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Pleomorphic Xanthoastrocytoma
Kevin R. Moore, MD; Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Pleomorphic xanthoastrocytoma (PXA)
              • Definitions

                • Astrocytic neoplasm with generally favorable prognosis in children and young adults
                  • Superficial location in cerebral hemispheres with involvement of meninges

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine 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. Lundar T et al: Neurosurgical treatment of pediatric pleomorphic xanthoastrocytomas: long-term follow-up of a single-institution, consecutive series of 12 patients. J Neurosurg Pediatr. 1-5, 2019
                                                2. Thomas AA et al: Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab. Pediatr Blood Cancer. 66(1):e27465, 2019
                                                3. Mallick S et al: Grade II pleomorphic xanthoastrocytoma; a meta-analysis of data from previously reported 167 cases. J Clin Neurosci. 54:57-62, 2018
                                                4. She D et al: Diagnostic accuracy of diffusion weighted imaging for differentiation of supratentorial pilocytic astrocytoma and pleomorphic xanthoastrocytoma. Neuroradiology. 60(7):725-733, 2018
                                                5. She D et al: MR Imaging features of anaplastic pleomorphic xanthoastrocytoma mimicking high-grade astrocytoma. AJNR Am J Neuroradiol. 39(8):1446-52, 2018
                                                6. Tonse R et al: Impact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma. J Neurooncol. 136(2):343-50, 2018
                                                7. Zou H et al: Molecular features of pleomorphic xanthoastrocytoma. Hum Pathol. 86:38-48, 2018
                                                8. Dodgshun AJ et al: Pediatric pleomorphic xanthoastrocytoma treated with surgical resection alone: clinicopathologic features. J Pediatr Hematol Oncol. 38(7):e202-6, 2016
                                                9. Ho CY et al: Relative cerebral blood volume from dynamic susceptibility contrast perfusion in the grading of pediatric primary brain tumors. Neuroradiology. 57(3):299-306, 2015
                                                10. Sui Y et al: Differentiation of low- and high-grade pediatric brain tumors with high b-value diffusion-weighted MR imaging and a fractional order calculus model. Radiology. 142156, 2015
                                                11. Ida CM et al: Pleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol. 25(5):575-86, 2014
                                                12. Koelsche C et al: BRAF-mutated pleomorphic xanthoastrocytoma is associated with temporal location, reticulin fiber deposition and CD34 expression. Brain Pathol. 24(3):221-9, 2014
                                                13. Moore W et al: Pleomorphic xanthoastrocytoma of childhood: MR imaging and diffusion MR imaging features. AJNR Am J Neuroradiol. 35(11):2192-6, 2014
                                                14. Oh T et al: Pleomorphic xanthoastrocytomas: institutional experience of 18 patients. J Clin Neurosci. 21(10):1767-72, 2014
                                                15. Borja MJ et al: Conventional and advanced MRI features of pediatric intracranial tumors: supratentorial tumors. AJR Am J Roentgenol. 200(5):W483-503, 2013
                                                16. Gallo P et al: Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic factors. Br J Neurosurg. 27(6):759-64, 2013
                                                17. Kahramancetin N et al: Aggressive behavior and anaplasia in pleomorphic xanthoastrocytoma: a plea for a revision of the current WHO classification. CNS Oncol. 2(6):523-30, 2013
                                                18. Neal MT et al: Pleomorphic xanthoastrocytoma in two siblings with neurofibromatosis type 1 (NF-1). Clin Neuropathol. 31(1):54-6, 2012
                                                19. Kim B et al: Pleomorphic xanthoastrocytoma associated with long-standing Taylor-type IIB-focal cortical dysplasia in an adult. Pathol Res Pract. 205(2):113-7, 2009
                                                20. Wind JJ et al: Pleomorphic xanthoastrocytoma presenting with life-threatening hemorrhage in a child. J Neurosurg Pediatr. 3(2):157-9, 2009
                                                21. Yeaney GA et al: A 16-year-old male with a cerebellar mass. Brain Pathol. 19(1):167-70, 2009
                                                22. Hirose T et al: Pleomorphic xanthoastrocytoma: a comparative pathological study between conventional and anaplastic types. Histopathology. 52(2):183-93, 2008
                                                23. Ng WH et al: Pleomorphic xanthoastrocytoma in elderly patients may portend a poor prognosis. J Clin Neurosci. 15(4):476-8, 2008
                                                24. Chacko G et al: Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma. J Neurooncol. 84(2):217-22, 2007
                                                25. Hamlat A et al: Cerebellar pleomorphic xanthoastrocytoma: case report and literature review. Surg Neurol. 68(1):89-94; discussion 94-5, 2007
                                                26. Ishizawa K et al: A neuroepithelial tumor showing combined histological features of dysembryoplastic neuroepithelial tumor and pleomorphic xanthoastrocytoma--a case report and review of the literature. Clin Neuropathol. 26(4):169-75, 2007
                                                27. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Pleomorphic xanthoastrocytoma. Lyon: IARC Press. 22-24, 2007
                                                28. Marton E et al: Malignant progression in pleomorphic xanthoastrocytoma: personal experience and review of the literature. J Neurol Sci. 252(2):144-53, 2007
