signaling pathway. Acta Neuropathol. 135(3):485-488, 2018
                                                              4. Thom M et al: Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia? Brain Pathol. 28(2):155-171, 2018
                                                              5. Alsufayan R et al: Natural history of lesions with the MR imaging appearance of multinodular and vacuolating neuronal tumor. Neuroradiology. 59(9):873-883, 2017
                                                              6. Nunes RH et al: Multinodular and vacuolating neuronal tumor of the cerebrum: a new "leave me alone" lesion with a characteristic imaging pattern. AJNR Am J Neuroradiol. 38(10):1899-1904, 2017
                                                              7. Huse JT et al: Multinodular and vacuolating neuronal tumors of the cerebrum: 10 cases of a distinctive seizure-associated lesion. Brain Pathol. 23(5):515-24, 2013