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Desmoplastic Infantile Tumors
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

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

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

              • Large WHO grade I cystic tumors of infants involving superficial cerebral cortex & 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. van Tilburg CM et al: Response in a child with a BRAF V600E mutated desmoplastic infantile astrocytoma upon retreatment with vemurafenib. Pediatr Blood Cancer. 65(3), 2018
                                              2. Wang AC et al: Desmoplastic infantile ganglioglioma/astrocytoma (DIG/DIA) are distinct entities with frequent BRAFV600 mutations. Mol Cancer Res. 16(10):1491-8, 2018
                                              3. Bianchi F et al: Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. Childs Nerv Syst. 32(10):1833-8, 2016
                                              4. 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
                                              5. Jurkiewicz E et al: MR imaging, apparent diffusion coefficient and histopathological features of desmoplastic infantile tumors-own experience and review of the literature. Childs Nerv Syst. 31(2):251-9, 2015
                                              6. Koelsche C et al: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol. 40(3):337-44, 2014
                                              7. Kralik SF et al: Diffusion imaging for tumor grading of supratentorial brain tumors in the first year of life. AJNR Am J Neuroradiol. 35(4):815-23, 2014
                                              8. 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
                                              9. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                              10. Hummel TR et al: Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. J Pediatr Hematol Oncol. 34(6):e232-6, 2012
                                              11. Phi JH et al: Desmoplastic infantile astrocytoma: recurrence with malignant transformation into glioblastoma: a case report. Childs Nerv Syst. 27(12):2177-81, 2011
                                              12. Gelabert-Gonzalez M et al: Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev. 34(2):151-8, 2010
                                              13. Balaji R et al: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst. 25(4):497-501, 2009
                                              14. Hoving EW et al: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr. 1(1):95-8, 2008
                                              15. Darwish B et al: Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci. 14(5):498-501, 2007
                                              16. Lönnrot K et al: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol. 68(3):304-8; discussion 308, 2007
                                              17. Cerdá-Nicolás M et al: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology. 48(5):617-21, 2006
                                              18. 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
                                              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)
                                                          • Desmoplastic infantile astrocytoma (DIA)
                                                        • Synonyms

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

                                                          • Large WHO grade I cystic tumors of infants involving superficial cerebral cortex & 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. van Tilburg CM et al: Response in a child with a BRAF V600E mutated desmoplastic infantile astrocytoma upon retreatment with vemurafenib. Pediatr Blood Cancer. 65(3), 2018
                                                                                          2. Wang AC et al: Desmoplastic infantile ganglioglioma/astrocytoma (DIG/DIA) are distinct entities with frequent BRAFV600 mutations. Mol Cancer Res. 16(10):1491-8, 2018
                                                                                          3. Bianchi F et al: Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. Childs Nerv Syst. 32(10):1833-8, 2016
                                                                                          4. 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
                                                                                          5. Jurkiewicz E et al: MR imaging, apparent diffusion coefficient and histopathological features of desmoplastic infantile tumors-own experience and review of the literature. Childs Nerv Syst. 31(2):251-9, 2015
                                                                                          6. Koelsche C et al: BRAF V600E expression and distribution in desmoplastic infantile astrocytoma/ganglioglioma. Neuropathol Appl Neurobiol. 40(3):337-44, 2014
                                                                                          7. Kralik SF et al: Diffusion imaging for tumor grading of supratentorial brain tumors in the first year of life. AJNR Am J Neuroradiol. 35(4):815-23, 2014
                                                                                          8. 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
                                                                                          9. Romero-Rojas AE et al: Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. Neuroradiol J. 26(6):649-54, 2013
                                                                                          10. Hummel TR et al: Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. J Pediatr Hematol Oncol. 34(6):e232-6, 2012
                                                                                          11. Phi JH et al: Desmoplastic infantile astrocytoma: recurrence with malignant transformation into glioblastoma: a case report. Childs Nerv Syst. 27(12):2177-81, 2011
                                                                                          12. Gelabert-Gonzalez M et al: Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev. 34(2):151-8, 2010
                                                                                          13. Balaji R et al: Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. Childs Nerv Syst. 25(4):497-501, 2009
                                                                                          14. Hoving EW et al: Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatr. 1(1):95-8, 2008
                                                                                          15. Darwish B et al: Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. J Clin Neurosci. 14(5):498-501, 2007
                                                                                          16. Lönnrot K et al: Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. Surg Neurol. 68(3):304-8; discussion 308, 2007
                                                                                          17. Cerdá-Nicolás M et al: Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. Histopathology. 48(5):617-21, 2006
                                                                                          18. 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