                                                29. Mohamed IS et al: Magnetoencephalography and diffusion tensor imaging in gelastic seizures secondary to a cingulate gyrus lesion. Clin Neurol Neurosurg. 109(2):182-7, 2007
                                                30. Baehring JM et al: Images in neuro-oncology: anaplastic pleomorphic xanthoastrocytoma. J Neurooncol. 79(2):151-2, 2006
                                                31. Chang HT et al: Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy. Childs Nerv Syst. 22(6):609-13, 2006
                                                32. Dickerman RD et al: Diffuse malignant transformation of pleomorphic xanthoastrocytoma 21 years later: a matter of time? Acta Neurochir (Wien). 148(1):95-7; author reply 97, 2006
                                                33. Nakamura M et al: Pleomorphic xanthoastrocytoma of the spinal cord. Case report. J Neurosurg Spine. 5(1):72-5, 2006
                                                34. Passone E et al: Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination. Childs Nerv Syst. 22(6):614-8, 2006
                                                35. Gelpi E et al: Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: implications for differential diagnosis. Neuropathology. 25(3):241-6, 2005
                                                36. Kilickesmez O et al: Coexistence of pleomorphic xanthoastrocytoma with Sturge-Weber syndrome: MRI features. Pediatr Radiol. 35(9):910-3, 2005
                                                37. Saikali S et al: Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. Case report and review of the literature. J Neurosurg. 102(2):376-81, 2005
                                                38. Yoshida D et al: A case of pleomorphic xanthoastrocytoma presenting with massive tumoral hemorrhage. J Neurooncol. 71(2):169-71, 2005
                                                39. Im SH et al: Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes. J Neurooncol. 66(1-2):17-27, 2004
                                                40. Reifenberger G et al: Expression of the CD34 antigen in pleomorphic xanthoastrocytomas. Acta Neuropathol (Berl). 105(4):358-64, 2003
                                                41. Burger PC et al: Surgical pathology of the nervous system and its coverings: The Brain: Tumors. 4th ed. Philadelphia: Churchill Livingstone. 215-20, 2002
                                                42. Etzl MM Jr et al: Positron emission tomography in three children with pleomorphic xanthoastrocytoma. J Child Neurol. 17(7):522-7, 2002
                                                43. Fouladi M et al: Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. Neuro-oncol. 3(3):184-92, 2001
                                                44. Koeller KK et al: From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 21(6):1533-56, 2001
                                                45. Tsuyuguchi N et al: Evaluation of pleomorphic xanthoastrocytoma by use of positron emission tomography with. AJNR Am J Neuroradiol. 22(2):311-3, 2001
                                                46. Kepes JJ et al: Pathology and genetics of tumours of the nervous system: Pleomorphic xanthoastrocytoma. Lyon: IARC Press. 52-4, 2000
                                                47. Chakrabarty A et al: Malignant transformation in pleomorphic xanthoastrocytoma--a report of two cases. Br J Neurosurg. 13(5):516-9, 1999
                                                48. Giannini C et al: Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer. 85(9):2033-45, 1999
                                                49. Ohta S et al: Eighteen-year survival of a patient with malignant pleomorphic xanthoastrocytoma associated with von Recklinghausen neurofibromatosis. Br J Neurosurg. 13(4):420-2, 1999
                                                50. Prayson RA et al: Anaplastic pleomorphic xanthoastrocytoma. Arch Pathol Lab Med. 122(12):1082-6, 1998
                                                51. Giannini C et al: Classification and grading of low-grade astrocytic tumors in children. Brain Pathol. 7(2):785-98, 1997
                                                52. Lach B et al: Association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. A report of three cases. Cancer. 78(12):2551-63, 1996
                                                53. Levy RA et al: Pleomorphic xanthoastrocytoma presenting with massive intracranial hemorrhage. AJNR Am J Neuroradiol. 17(1):154-6, 1996
                                                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

                                                              • Pleomorphic xanthoastrocytoma (PXA)
                                                            • Definitions

                                                              • Astrocytic neoplasm with generally favorable prognosis in children and young adults
                                                                • Superficial location in cerebral hemispheres with involvement of meninges

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Angiographic Findings

                                                                    • Nuclear Medicine 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. Lundar T et al: Neurosurgical treatment of pediatric pleomorphic xanthoastrocytomas: long-term follow-up of a single-institution, consecutive series of 12 patients. J Neurosurg Pediatr. 1-5, 2019
                                                                                              2. Thomas AA et al: Anaplastic pleomorphic xanthoastrocytoma with leptomeningeal dissemination responsive to BRAF inhibition and bevacizumab. Pediatr Blood Cancer. 66(1):e27465, 2019
                                                                                              3. Mallick S et al: Grade II pleomorphic xanthoastrocytoma; a meta-analysis of data from previously reported 167 cases. J Clin Neurosci. 54:57-62, 2018
