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Pilomyxoid Astrocytoma
Luke L. Linscott, MD; Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Pilomyxoid astrocytoma (PMA)
                • Myxoid variant of pilocytic astrocytoma (PA)
              • Definitions

                • Tumor with monomorphic piloid cells dispersed in mucopolysaccharide-rich matrix
                  • More aggressive tumor than PA with high risk of local recurrence, dissemination

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Other Modality 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. He J et al: Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients. Childs Nerv Syst. 34(1):149-53, 2018
                                              2. 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
                                              3. Wang Z et al: Spontaneous intratumoural and intraventricular haemorrhage associated with a pilomyxoid astrocytoma in the hypothalamic/chiasmatic region. J Clin Neurosci. 33:217-20, 2016
                                              4. Alkonyi B et al: Differential imaging characteristics and dissemination potential of pilomyxoid astrocytomas versus pilocytic astrocytomas. Neuroradiology. 57(6):625-38, 2015
                                              5. Amarasinghe SG et al: A rare case of multicystic disseminated astrocytoma with pilomyxoid characteristics in a 4-year-old child. Childs Nerv Syst. 31(4):625-9, 2015
                                              6. Nabavizadeh SA et al: High accuracy of arterial spin labeling perfusion imaging in differentiation of pilomyxoid from pilocytic astrocytoma. Neuroradiology. 57(5):527-33, 2015
                                              7. El Beltagy MA et al: Surgical and clinical aspects of cerebellar pilomyxoid-spectrum astrocytomas in children. Childs Nerv Syst. 30(6):1045-53, 2014
                                              8. Kleinschmidt-DeMasters BK et al: Pilomyxoid astrocytoma (PMA) shows significant differences in gene expression vs. pilocytic astrocytoma (PA) and variable tendency toward maturation to PA. Brain Pathol. 25(4):429-40, 2015
                                              9. Amirjamshidi A et al: Pilomyxoid astrocytoma. J Neurosurg Pediatr. 11(5):613, 2013
                                              10. Bhargava D et al: Occurrence and distribution of pilomyxoid astrocytoma. Br J Neurosurg. 27(4):413-8, 2013
                                              11. Lee IH et al: Imaging characteristics of pilomyxoid astrocytomas in comparison with pilocytic astrocytomas. Eur J Radiol. 79(2):311-6, 2011
                                              12. Johnson MW et al: Spectrum of pilomyxoid astrocytomas: intermediate pilomyxoid tumors. Am J Surg Pathol. 34(12):1783-91, 2010
                                              13. Amatya VJ et al: Clinicopathological and immunohistochemical features of three pilomyxoid astrocytomas: comparative study with 11 pilocytic astrocytomas. Pathol Int. 59(2):80-5, 2009
                                              14. Buccoliero AM et al: Occipital pilomyxoid astrocytoma in a 14-year-old girl--case report. Clin Neuropathol. 27(6):373-7, 2008
                                              15. Komotar RJ et al: Magnetic resonance imaging characteristics of pilomyxoid astrocytoma. Neurol Res. 30(9):945-51, 2008
                                              16. Linscott LL et al: Pilomyxoid astrocytoma: expanding the imaging spectrum. AJNR Am J Neuroradiol. 29(10):1861-6, 2008
                                              17. Brat DJ et al: Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. Brain Pathol. 17(3):319-24, 2007
                                              18. Ceppa EP et al: The pilomyxoid astrocytoma and its relationship to pilocytic astrocytoma: report of a case and a critical review of the entity. J Neurooncol. 81(2):191-6, 2007
                                              19. Morales H et al: Magnetic resonance imaging and spectroscopy of pilomyxoid astrocytomas: case reports and comparison with pilocytic astrocytomas. J Comput Assist Tomogr. 31(5):682-7, 2007
                                              20. Komotar RJ et al: Astrocytoma with pilomyxoid features presenting in an adult. Neuropathology. 26(1):89-93, 2006
                                              21. Melendez B et al: BCR gene disruption in a pilomyxoid astrocytoma. Neuropathology. 26(5):442-6, 2006
                                              22. Cirak B et al: Proton magnetic resonance spectroscopic imaging in pediatric pilomyxoid astrocytoma. Childs Nerv Syst. 21(5):404-9, 2005
                                              23. Komotar RJ et al: Pilomyxoid astrocytoma of the spinal cord: report of three cases. Neurosurgery. 56(1):191, 2005
                                              24. Chikai K et al: Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway. Acta Neuropathol (Berl). 108(2):109-14, 2004
                                              25. Darwish B et al: Juvenile pilocytic astrocytoma 'pilomyxoid variant' with spinal metastases. J Clin Neurosci. 11(6):640-2, 2004
                                              26. Komotar RJ et al: Pilomyxoid astrocytoma: a review. MedGenMed. 6(4):42, 2004
                                              27. Arslanoglu A et al: MR imaging characteristics of pilomyxoid astrocytomas. AJNR Am J Neuroradiol. 24(9):1906-8, 2003
                                              28. Burger PC et al: Pathology of diencephalic astrocytomas. Pediatr Neurosurg. 32(4):214-9, 2000
                                              29. Tihan T et al: Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. J Neuropathol Exp Neurol. 58(10):1061-8, 1999
                                              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

