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Ganglioglioma
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Ganglioglioma (GG)
            • Definitions

              • Well-differentiated, slowly growing neuroepithelial tumor composed of neoplastic ganglion cells and neoplastic glial cells
              • Most common neoplasm to cause temporal lobe epilepsy (TLE)

            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. Abdel Razek AAK et al: Imaging of neuronal and mixed glioneuronal tumors. J Comput Assist Tomogr. 44(3):356-69, 2020
                                            2. Fukuoka K et al: Clinical impact of combined epigenetic and molecular analysis of pediatric low grade gliomas. Neuro Oncol. ePub, 2020
                                            3. Ramaglia A et al: Role of diffusion weighted imaging for differentiating cerebral pilocytic astrocytoma and ganglioglioma BRAF V600E-mutant from wild type. Neuroradiology. 62(1):71-80, 2020
                                            4. Yahanda AT et al: A multi-institutional analysis of factors influencing surgical outcomes for patients with newly diagnosed grade I gliomas. World Neurosurg. 135:e754-64, 2020
                                            5. 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
                                            6. AlRayahi J et al: Pediatric brain tumor genetics: what radiologists need to know. Radiographics. 38(7):2102-22, 2018
                                            7. Zaky W et al: Ganglioglioma in children and young adults: single institution experience and review of the literature. J Neurooncol.139(3):739-47, 2018
                                            8. Al-Hajri A et al: Pathology-MRI correlations in diffuse low-grade epilepsy associated tumors. J Neuropathol Exp Neurol. 76(12):1023-33, 2017
                                            9. Louis DN et al: Ganglioma and gangliocytoma. In: WHO Classification of Tumours of the Central Nervous System. IARC Press. 138-41, 2016
                                            10. Dudley RW et al: Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. Neurosurgery. 76(3):313-9; discussion 319; quiz 319-20, 2015
                                            11. Lucas JT Jr et al: Anaplastic ganglioglioma: a report of three cases and review of the literature. J Neurooncol. 123(1):171-7, 2015
                                            12. Yang C et al: Intramedullary gangliogliomas: clinical features, surgical outcomes, and neuropathic scoliosis. J Neurooncol. 116(1):135-43, 2014
                                            13. Consales A et al: Glioneuronal tumors and epilepsy in children: seizure outcome related to lesionectomy. Minerva Pediatr. 65(6):609-16, 2013
                                            14. Deling L et al: Intraventricular ganglioglioma prognosis and hydrocephalus: the largest case series and systematic literature review. Acta Neurochir (Wien). 155(7):1253-60, 2013
                                            15. Gopalakrishnan CV et al: Brainstem ganglioglioma in an infant: case report and review of literature. J Pediatr Neurosci. 8(1):41-5, 2013
                                            16. Gundamaneni SK et al: Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association. Pediatr Neurosurg. 49(2):105-9, 2013
                                            17. Lim KC et al: Focal malformations of cortical development: new vistas for molecular pathogenesis. Neuroscience. 252:262-76, 2013
                                            18. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                            19. Caseiras GB et al: Relative cerebral blood volume measurements of low-grade gliomas predict patient outcome in a multi-institution setting. Eur J Radiol. 73(2):215-20, 2010
                                            20. Alexiou GA et al: Benign lesions accompanied by intractable epilepsy in children. J Child Neurol. 24(6):697-700, 2009
                                            21. Allende DS et al: The expanding family of glioneuronal tumors. Adv Anat Pathol. 16(1):33-9, 2009
                                            22. Karremann M et al: Anaplastic ganglioglioma in children. J Neurooncol. 92(2):157-63, 2009
                                            23. Lotfinia I et al: 'Intramedullary cervical spinal cord ganglioglioma, review of the literature and therapeutic controversies'. Spinal Cord. 47(1):87-90, 2009
                                            24. Per H et al: A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. Neurosurg Rev. 32(3):369-74; discussion 374, 2009
                                            25. Samdani AF et al: Intraventricular ganglioglioma: a short illustrated review. Acta Neurochir (Wien). 151(6):635-40, 2009
                                            26. Westwood DA et al: Pontomedullary ganglioglioma: a rare tumour in an unusual location. J Clin Neurosci. 16(1):108-10, 2009
                                            27. Adachi Y et al: Gangliogliomas: characteristic imaging findings and role in the temporal lobe epilepsy. Neuroradiology. 50(10):829-34, 2008
                                            28. Brat DJ et al: Surgical neuropathology update: a review of changes introduced by the WHO classification of tumours of the central nervous system, 4th edition. Arch Pathol Lab Med. 132(6):993-1007, 2008
                                            29. De Tommasi A et al: Molecular genetic analysis in a case of ganglioglioma: identification of a new mutation. Neurosurgery. 63(5):976-80; discussion 980, 2008
