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Desmoplastic Infantile Astrocytoma and Ganglioglioma
Chang Yueh Ho, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Desmoplastic infantile ganglioglioma (DIG/DIGG)
              • Desmoplastic infantile astrocytoma (DIA)
            • Synonyms

              • Desmoplastic supratentorial neuroepithelial tumors of infancy
              • Superficial cerebral astrocytoma ± neuronal elements attached to dura
            • Definitions

              • Large cystic tumors of infants involving superficial cerebral cortex and leptomeninges, often attached to dura
              • DIG: Prominent desmoplastic stroma + neoplastic astrocytes, variable neuronal component
              • DIA: Desmoplastic stroma + neoplastic astrocytes

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                • Immunohistochemistry

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Bader A et al: Radiological features of infantile glioblastoma and desmoplastic infantile tumors: British Columbia's Children's Hospital experience. J Neurosurg Pediatr. 1-7, 2015
                                              2. Koelsche C et al: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol. 40(3):337-44, 2014
                                              3. Gessi M et al: Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. J Neuropathol Exp Neurol. 72(9):807-15, 2013
                                              4. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                              5. Chandrashekhar TN et al: Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute. Neuropathology. 32(1):1-12, 2012
                                              6. Schindler G et al: Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol. 121(3):397-405, 2011
                                              7. Balaji R et al: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst. 25(4):497-501, 2009
                                              8. Hoving EW et al: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr. 1(1):95-8, 2008
                                              9. Brat DJ et al: Desmoplastic infantile astrocytoma and ganglioglioma. In Louis DN et al: Tumours of the Central Nervous System. Lyon: IARC Press. 96-8, 2007
                                              10. Darwish B et al: Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci. 14(5):498-501, 2007
                                              11. Lönnrot K et al: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol. 68(3):304-8; discussion 308, 2007
                                              12. Bhardwaj M et al: Desmoplastic infantile ganglioglioma with calcification. Neuropathology. 26(4):318-22, 2006
                                              13. Cerdá-Nicolás M et al: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology. 48(5):617-21, 2006
                                              14. Bächli H et al: Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst. 19(5-6):359-66, 2003
                                              15. Tamburrini G et al: Desmoplastic infantile ganglioglioma. Childs Nerv Syst. 19(5-6):292-7, 2003
                                              16. Shin JH et al: Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics. 22(5):1177-89, 2002
                                              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

                                                          • Desmoplastic infantile ganglioglioma (DIG/DIGG)
                                                          • Desmoplastic infantile astrocytoma (DIA)
                                                        • Synonyms

                                                          • Desmoplastic supratentorial neuroepithelial tumors of infancy
                                                          • Superficial cerebral astrocytoma ± neuronal elements attached to dura
                                                        • Definitions

                                                          • Large cystic tumors of infants involving superficial cerebral cortex and leptomeninges, often attached to dura
                                                          • DIG: Prominent desmoplastic stroma + neoplastic astrocytes, variable neuronal component
                                                          • DIA: Desmoplastic stroma + neoplastic astrocytes

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            • Immunohistochemistry

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Bader A et al: Radiological features of infantile glioblastoma and desmoplastic infantile tumors: British Columbia's Children's Hospital experience. J Neurosurg Pediatr. 1-7, 2015
                                                                                          2. Koelsche C et al: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol. 40(3):337-44, 2014
                                                                                          3. Gessi M et al: Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. J Neuropathol Exp Neurol. 72(9):807-15, 2013
                                                                                          4. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                                                                          5. Chandrashekhar TN et al: Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute. Neuropathology. 32(1):1-12, 2012
                                                                                          6. Schindler G et al: Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol. 121(3):397-405, 2011
                                                                                          7. Balaji R et al: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst. 25(4):497-501, 2009
                                                                                          8. Hoving EW et al: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr. 1(1):95-8, 2008
                                                                                          9. Brat DJ et al: Desmoplastic infantile astrocytoma and ganglioglioma. In Louis DN et al: Tumours of the Central Nervous System. Lyon: IARC Press. 96-8, 2007
                                                                                          10. Darwish B et al: Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci. 14(5):498-501, 2007
                                                                                          11. Lönnrot K et al: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol. 68(3):304-8; discussion 308, 2007
                                                                                          12. Bhardwaj M et al: Desmoplastic infantile ganglioglioma with calcification. Neuropathology. 26(4):318-22, 2006
                                                                                          13. Cerdá-Nicolás M et al: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology. 48(5):617-21, 2006
                                                                                          14. Bächli H et al: Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. Childs Nerv Syst. 19(5-6):359-66, 2003
                                                                                          15. Tamburrini G et al: Desmoplastic infantile ganglioglioma. Childs Nerv Syst. 19(5-6):292-7, 2003
                                                                                          16. Shin JH et al: Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics. 22(5):1177-89, 2002