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Supratentorial PNET
Anne G. Osborn, MD, FACR; Laurie A. Loevner, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • CNS supratentorial primitive neuroectodermal tumor (S-PNET)
              • Definitions

                • CNS PNET: Generic designation comprising histologically diverse group of tumors
                  • Composed of undifferentiated neuroepithelial cells
                  • Broad capacity for divergent differentiation
                    • Astrocytic, ependymal, neuronal, muscular, melanotic elements

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic 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. Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. ePub, 2015
                                                2. Korshunov A et al: Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 128(2):279-89, 2014
                                                3. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                                4. Nowak J et al: MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases. AJNR Am J Neuroradiol. 35(10):1996-2001, 2014
                                                5. Perreault S et al: Surveillance imaging in children with malignant CNS tumors: low yield of spine MRI. J Neurooncol. 116(3):617-23, 2014
                                                6. Spence T et al: CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity. Acta Neuropathol. 128(2):291-303, 2014
                                                7. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                                8. Bendersky D et al: Epilepsy due to a temporal ganglioglioma and its subsequent malignant transformation into a primitive neuroectodermal tumor. Surg Neurol Int. 3:79, 2012
                                                9. Jethwa PR et al: Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy. J Neurosurg Pediatr. 8(5):468-75, 2011
                                                10. Brandes AA et al: Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET). Crit Rev Oncol Hematol. 71(2):165-79, 2009
                                                11. Pal D et al: Primitive neuroectodermal tumour arising within low grade astrocytoma: transformation, de novo or radiation induced? Report of three cases and review of literature. Br J Neurosurg. 22(3):402-8, 2008
                                                12. Smee RI et al: Medulloblastomas-primitive neuroectodermal tumours in the adult population. J Med Imaging Radiat Oncol. 52(1):72-6, 2008
                                                13. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                                14. Murakami R et al: Malignant supratentorial astrocytoma treated with postoperative radiation therapy: prognostic value of pretreatment quantitative diffusion-weighted MR imaging. Radiology. 243(2):493-9, 2007
                                                15. Pfister S et al: Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas. Genes Chromosomes Cancer. 46(9):839-51, 2007
                                                16. Larouche V et al: Supratentorial primitive neuroectodermal tumors in young children. J Clin Oncol. 24(35):5609; author reply 5609-10, 2006
                                                17. Didiano D et al: Telomere maintenance in childhood primitive neuroectodermal brain tumors. Neuro Oncol. 6(1):1-8, 2004
                                                18. Yamada T et al: Prenatal imaging of congenital cerebral primitive neuroectodermal tumor. Fetal Diagn Ther. 18(3):137-9, 2003
                                                19. Etuş V et al: Primary cerebral neuroblastoma: a case report and review. Tohoku J Exp Med. 197(1):55-65, 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

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • CNS supratentorial primitive neuroectodermal tumor (S-PNET)
                                                            • Definitions

                                                              • CNS PNET: Generic designation comprising histologically diverse group of tumors
                                                                • Composed of undifferentiated neuroepithelial cells
                                                                • Broad capacity for divergent differentiation
                                                                  • Astrocytic, ependymal, neuronal, muscular, melanotic elements

                                                            IMAGING

                                                            • General Features

                                                              • Radiographic Findings

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Ultrasonographic 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. Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. ePub, 2015
                                                                                              2. Korshunov A et al: Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 128(2):279-89, 2014
                                                                                              3. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                                                                              4. Nowak J et al: MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases. AJNR Am J Neuroradiol. 35(10):1996-2001, 2014
                                                                                              5. Perreault S et al: Surveillance imaging in children with malignant CNS tumors: low yield of spine MRI. J Neurooncol. 116(3):617-23, 2014
                                                                                              6. Spence T et al: CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity. Acta Neuropathol. 128(2):291-303, 2014
                                                                                              7. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                                                                              8. Bendersky D et al: Epilepsy due to a temporal ganglioglioma and its subsequent malignant transformation into a primitive neuroectodermal tumor. Surg Neurol Int. 3:79, 2012
                                                                                              9. Jethwa PR et al: Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy. J Neurosurg Pediatr. 8(5):468-75, 2011
                                                                                              10. Brandes AA et al: Adult neuroectodermal tumors of posterior fossa (medulloblastoma) and of supratentorial sites (stPNET). Crit Rev Oncol Hematol. 71(2):165-79, 2009
                                                                                              11. Pal D et al: Primitive neuroectodermal tumour arising within low grade astrocytoma: transformation, de novo or radiation induced? Report of three cases and review of literature. Br J Neurosurg. 22(3):402-8, 2008
                                                                                              12. Smee RI et al: Medulloblastomas-primitive neuroectodermal tumours in the adult population. J Med Imaging Radiat Oncol. 52(1):72-6, 2008
                                                                                              13. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                                                                              14. Murakami R et al: Malignant supratentorial astrocytoma treated with postoperative radiation therapy: prognostic value of pretreatment quantitative diffusion-weighted MR imaging. Radiology. 243(2):493-9, 2007
                                                                                              15. Pfister S et al: Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas. Genes Chromosomes Cancer. 46(9):839-51, 2007
                                                                                              16. Larouche V et al: Supratentorial primitive neuroectodermal tumors in young children. J Clin Oncol. 24(35):5609; author reply 5609-10, 2006
                                                                                              17. Didiano D et al: Telomere maintenance in childhood primitive neuroectodermal brain tumors. Neuro Oncol. 6(1):1-8, 2004
                                                                                              18. Yamada T et al: Prenatal imaging of congenital cerebral primitive neuroectodermal tumor. Fetal Diagn Ther. 18(3):137-9, 2003
                                                                                              19. Etuş V et al: Primary cerebral neuroblastoma: a case report and review. Tohoku J Exp Med. 197(1):55-65, 2002