                                            30. Hauck EF et al: Intraventricular ganglioglioma. J Clin Neurosci. 15(11):1291-3, 2008
                                            31. Hoischen A et al: Comprehensive characterization of genomic aberrations in gangliogliomas by CGH, array-based CGH and interphase FISH. Brain Pathol. 18(3):326-37, 2008
                                            32. Jalali R et al: Suprasellar ganglioglioma with unusual diffuse involvement of the entire optico-chiasmal hypothalamic pathway. J Cancer Res Ther. 4(3):140-3, 2008
                                            33. Majores M et al: Tumor recurrence and malignant progression of gangliogliomas. Cancer. 113(12):3355-63, 2008
                                            34. Mpairamidis E et al: Brainstem ganglioglioma. J Child Neurol. 23(12):1481-3, 2008
                                            35. Park SH et al: Cerebellar ganglioglioma. J Korean Neurosurg Soc. 43(3):165-8, 2008
                                            36. Park YS et al: Factors contributing to resectability and seizure outcomes in 44 patients with ganglioglioma. Clin Neurol Neurosurg. 110(7):667-73, 2008
                                            37. Scheithauer BW et al: Ganglioglioma of the neurohypophysis. Endocr Pathol. 19(2):112-6, 2008
                                            38. Urbach H: MRI of long-term epilepsy-associated tumors. Semin Ultrasound CT MR. 29(1):40-6, 2008
                                            39. Aryan HE et al: Hypothalamic ganglioglioma treated by temporal lobectomy. Case report and review of the literature. J Neurosurg. 100(2 Suppl Pediatrics):217-9, 2004
                                            40. Clusmann H et al: Prognostic factors and outcome after different types of resection for temporal lobe epilepsy. J Neurosurg. 97(5):1131-41, 2002
                                            41. Im SH et al: Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. J Neurooncol. 57(1):59-66, 2002
                                            42. The brain: tumor. In Burger PC et al: Surgical pathology of the nervous system and its coverings. 4th ed. Churchill Livingstone. 250-4, 2002
                                            43. Hayashi Y et al: Malignant transformation of a gangliocytoma/ganglioglioma into a glioblastoma multiforme: a molecular genetic analysis. Case report. J Neurosurg. 95(1):138-42, 2001
                                            44. Koeller KK et al: From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 21(6):1533-56, 2001
                                            45. Kwon JW et al: Cerebellopontine angle ganglioglioma: MR findings. AJNR Am J Neuroradiol. 22(7):1377-9, 2001
                                            46. Meyer PT et al: High F-18 FDG uptake in a low-grade supratentorial ganglioma: a positron emission tomography case report. Clin Nucl Med. 25(9):694-7, 2000
                                            47. Provenzale JM et al: Comparison of patient age with MR imaging features of gangliogliomas. AJR Am J Roentgenol. 174(3):859-62, 2000
                                            48. Tamiya T et al: Ganglioglioma in a patient with Turcot syndrome. Case report. J Neurosurg. 92(1):170-5, 2000
                                            49. Kumabe T et al: Thallium-201 single-photon emission computed tomographic and proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports. Neurosurgery. 45(1):183-7; discussion 187, 1999
                                            50. Provenzale JM et al: Gangliogliomas: characterization by registered positron emission tomography-MR images. AJR Am J Roentgenol. 172(4):1103-7, 1999
                                            51. Kincaid PK et al: Cerebral gangliogliomas: preoperative grading using FDG-PET and 201Tl-SPECT. AJNR Am J Neuroradiol. 19(5):801-6, 1998
                                            52. Kurian NI et al: Anaplastic ganglioglioma: case report and review of the literature. Br J Neurosurg. 12(3):277-80, 1998
                                            53. Nakajima M et al: Anaplastic ganglioglioma with dissemination to the spinal cord: a case report. Surg Neurol. 49(4):445-8, 1998
                                            54. Selch MT et al: Gangliogliomas: experience with 34 patients and review of the literature. Am J Clin Oncol. 21(6):557-64, 1998
                                            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

                                                        • Ganglioglioma (GG)
                                                      • Definitions

                                                        • Well-differentiated, slowly growing neuroepithelial tumor composed of neoplastic ganglion cells and neoplastic glial cells
                                                        • Most common neoplasm to cause temporal lobe epilepsy (TLE)

                                                      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. Abdel Razek AAK et al: Imaging of neuronal and mixed glioneuronal tumors. J Comput Assist Tomogr. 44(3):356-69, 2020
                                                                                      2. Fukuoka K et al: Clinical impact of combined epigenetic and molecular analysis of pediatric low grade gliomas. Neuro Oncol. ePub, 2020
                                                                                      3. Ramaglia A et al: Role of diffusion weighted imaging for differentiating cerebral pilocytic astrocytoma and ganglioglioma BRAF V600E-mutant from wild type. Neuroradiology. 62(1):71-80, 2020
