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Gliomatosis Cerebri
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Gliomatosis cerebri (GC)
            • Synonyms

              • Gliomatosis, diffuse cerebral gliomatosis
            • Definitions

              • Diffusely infiltrating, frequently bilateral glial tumor involving at least 3 lobes
              • Infiltrative extent of tumor is out of proportion to histologic and clinical features

            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. Bertero L et al: Nocardia abscesses mimicking tumor progression in gliomatosis cerebri responding to temozolomide. J Neurooncol. 121(1):225-7, 2015
                                            2. Jansen NL et al: Prognostic significance of dynamic 18F-FET PET in newly diagnosed astrocytic high-grade glioma. J Nucl Med. 56(1):9-15, 2015
                                            3. Jung TY et al: Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience. Clin Neurol Neurosurg. 130:1-5, 2015
                                            4. Fernandez-Vega I et al: Gliomatosis cerebri type 1 with extensive involvement of the spinal cord and BRAF V600E mutation. Pathol Oncol Res. 20(1):215-20, 2014
                                            5. Kandula S et al: Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy. Cancer. 120(17):2713-20, 2014
                                            6. McCusker MW et al: Gliomatosis cerebri mimicking viral encephalitis in a 4-year-old child. BMJ Case Rep. 2014, 2014
                                            7. Nakahara A et al: "Gliomatosis encephali" as a novel category of brain tumors by the first autopsy case report of gliomatosis cerebelli. Neuropathology. 34(3):295-303, 2014
                                            8. Navarria P et al: Investigation on the role of integrated PET/MRI for target volume definition and radiotherapy planning in patients with high grade glioma. Radiother Oncol. 112(3):425-9, 2014
                                            9. Chappé C et al: Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases. Childs Nerv Syst. 29(4):565-71, 2013
                                            10. Chen S et al: Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol. 112(2):267-75, 2013
                                            11. Kwon MJ et al: Mutated IDH1 is a favorable prognostic factor for type 2 gliomatosis cerebri. Brain Pathol. 22(3):307-17, 2012
                                            12. Mattox AK et al: Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy. Clin Neurol Neurosurg. 114(4):299-306, 2012
                                            13. Park S et al: Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. Clin Neuropathol. 28(2):73-82, 2009
                                            14. Glas M et al: Procarbazine and CCNU as initial treatment in gliomatosis cerebri. Oncology. 75(3-4):182-5, 2008
                                            15. Kaloshi G et al: Genetic markers predictive of chemosensitivity and outcome in gliomatosis cerebri. Neurology. 70(8):590-5, 2008
                                            16. Pal L et al: Gliomatosis cerebri--an uncommon neuroepithelial tumor in children with oligodendroglial phenotype. Pediatr Neurosurg. 44(3):212-5, 2008
                                            17. Romeike BF et al: Gliomatosis cerebri: growing evidence for diffuse gliomas with wide invasion. Expert Rev Neurother. 8(4):587-97, 2008
                                            18. Rorke-Adams LB et al: Long-term survival of an infant with gliomatosis cerebelli. J Neurosurg Pediatr. 2(5):346-50, 2008
                                            19. Braeuninger S et al: Evaluation of molecular genetic alterations associated with tumor progression in a case of gliomatosis cerebri. J Neurooncol. 82(1):23-7, 2007
                                            20. Fuller GN et al: WHO Classification of Tumours of the Central Nervous System: Gliomatosis cerebri. Lyon: IARC Press. 50-2, 2007
                                            21. Ghostine S et al: Gliomatosis cerebri mimicking Rasmussen encephalitis. Case report. J Neurosurg. 107(2 Suppl):143-6, 2007
                                            22. Guzmán-de-Villoria JA et al: 1H MR spectroscopy in the assessment of gliomatosis cerebri. AJR Am J Roentgenol. 188(3):710-4, 2007
                                            23. Ware ML et al: Genetic aberrations in gliomatosis cerebri. Neurosurgery. 60(1):150-8; discussion 158, 2007
                                            24. Armstrong GT et al: Gliomatosis cerebri: 20 years of experience at the Children's Hospital of Philadelphia. Cancer. 107(7):1597-606, 2006
                                            25. Kararizou E et al: Magnetic resonance spectroscopy: a noninvasive diagnosis of gliomatosis cerebri. Magn Reson Imaging. 24(2):205-7, 2006
                                            26. Maramattom BV et al: Gliomatosis cerebri angiographically mimicking central nervous system angiitis: case report. Neurosurgery. 58(6):E1209; discussion E1209, 2006
                                            27. Piccirilli M et al: Gliomatosis cerebri treatment in 11 elderly patients. J Exp Clin Cancer Res. 25(2):183-7, 2006
                                            28. Slee M et al: Parkinsonism and dementia due to gliomatosis cerebri mimicking sporadic Creutzfeldt-Jakob disease (CJD). J Neurol Neurosurg Psychiatry. 77(2):283-4, 2006
                                            29. Taillibert S et al: Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature. J Neurooncol. 76(2):201-5, 2006
                                            30. Yu A et al: Value of diagnosis and differential diagnosis of MRI and MR spectroscopy in gliomatosis cerebri. Eur J Radiol. 59(2):216-21, 2006
                                            31. Mawrin C et al: Alterations of cell cycle regulators in gliomatosis cerebri. J Neurooncol. 72(2):115-22, 2005
                                            32. Vates GE et al: Gliomatosis cerebri: a review of 22 cases. Neurosurgery. 53(2): 261-71; discussion 271, 2003
                                            33. Meligonis G et al: Gliomatosis of the brain and spinal cord masquerading as infective lesions. Surg Neurol. 57(6):399-404; discussion 404, 2002
                                            34. Peretti-Viton P et al: Histological and MR correlations in Gliomatosis cerebri. J Neurooncol. 59(3): 249-59, 2002
                                            35. Yang S et al: Dynamic contrast-enhanced T2*-weighted MR imaging of gliomatosis cerebri. AJNR Am J Neuroradiol. 23(3): 350-5, 2002
                                            36. Rust P et al: Gliomatosis cerebri: pitfalls in diagnosis. J Clin Neurosci. 8(4): 361-3, 2001
                                            37. Bendszus M et al: MR spectroscopy in gliomatosis cerebri. AJNR Am J Neuroradiol. 21(2): 375-80, 2000
                                            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