                                                                                              4. She D et al: Diagnostic accuracy of diffusion weighted imaging for differentiation of supratentorial pilocytic astrocytoma and pleomorphic xanthoastrocytoma. Neuroradiology. 60(7):725-733, 2018
                                                                                              5. She D et al: MR Imaging features of anaplastic pleomorphic xanthoastrocytoma mimicking high-grade astrocytoma. AJNR Am J Neuroradiol. 39(8):1446-52, 2018
                                                                                              6. Tonse R et al: Impact of WHO 2016 update of brain tumor classification, molecular markers and clinical outcomes in pleomorphic xanthoastrocytoma. J Neurooncol. 136(2):343-50, 2018
                                                                                              7. Zou H et al: Molecular features of pleomorphic xanthoastrocytoma. Hum Pathol. 86:38-48, 2018
                                                                                              8. Dodgshun AJ et al: Pediatric pleomorphic xanthoastrocytoma treated with surgical resection alone: clinicopathologic features. J Pediatr Hematol Oncol. 38(7):e202-6, 2016
                                                                                              9. Ho CY et al: Relative cerebral blood volume from dynamic susceptibility contrast perfusion in the grading of pediatric primary brain tumors. Neuroradiology. 57(3):299-306, 2015
                                                                                              10. Sui Y et al: Differentiation of low- and high-grade pediatric brain tumors with high b-value diffusion-weighted MR imaging and a fractional order calculus model. Radiology. 142156, 2015
                                                                                              11. Ida CM et al: Pleomorphic xanthoastrocytoma: natural history and long-term follow-up. Brain Pathol. 25(5):575-86, 2014
                                                                                              12. Koelsche C et al: BRAF-mutated pleomorphic xanthoastrocytoma is associated with temporal location, reticulin fiber deposition and CD34 expression. Brain Pathol. 24(3):221-9, 2014
                                                                                              13. Moore W et al: Pleomorphic xanthoastrocytoma of childhood: MR imaging and diffusion MR imaging features. AJNR Am J Neuroradiol. 35(11):2192-6, 2014
                                                                                              14. Oh T et al: Pleomorphic xanthoastrocytomas: institutional experience of 18 patients. J Clin Neurosci. 21(10):1767-72, 2014
                                                                                              15. Borja MJ et al: Conventional and advanced MRI features of pediatric intracranial tumors: supratentorial tumors. AJR Am J Roentgenol. 200(5):W483-503, 2013
                                                                                              16. Gallo P et al: Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic factors. Br J Neurosurg. 27(6):759-64, 2013
                                                                                              17. Kahramancetin N et al: Aggressive behavior and anaplasia in pleomorphic xanthoastrocytoma: a plea for a revision of the current WHO classification. CNS Oncol. 2(6):523-30, 2013
                                                                                              18. Neal MT et al: Pleomorphic xanthoastrocytoma in two siblings with neurofibromatosis type 1 (NF-1). Clin Neuropathol. 31(1):54-6, 2012
                                                                                              19. Kim B et al: Pleomorphic xanthoastrocytoma associated with long-standing Taylor-type IIB-focal cortical dysplasia in an adult. Pathol Res Pract. 205(2):113-7, 2009
                                                                                              20. Wind JJ et al: Pleomorphic xanthoastrocytoma presenting with life-threatening hemorrhage in a child. J Neurosurg Pediatr. 3(2):157-9, 2009
                                                                                              21. Yeaney GA et al: A 16-year-old male with a cerebellar mass. Brain Pathol. 19(1):167-70, 2009
                                                                                              22. Hirose T et al: Pleomorphic xanthoastrocytoma: a comparative pathological study between conventional and anaplastic types. Histopathology. 52(2):183-93, 2008
                                                                                              23. Ng WH et al: Pleomorphic xanthoastrocytoma in elderly patients may portend a poor prognosis. J Clin Neurosci. 15(4):476-8, 2008
                                                                                              24. Chacko G et al: Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma. J Neurooncol. 84(2):217-22, 2007
                                                                                              25. Hamlat A et al: Cerebellar pleomorphic xanthoastrocytoma: case report and literature review. Surg Neurol. 68(1):89-94; discussion 94-5, 2007
                                                                                              26. Ishizawa K et al: A neuroepithelial tumor showing combined histological features of dysembryoplastic neuroepithelial tumor and pleomorphic xanthoastrocytoma--a case report and review of the literature. Clin Neuropathol. 26(4):169-75, 2007
                                                                                              27. Louis DN et al: WHO Classification of Tumours of the Central Nervous System: Pleomorphic xanthoastrocytoma. Lyon: IARC Press. 22-24, 2007