                                                            • Pilomyxoid astrocytoma (PMA)
                                                            • Myxoid variant of pilocytic astrocytoma (PA)
                                                          • Definitions

                                                            • Tumor with monomorphic piloid cells dispersed in mucopolysaccharide-rich matrix
                                                              • More aggressive tumor than PA with high risk of local recurrence, dissemination

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Other Modality 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. He J et al: Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients. Childs Nerv Syst. 34(1):149-53, 2018
                                                                                          2. 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
                                                                                          3. Wang Z et al: Spontaneous intratumoural and intraventricular haemorrhage associated with a pilomyxoid astrocytoma in the hypothalamic/chiasmatic region. J Clin Neurosci. 33:217-20, 2016
                                                                                          4. Alkonyi B et al: Differential imaging characteristics and dissemination potential of pilomyxoid astrocytomas versus pilocytic astrocytomas. Neuroradiology. 57(6):625-38, 2015
                                                                                          5. Amarasinghe SG et al: A rare case of multicystic disseminated astrocytoma with pilomyxoid characteristics in a 4-year-old child. Childs Nerv Syst. 31(4):625-9, 2015
                                                                                          6. Nabavizadeh SA et al: High accuracy of arterial spin labeling perfusion imaging in differentiation of pilomyxoid from pilocytic astrocytoma. Neuroradiology. 57(5):527-33, 2015
                                                                                          7. El Beltagy MA et al: Surgical and clinical aspects of cerebellar pilomyxoid-spectrum astrocytomas in children. Childs Nerv Syst. 30(6):1045-53, 2014
                                                                                          8. Kleinschmidt-DeMasters BK et al: Pilomyxoid astrocytoma (PMA) shows significant differences in gene expression vs. pilocytic astrocytoma (PA) and variable tendency toward maturation to PA. Brain Pathol. 25(4):429-40, 2015
                                                                                          9. Amirjamshidi A et al: Pilomyxoid astrocytoma. J Neurosurg Pediatr. 11(5):613, 2013
                                                                                          10. Bhargava D et al: Occurrence and distribution of pilomyxoid astrocytoma. Br J Neurosurg. 27(4):413-8, 2013
                                                                                          11. Lee IH et al: Imaging characteristics of pilomyxoid astrocytomas in comparison with pilocytic astrocytomas. Eur J Radiol. 79(2):311-6, 2011
                                                                                          12. Johnson MW et al: Spectrum of pilomyxoid astrocytomas: intermediate pilomyxoid tumors. Am J Surg Pathol. 34(12):1783-91, 2010
                                                                                          13. Amatya VJ et al: Clinicopathological and immunohistochemical features of three pilomyxoid astrocytomas: comparative study with 11 pilocytic astrocytomas. Pathol Int. 59(2):80-5, 2009
                                                                                          14. Buccoliero AM et al: Occipital pilomyxoid astrocytoma in a 14-year-old girl--case report. Clin Neuropathol. 27(6):373-7, 2008
                                                                                          15. Komotar RJ et al: Magnetic resonance imaging characteristics of pilomyxoid astrocytoma. Neurol Res. 30(9):945-51, 2008
                                                                                          16. Linscott LL et al: Pilomyxoid astrocytoma: expanding the imaging spectrum. AJNR Am J Neuroradiol. 29(10):1861-6, 2008
                                                                                          17. Brat DJ et al: Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. Brain Pathol. 17(3):319-24, 2007
                                                                                          18. Ceppa EP et al: The pilomyxoid astrocytoma and its relationship to pilocytic astrocytoma: report of a case and a critical review of the entity. J Neurooncol. 81(2):191-6, 2007
                                                                                          19. Morales H et al: Magnetic resonance imaging and spectroscopy of pilomyxoid astrocytomas: case reports and comparison with pilocytic astrocytomas. J Comput Assist Tomogr. 31(5):682-7, 2007
                                                                                          20. Komotar RJ et al: Astrocytoma with pilomyxoid features presenting in an adult. Neuropathology. 26(1):89-93, 2006
                                                                                          21. Melendez B et al: BCR gene disruption in a pilomyxoid astrocytoma. Neuropathology. 26(5):442-6, 2006
                                                                                          22. Cirak B et al: Proton magnetic resonance spectroscopic imaging in pediatric pilomyxoid astrocytoma. Childs Nerv Syst. 21(5):404-9, 2005
                                                                                          23. Komotar RJ et al: Pilomyxoid astrocytoma of the spinal cord: report of three cases. Neurosurgery. 56(1):191, 2005
                                                                                          24. Chikai K et al: Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway. Acta Neuropathol (Berl). 108(2):109-14, 2004
                                                                                          25. Darwish B et al: Juvenile pilocytic astrocytoma 'pilomyxoid variant' with spinal metastases. J Clin Neurosci. 11(6):640-2, 2004
                                                                                          26. Komotar RJ et al: Pilomyxoid astrocytoma: a review. MedGenMed. 6(4):42, 2004
                                                                                          27. Arslanoglu A et al: MR imaging characteristics of pilomyxoid astrocytomas. AJNR Am J Neuroradiol. 24(9):1906-8, 2003
                                                                                          28. Burger PC et al: Pathology of diencephalic astrocytomas. Pediatr Neurosurg. 32(4):214-9, 2000
                                                                                          29. Tihan T et al: Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. J Neuropathol Exp Neurol. 58(10):1061-8, 1999