                                                                                      4. Yahanda AT et al: A multi-institutional analysis of factors influencing surgical outcomes for patients with newly diagnosed grade I gliomas. World Neurosurg. 135:e754-64, 2020
                                                                                      5. 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
                                                                                      6. AlRayahi J et al: Pediatric brain tumor genetics: what radiologists need to know. Radiographics. 38(7):2102-22, 2018
                                                                                      7. Zaky W et al: Ganglioglioma in children and young adults: single institution experience and review of the literature. J Neurooncol.139(3):739-47, 2018
                                                                                      8. Al-Hajri A et al: Pathology-MRI correlations in diffuse low-grade epilepsy associated tumors. J Neuropathol Exp Neurol. 76(12):1023-33, 2017
                                                                                      9. Louis DN et al: Ganglioma and gangliocytoma. In: WHO Classification of Tumours of the Central Nervous System. IARC Press. 138-41, 2016
                                                                                      10. Dudley RW et al: Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. Neurosurgery. 76(3):313-9; discussion 319; quiz 319-20, 2015
                                                                                      11. Lucas JT Jr et al: Anaplastic ganglioglioma: a report of three cases and review of the literature. J Neurooncol. 123(1):171-7, 2015
                                                                                      12. Yang C et al: Intramedullary gangliogliomas: clinical features, surgical outcomes, and neuropathic scoliosis. J Neurooncol. 116(1):135-43, 2014
                                                                                      13. Consales A et al: Glioneuronal tumors and epilepsy in children: seizure outcome related to lesionectomy. Minerva Pediatr. 65(6):609-16, 2013
                                                                                      14. Deling L et al: Intraventricular ganglioglioma prognosis and hydrocephalus: the largest case series and systematic literature review. Acta Neurochir (Wien). 155(7):1253-60, 2013
                                                                                      15. Gopalakrishnan CV et al: Brainstem ganglioglioma in an infant: case report and review of literature. J Pediatr Neurosci. 8(1):41-5, 2013
                                                                                      16. Gundamaneni SK et al: Non-infantile desmoplastic cerebellar ganglioglioma in a patient with multiple congenital anomalies: a rare association. Pediatr Neurosurg. 49(2):105-9, 2013
                                                                                      17. Lim KC et al: Focal malformations of cortical development: new vistas for molecular pathogenesis. Neuroscience. 252:262-76, 2013
                                                                                      18. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                                                                      19. Caseiras GB et al: Relative cerebral blood volume measurements of low-grade gliomas predict patient outcome in a multi-institution setting. Eur J Radiol. 73(2):215-20, 2010
                                                                                      20. Alexiou GA et al: Benign lesions accompanied by intractable epilepsy in children. J Child Neurol. 24(6):697-700, 2009
                                                                                      21. Allende DS et al: The expanding family of glioneuronal tumors. Adv Anat Pathol. 16(1):33-9, 2009
                                                                                      22. Karremann M et al: Anaplastic ganglioglioma in children. J Neurooncol. 92(2):157-63, 2009
                                                                                      23. Lotfinia I et al: 'Intramedullary cervical spinal cord ganglioglioma, review of the literature and therapeutic controversies'. Spinal Cord. 47(1):87-90, 2009
                                                                                      24. Per H et al: A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. Neurosurg Rev. 32(3):369-74; discussion 374, 2009
                                                                                      25. Samdani AF et al: Intraventricular ganglioglioma: a short illustrated review. Acta Neurochir (Wien). 151(6):635-40, 2009
                                                                                      26. Westwood DA et al: Pontomedullary ganglioglioma: a rare tumour in an unusual location. J Clin Neurosci. 16(1):108-10, 2009
                                                                                      27. Adachi Y et al: Gangliogliomas: characteristic imaging findings and role in the temporal lobe epilepsy. Neuroradiology. 50(10):829-34, 2008
                                                                                      28. Brat DJ et al: Surgical neuropathology update: a review of changes introduced by the WHO classification of tumours of the central nervous system, 4th edition. Arch Pathol Lab Med. 132(6):993-1007, 2008
