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CNS-PNET
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Supratentorial primitive neuroectodermal tumor
              • Supratentorial primitive neuroepithelial tumor
            • Definitions

              • Central nervous system primitive neuroectodermal tumor (CNS-PNET): Antiquated term for primitive cerebral embryonal tumor; no longer in WHO 2016
                • Umbrella term formerly containing CNS neuroblastoma, ependymoblastoma, medulloepithelioma, & ETANTR (ependymal tumor with abundant neuropil & true rosettes)
              • Now molecularly & genetically distinct entities considered under embryonal tumors (all WHO grade IV) include
                • Embryonal tumor with multilayered rosettes, C19MC-altered
                • Embryonal tumor with multilayered rosettes, not otherwise specified (NOS)
                • CNS embryonal tumor, NOS
                  • Most CNS-PNET lesions likely here
                • Medulloepithelioma
                • Medulloblastoma
                • Atypical teratoid/rhabdoid tumor
                • CNS embryonal tumor with rhabdoid features

            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

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Louis DN et al: The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 131(6):803-20, 2016
                                          2. Biswas A et al: Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India. Acta Neurochir (Wien). 157(7):1251-66, 2015
                                          3. Jakacki RI et al: Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children's Oncology Group. Pediatr Blood Cancer. 62(5):776-83, 2015
                                          4. Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. 17(8):1157-65, 2015
                                          5. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                          6. De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
                                          7. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                          8. von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
                                          9. Behdad A et al: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol. 20(2):441-50, 2010
                                          10. Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
                                          11. Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
                                          12. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                          13. 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
                                          14. Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
                                          15. Etuş V et al: Primary cerebral neuroblastoma: a case report and review. Tohoku J Exp Med. 197(1):55-65, 2002
                                          16. Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 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

                                                    • Synonyms

                                                      • Supratentorial primitive neuroectodermal tumor
                                                      • Supratentorial primitive neuroepithelial tumor
                                                    • Definitions

                                                      • Central nervous system primitive neuroectodermal tumor (CNS-PNET): Antiquated term for primitive cerebral embryonal tumor; no longer in WHO 2016
                                                        • Umbrella term formerly containing CNS neuroblastoma, ependymoblastoma, medulloepithelioma, & ETANTR (ependymal tumor with abundant neuropil & true rosettes)
                                                      • Now molecularly & genetically distinct entities considered under embryonal tumors (all WHO grade IV) include
                                                        • Embryonal tumor with multilayered rosettes, C19MC-altered
                                                        • Embryonal tumor with multilayered rosettes, not otherwise specified (NOS)
                                                        • CNS embryonal tumor, NOS
                                                          • Most CNS-PNET lesions likely here
                                                        • Medulloepithelioma
                                                        • Medulloblastoma
                                                        • Atypical teratoid/rhabdoid tumor
                                                        • CNS embryonal tumor with rhabdoid features

                                                    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

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Louis DN et al: The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 131(6):803-20, 2016
                                                                                  2. Biswas A et al: Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India. Acta Neurochir (Wien). 157(7):1251-66, 2015
                                                                                  3. Jakacki RI et al: Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children's Oncology Group. Pediatr Blood Cancer. 62(5):776-83, 2015
                                                                                  4. Nowak J et al: Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. Neuro Oncol. 17(8):1157-65, 2015
                                                                                  5. Lester RA et al: Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. J Neurooncol. 120(2):371-9, 2014
                                                                                  6. De Braganca KC et al: Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 15(5):593-606, 2013
                                                                                  7. Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
                                                                                  8. von Bueren AO: CNS PNET molecular subgroups with distinct clinical features. Lancet Oncol. 13(8):753-4, 2012
                                                                                  9. Behdad A et al: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol. 20(2):441-50, 2010
                                                                                  10. Pfister SM et al: Molecular diagnostics of CNS embryonal tumors. Acta Neuropathol. 120(5):553-66, 2010
                                                                                  11. Johnston DL et al: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol. 86(1):101-8, 2008
                                                                                  12. Chawla A et al: Paediatric PNET: pre-surgical MRI features. Clin Radiol. 62(1):43-52, 2007
                                                                                  13. 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
                                                                                  14. Inda MM et al: Genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumours of the central nervous system. Histopathology. 47(6):631-7, 2005
                                                                                  15. Etuş V et al: Primary cerebral neuroblastoma: a case report and review. Tohoku J Exp Med. 197(1):55-65, 2002
                                                                                  16. Pomeroy SL et al: Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 415(6870):436-42, 2002