                                                                                              28. Marton E et al: Malignant progression in pleomorphic xanthoastrocytoma: personal experience and review of the literature. J Neurol Sci. 252(2):144-53, 2007
                                                                                              29. Mohamed IS et al: Magnetoencephalography and diffusion tensor imaging in gelastic seizures secondary to a cingulate gyrus lesion. Clin Neurol Neurosurg. 109(2):182-7, 2007
                                                                                              30. Baehring JM et al: Images in neuro-oncology: anaplastic pleomorphic xanthoastrocytoma. J Neurooncol. 79(2):151-2, 2006
                                                                                              31. Chang HT et al: Pediatric cerebellar pleomorphic xanthoastrocytoma with anaplastic features: a case of long-term survival after multimodality therapy. Childs Nerv Syst. 22(6):609-13, 2006
                                                                                              32. Dickerman RD et al: Diffuse malignant transformation of pleomorphic xanthoastrocytoma 21 years later: a matter of time? Acta Neurochir (Wien). 148(1):95-7; author reply 97, 2006
                                                                                              33. Nakamura M et al: Pleomorphic xanthoastrocytoma of the spinal cord. Case report. J Neurosurg Spine. 5(1):72-5, 2006
                                                                                              34. Passone E et al: Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination. Childs Nerv Syst. 22(6):614-8, 2006
                                                                                              35. Gelpi E et al: Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: implications for differential diagnosis. Neuropathology. 25(3):241-6, 2005
                                                                                              36. Kilickesmez O et al: Coexistence of pleomorphic xanthoastrocytoma with Sturge-Weber syndrome: MRI features. Pediatr Radiol. 35(9):910-3, 2005
                                                                                              37. Saikali S et al: Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. Case report and review of the literature. J Neurosurg. 102(2):376-81, 2005
                                                                                              38. Yoshida D et al: A case of pleomorphic xanthoastrocytoma presenting with massive tumoral hemorrhage. J Neurooncol. 71(2):169-71, 2005
                                                                                              39. Im SH et al: Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes. J Neurooncol. 66(1-2):17-27, 2004
                                                                                              40. Reifenberger G et al: Expression of the CD34 antigen in pleomorphic xanthoastrocytomas. Acta Neuropathol (Berl). 105(4):358-64, 2003
                                                                                              41. Burger PC et al: Surgical pathology of the nervous system and its coverings: The Brain: Tumors. 4th ed. Philadelphia: Churchill Livingstone. 215-20, 2002
                                                                                              42. Etzl MM Jr et al: Positron emission tomography in three children with pleomorphic xanthoastrocytoma. J Child Neurol. 17(7):522-7, 2002
                                                                                              43. Fouladi M et al: Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. Neuro-oncol. 3(3):184-92, 2001
                                                                                              44. Koeller KK et al: From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 21(6):1533-56, 2001
                                                                                              45. Tsuyuguchi N et al: Evaluation of pleomorphic xanthoastrocytoma by use of positron emission tomography with. AJNR Am J Neuroradiol. 22(2):311-3, 2001
                                                                                              46. Kepes JJ et al: Pathology and genetics of tumours of the nervous system: Pleomorphic xanthoastrocytoma. Lyon: IARC Press. 52-4, 2000
                                                                                              47. Chakrabarty A et al: Malignant transformation in pleomorphic xanthoastrocytoma--a report of two cases. Br J Neurosurg. 13(5):516-9, 1999
                                                                                              48. Giannini C et al: Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer. 85(9):2033-45, 1999
                                                                                              49. Ohta S et al: Eighteen-year survival of a patient with malignant pleomorphic xanthoastrocytoma associated with von Recklinghausen neurofibromatosis. Br J Neurosurg. 13(4):420-2, 1999
                                                                                              50. Prayson RA et al: Anaplastic pleomorphic xanthoastrocytoma. Arch Pathol Lab Med. 122(12):1082-6, 1998
                                                                                              51. Giannini C et al: Classification and grading of low-grade astrocytic tumors in children. Brain Pathol. 7(2):785-98, 1997
                                                                                              52. Lach B et al: Association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. A report of three cases. Cancer. 78(12):2551-63, 1996
                                                                                              53. Levy RA et al: Pleomorphic xanthoastrocytoma presenting with massive intracranial hemorrhage. AJNR Am J Neuroradiol. 17(1):154-6, 1996