                                                        • Gliomatosis cerebri (GC)
                                                      • Synonyms

                                                        • Gliomatosis, diffuse cerebral gliomatosis
                                                      • Definitions

                                                        • Diffusely infiltrating, frequently bilateral glial tumor involving at least 3 lobes
                                                        • Infiltrative extent of tumor is out of proportion to histologic and clinical features

                                                      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. Bertero L et al: Nocardia abscesses mimicking tumor progression in gliomatosis cerebri responding to temozolomide. J Neurooncol. 121(1):225-7, 2015
                                                                                      2. Jansen NL et al: Prognostic significance of dynamic 18F-FET PET in newly diagnosed astrocytic high-grade glioma. J Nucl Med. 56(1):9-15, 2015
                                                                                      3. Jung TY et al: Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience. Clin Neurol Neurosurg. 130:1-5, 2015
                                                                                      4. Fernandez-Vega I et al: Gliomatosis cerebri type 1 with extensive involvement of the spinal cord and BRAF V600E mutation. Pathol Oncol Res. 20(1):215-20, 2014
                                                                                      5. Kandula S et al: Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy. Cancer. 120(17):2713-20, 2014
                                                                                      6. McCusker MW et al: Gliomatosis cerebri mimicking viral encephalitis in a 4-year-old child. BMJ Case Rep. 2014, 2014
                                                                                      7. Nakahara A et al: "Gliomatosis encephali" as a novel category of brain tumors by the first autopsy case report of gliomatosis cerebelli. Neuropathology. 34(3):295-303, 2014
                                                                                      8. Navarria P et al: Investigation on the role of integrated PET/MRI for target volume definition and radiotherapy planning in patients with high grade glioma. Radiother Oncol. 112(3):425-9, 2014
                                                                                      9. Chappé C et al: Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases. Childs Nerv Syst. 29(4):565-71, 2013
                                                                                      10. Chen S et al: Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol. 112(2):267-75, 2013
                                                                                      11. Kwon MJ et al: Mutated IDH1 is a favorable prognostic factor for type 2 gliomatosis cerebri. Brain Pathol. 22(3):307-17, 2012
                                                                                      12. Mattox AK et al: Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy. Clin Neurol Neurosurg. 114(4):299-306, 2012
                                                                                      13. Park S et al: Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. Clin Neuropathol. 28(2):73-82, 2009
                                                                                      14. Glas M et al: Procarbazine and CCNU as initial treatment in gliomatosis cerebri. Oncology. 75(3-4):182-5, 2008
                                                                                      15. Kaloshi G et al: Genetic markers predictive of chemosensitivity and outcome in gliomatosis cerebri. Neurology. 70(8):590-5, 2008
                                                                                      16. Pal L et al: Gliomatosis cerebri--an uncommon neuroepithelial tumor in children with oligodendroglial phenotype. Pediatr Neurosurg. 44(3):212-5, 2008
                                                                                      17. Romeike BF et al: Gliomatosis cerebri: growing evidence for diffuse gliomas with wide invasion. Expert Rev Neurother. 8(4):587-97, 2008
