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Pineal Parenchymal Tumor of Intermediate Differentiation
Karen L. Salzman, MD; Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Pineal parenchymal tumor of intermediate differentiation (PPTID)
            • Synonyms

              • Atypical pineocytoma (outdated term)
            • Definitions

              • Primary parenchymal neoplasm of pineal gland
                • Intermediate in malignancy between pineocytoma and pineoblastoma
                  • Pineocytoma (WHO grade 1): Slow-growing neoplasm with small mature cells that resemble pineocytes
                    • Adults predominate
                    • Well-differentiated histology with large "pineocytomatous rosettes"
                    • Indolent course (imaging may remain stable for years)
                    • Generally good prognosis
                  • Pineoblastoma (WHO grade 4): Highly malignant primitive embryonal tumor of pineal gland
                    • Occurs primarily (but not exclusively) in children
                    • Poorly differentiated
                    • Aggressive behavior
                    • Poor prognosis

            IMAGING

            • General Features

              • CT Findings

                • MR 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

                                          • Reporting Tips

                                            Selected References

                                            1. Li BK et al: Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study. Acta Neuropathol. 139(2):223-41, 2020
                                            2. Choque-Velasquez J et al: Pineal parenchymal tumors of intermediate differentiation: a long-term follow-up study in Helsinki neurosurgery. World Neurosurg. 122:e729-39, 2019
                                            3. Corrigenda. Neuro Oncol. 19(12):1702, 2017
                                            4. Tamrazi B et al: Pineal region masses in pediatric patients. Neuroimaging Clin N Am. 27(1):85-97, 2017
                                            5. Jouvet A. et al: Pineal parenchymal tumour of intermediate differentiation. In DN Louis et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 173-75, 2016
                                            6. Yu T et al: Twenty-seven cases of pineal parenchymal tumours of intermediate differentiation: mitotic count, Ki-67 labelling index and extent of resection predict prognosis. J Neurol Neurosurg Psychiatry. ePub, 2015
                                            7. Ito T et al: Clinicopathologic study of pineal parenchymal tumors of intermediate differentiation. World Neurosurg. 81(5-6):783-9, 2014
                                            8. Kakigi T et al: Quantitative imaging values of CT, MR, and FDG-PET to differentiate pineal parenchymal tumors and germinomas: are they useful? Neuroradiology. 56(4):297-303, 2014
                                            9. Watanabe T et al: Pineal parenchymal tumor of intermediate differentiation: Treatment outcomes of five cases. Mol Clin Oncol. 2(2):197-202, 2014
                                            10. Fukuoka K et al: Pineal parenchymal tumor of intermediate differentiation with marked elevation of MIB-1 labeling index. Brain Tumor Pathol. Epub ahead of print, 2012
                                            11. Han SJ et al: Pathology of pineal parenchymal tumors. Neurosurg Clin N Am. 22(3):335-40, 2011
                                            12. Komakula S et al: Pineal parenchymal tumor of intermediate differentiation: imaging spectrum of an unusual tumor in 11 cases. Neuroradiology. 53(8):577-84, 2011
                                            13. Kim BS et al: Pineal parenchymal tumor of intermediate differentiation showing malignant progression at relapse. Neuropathology. 29(5):602-8, 2009
                                            14. Sato K et al: Pathology of pineal parenchymal tumors. Prog Neurol Surg. 23:12-25, 2009
                                            15. Fèvre-Montange M et al: Pineocytoma and pineal parenchymal tumors of intermediate differentiation presenting cytologic pleomorphism: a multicenter study. Brain Pathol. 18(3):354-9, 2008
                                            16. Rousseau A et al: The 2007 WHO classification of tumors of the central nervous system - what has changed? Curr Opin Neurol. 21(6):720-7, 2008
                                            17. Senft C et al: Pineal parenchymal tumor of intermediate differentiation: diagnostic pitfalls and discussion of treatment options of a rare tumor entity. Neurosurg Rev. 31(2):231-6, 2008
                                            18. Nakazato Y et al: Pineal parenchymal tumour of intermediate differentiation. In DN Louis et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 124-5, 2007
                                            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