                                                                                      29. De Tommasi A et al: Molecular genetic analysis in a case of ganglioglioma: identification of a new mutation. Neurosurgery. 63(5):976-80; discussion 980, 2008
                                                                                      30. Hauck EF et al: Intraventricular ganglioglioma. J Clin Neurosci. 15(11):1291-3, 2008
                                                                                      31. Hoischen A et al: Comprehensive characterization of genomic aberrations in gangliogliomas by CGH, array-based CGH and interphase FISH. Brain Pathol. 18(3):326-37, 2008
                                                                                      32. Jalali R et al: Suprasellar ganglioglioma with unusual diffuse involvement of the entire optico-chiasmal hypothalamic pathway. J Cancer Res Ther. 4(3):140-3, 2008
                                                                                      33. Majores M et al: Tumor recurrence and malignant progression of gangliogliomas. Cancer. 113(12):3355-63, 2008
                                                                                      34. Mpairamidis E et al: Brainstem ganglioglioma. J Child Neurol. 23(12):1481-3, 2008
                                                                                      35. Park SH et al: Cerebellar ganglioglioma. J Korean Neurosurg Soc. 43(3):165-8, 2008
                                                                                      36. Park YS et al: Factors contributing to resectability and seizure outcomes in 44 patients with ganglioglioma. Clin Neurol Neurosurg. 110(7):667-73, 2008
                                                                                      37. Scheithauer BW et al: Ganglioglioma of the neurohypophysis. Endocr Pathol. 19(2):112-6, 2008
                                                                                      38. Urbach H: MRI of long-term epilepsy-associated tumors. Semin Ultrasound CT MR. 29(1):40-6, 2008
                                                                                      39. Aryan HE et al: Hypothalamic ganglioglioma treated by temporal lobectomy. Case report and review of the literature. J Neurosurg. 100(2 Suppl Pediatrics):217-9, 2004
                                                                                      40. Clusmann H et al: Prognostic factors and outcome after different types of resection for temporal lobe epilepsy. J Neurosurg. 97(5):1131-41, 2002
                                                                                      41. Im SH et al: Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. J Neurooncol. 57(1):59-66, 2002
                                                                                      42. The brain: tumor. In Burger PC et al: Surgical pathology of the nervous system and its coverings. 4th ed. Churchill Livingstone. 250-4, 2002
                                                                                      43. Hayashi Y et al: Malignant transformation of a gangliocytoma/ganglioglioma into a glioblastoma multiforme: a molecular genetic analysis. Case report. J Neurosurg. 95(1):138-42, 2001
                                                                                      44. Koeller KK et al: From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 21(6):1533-56, 2001
                                                                                      45. Kwon JW et al: Cerebellopontine angle ganglioglioma: MR findings. AJNR Am J Neuroradiol. 22(7):1377-9, 2001
                                                                                      46. Meyer PT et al: High F-18 FDG uptake in a low-grade supratentorial ganglioma: a positron emission tomography case report. Clin Nucl Med. 25(9):694-7, 2000
                                                                                      47. Provenzale JM et al: Comparison of patient age with MR imaging features of gangliogliomas. AJR Am J Roentgenol. 174(3):859-62, 2000
                                                                                      48. Tamiya T et al: Ganglioglioma in a patient with Turcot syndrome. Case report. J Neurosurg. 92(1):170-5, 2000
                                                                                      49. Kumabe T et al: Thallium-201 single-photon emission computed tomographic and proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports. Neurosurgery. 45(1):183-7; discussion 187, 1999
                                                                                      50. Provenzale JM et al: Gangliogliomas: characterization by registered positron emission tomography-MR images. AJR Am J Roentgenol. 172(4):1103-7, 1999
                                                                                      51. Kincaid PK et al: Cerebral gangliogliomas: preoperative grading using FDG-PET and 201Tl-SPECT. AJNR Am J Neuroradiol. 19(5):801-6, 1998
                                                                                      52. Kurian NI et al: Anaplastic ganglioglioma: case report and review of the literature. Br J Neurosurg. 12(3):277-80, 1998
                                                                                      53. Nakajima M et al: Anaplastic ganglioglioma with dissemination to the spinal cord: a case report. Surg Neurol. 49(4):445-8, 1998
                                                                                      54. Selch MT et al: Gangliogliomas: experience with 34 patients and review of the literature. Am J Clin Oncol. 21(6):557-64, 1998