                                                                                      18. Rorke-Adams LB et al: Long-term survival of an infant with gliomatosis cerebelli. J Neurosurg Pediatr. 2(5):346-50, 2008
                                                                                      19. Braeuninger S et al: Evaluation of molecular genetic alterations associated with tumor progression in a case of gliomatosis cerebri. J Neurooncol. 82(1):23-7, 2007
                                                                                      20. Fuller GN et al: WHO Classification of Tumours of the Central Nervous System: Gliomatosis cerebri. Lyon: IARC Press. 50-2, 2007
                                                                                      21. Ghostine S et al: Gliomatosis cerebri mimicking Rasmussen encephalitis. Case report. J Neurosurg. 107(2 Suppl):143-6, 2007
                                                                                      22. Guzmán-de-Villoria JA et al: 1H MR spectroscopy in the assessment of gliomatosis cerebri. AJR Am J Roentgenol. 188(3):710-4, 2007
                                                                                      23. Ware ML et al: Genetic aberrations in gliomatosis cerebri. Neurosurgery. 60(1):150-8; discussion 158, 2007
                                                                                      24. Armstrong GT et al: Gliomatosis cerebri: 20 years of experience at the Children's Hospital of Philadelphia. Cancer. 107(7):1597-606, 2006
                                                                                      25. Kararizou E et al: Magnetic resonance spectroscopy: a noninvasive diagnosis of gliomatosis cerebri. Magn Reson Imaging. 24(2):205-7, 2006
                                                                                      26. Maramattom BV et al: Gliomatosis cerebri angiographically mimicking central nervous system angiitis: case report. Neurosurgery. 58(6):E1209; discussion E1209, 2006
                                                                                      27. Piccirilli M et al: Gliomatosis cerebri treatment in 11 elderly patients. J Exp Clin Cancer Res. 25(2):183-7, 2006
                                                                                      28. Slee M et al: Parkinsonism and dementia due to gliomatosis cerebri mimicking sporadic Creutzfeldt-Jakob disease (CJD). J Neurol Neurosurg Psychiatry. 77(2):283-4, 2006
                                                                                      29. Taillibert S et al: Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature. J Neurooncol. 76(2):201-5, 2006
                                                                                      30. Yu A et al: Value of diagnosis and differential diagnosis of MRI and MR spectroscopy in gliomatosis cerebri. Eur J Radiol. 59(2):216-21, 2006
                                                                                      31. Mawrin C et al: Alterations of cell cycle regulators in gliomatosis cerebri. J Neurooncol. 72(2):115-22, 2005
                                                                                      32. Vates GE et al: Gliomatosis cerebri: a review of 22 cases. Neurosurgery. 53(2): 261-71; discussion 271, 2003
                                                                                      33. Meligonis G et al: Gliomatosis of the brain and spinal cord masquerading as infective lesions. Surg Neurol. 57(6):399-404; discussion 404, 2002
                                                                                      34. Peretti-Viton P et al: Histological and MR correlations in Gliomatosis cerebri. J Neurooncol. 59(3): 249-59, 2002
                                                                                      35. Yang S et al: Dynamic contrast-enhanced T2*-weighted MR imaging of gliomatosis cerebri. AJNR Am J Neuroradiol. 23(3): 350-5, 2002
                                                                                      36. Rust P et al: Gliomatosis cerebri: pitfalls in diagnosis. J Clin Neurosci. 8(4): 361-3, 2001
                                                                                      37. Bendszus M et al: MR spectroscopy in gliomatosis cerebri. AJNR Am J Neuroradiol. 21(2): 375-80, 2000