                                                        • Pineal parenchymal tumor of intermediate differentiation (PPTID)
                                                      • Synonyms

                                                        • Atypical pineocytoma (outdated term)
                                                      • Definitions

                                                        • Primary parenchymal neoplasm of pineal gland
                                                          • Intermediate in malignancy between pineocytoma and pineoblastoma
                                                            • Pineocytoma (WHO grade 1): Slow-growing neoplasm with small mature cells that resemble pineocytes
                                                              • Adults predominate
                                                              • Well-differentiated histology with large "pineocytomatous rosettes"
                                                              • Indolent course (imaging may remain stable for years)
                                                              • Generally good prognosis
                                                            • Pineoblastoma (WHO grade 4): Highly malignant primitive embryonal tumor of pineal gland
                                                              • Occurs primarily (but not exclusively) in children
                                                              • Poorly differentiated
                                                              • Aggressive behavior
                                                              • Poor prognosis

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR 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

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Li BK et al: Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study. Acta Neuropathol. 139(2):223-41, 2020
                                                                                      2. Choque-Velasquez J et al: Pineal parenchymal tumors of intermediate differentiation: a long-term follow-up study in Helsinki neurosurgery. World Neurosurg. 122:e729-39, 2019
                                                                                      3. Corrigenda. Neuro Oncol. 19(12):1702, 2017
                                                                                      4. Tamrazi B et al: Pineal region masses in pediatric patients. Neuroimaging Clin N Am. 27(1):85-97, 2017
                                                                                      5. Jouvet A. et al: Pineal parenchymal tumour of intermediate differentiation. In DN Louis et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 173-75, 2016
                                                                                      6. Yu T et al: Twenty-seven cases of pineal parenchymal tumours of intermediate differentiation: mitotic count, Ki-67 labelling index and extent of resection predict prognosis. J Neurol Neurosurg Psychiatry. ePub, 2015
                                                                                      7. Ito T et al: Clinicopathologic study of pineal parenchymal tumors of intermediate differentiation. World Neurosurg. 81(5-6):783-9, 2014
                                                                                      8. Kakigi T et al: Quantitative imaging values of CT, MR, and FDG-PET to differentiate pineal parenchymal tumors and germinomas: are they useful? Neuroradiology. 56(4):297-303, 2014
                                                                                      9. Watanabe T et al: Pineal parenchymal tumor of intermediate differentiation: Treatment outcomes of five cases. Mol Clin Oncol. 2(2):197-202, 2014
                                                                                      10. Fukuoka K et al: Pineal parenchymal tumor of intermediate differentiation with marked elevation of MIB-1 labeling index. Brain Tumor Pathol. Epub ahead of print, 2012
                                                                                      11. Han SJ et al: Pathology of pineal parenchymal tumors. Neurosurg Clin N Am. 22(3):335-40, 2011
                                                                                      12. Komakula S et al: Pineal parenchymal tumor of intermediate differentiation: imaging spectrum of an unusual tumor in 11 cases. Neuroradiology. 53(8):577-84, 2011
                                                                                      13. Kim BS et al: Pineal parenchymal tumor of intermediate differentiation showing malignant progression at relapse. Neuropathology. 29(5):602-8, 2009
                                                                                      14. Sato K et al: Pathology of pineal parenchymal tumors. Prog Neurol Surg. 23:12-25, 2009
                                                                                      15. Fèvre-Montange M et al: Pineocytoma and pineal parenchymal tumors of intermediate differentiation presenting cytologic pleomorphism: a multicenter study. Brain Pathol. 18(3):354-9, 2008
                                                                                      16. Rousseau A et al: The 2007 WHO classification of tumors of the central nervous system - what has changed? Curr Opin Neurol. 21(6):720-7, 2008
                                                                                      17. Senft C et al: Pineal parenchymal tumor of intermediate differentiation: diagnostic pitfalls and discussion of treatment options of a rare tumor entity. Neurosurg Rev. 31(2):231-6, 2008
                                                                                      18. Nakazato Y et al: Pineal parenchymal tumour of intermediate differentiation. In DN Louis et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 124-5, 2007