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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. Ho CY et al: Differentiation of pilocytic and pilomyxoid astrocytomas using dynamic susceptibility contrast perfusion and diffusion weighted imaging. Neuroradiology. 62(1):81-8, 2020
                                              2. Ishi Y et al: Association of the FGFR1 mutation with spontaneous hemorrhage in low-grade gliomas in pediatric and young adult patients. J Neurosurg. 1-9, 2020
                                              3. Kulac I et al: Pilomyxoid astrocytomas: a short review. Brain Tumor Pathol. 36(2):52-5, 2019
                                              4. He J et al: Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients. Childs Nerv Syst. 34(1):149-53, 2018
                                              5. 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
                                              6. Rosenfeld A et al: A case series characterizing pilomyxoid astrocytomas in childhood. J Pediatr Hematol Oncol. 38(2):e63-6, 2016
                                              7. 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
                                              8. Alkonyi B et al: Differential imaging characteristics and dissemination potential of pilomyxoid astrocytomas versus pilocytic astrocytomas. Neuroradiology. 57(6):625-38, 2015
                                              9. 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
                                              10. 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
                                              11. El Beltagy MA et al: Surgical and clinical aspects of cerebellar pilomyxoid-spectrum astrocytomas in children. Childs Nerv Syst. 30(6):1045-53, 2014
                                              12. 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
                                              13. Amirjamshidi A et al: Pilomyxoid astrocytoma. J Neurosurg Pediatr. 11(5):613, 2013
                                              14. Bhargava D et al: Occurrence and distribution of pilomyxoid astrocytoma. Br J Neurosurg. 27(4):413-8, 2013
                                              15. Lee IH et al: Imaging characteristics of pilomyxoid astrocytomas in comparison with pilocytic astrocytomas. Eur J Radiol. 79(2):311-6, 2011
                                              16. Johnson MW et al: Spectrum of pilomyxoid astrocytomas: intermediate pilomyxoid tumors. Am J Surg Pathol. 34(12):1783-91, 2010
                                              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. Ho CY et al: Differentiation of pilocytic and pilomyxoid astrocytomas using dynamic susceptibility contrast perfusion and diffusion weighted imaging. Neuroradiology. 62(1):81-8, 2020
                                                                                          2. Ishi Y et al: Association of the FGFR1 mutation with spontaneous hemorrhage in low-grade gliomas in pediatric and young adult patients. J Neurosurg. 1-9, 2020
                                                                                          3. Kulac I et al: Pilomyxoid astrocytomas: a short review. Brain Tumor Pathol. 36(2):52-5, 2019
                                                                                          4. He J et al: Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients. Childs Nerv Syst. 34(1):149-53, 2018
                                                                                          5. 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
                                                                                          6. Rosenfeld A et al: A case series characterizing pilomyxoid astrocytomas in childhood. J Pediatr Hematol Oncol. 38(2):e63-6, 2016
                                                                                          7. 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
                                                                                          8. Alkonyi B et al: Differential imaging characteristics and dissemination potential of pilomyxoid astrocytomas versus pilocytic astrocytomas. Neuroradiology. 57(6):625-38, 2015
                                                                                          9. 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
                                                                                          10. 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
                                                                                          11. El Beltagy MA et al: Surgical and clinical aspects of cerebellar pilomyxoid-spectrum astrocytomas in children. Childs Nerv Syst. 30(6):1045-53, 2014
                                                                                          12. 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
                                                                                          13. Amirjamshidi A et al: Pilomyxoid astrocytoma. J Neurosurg Pediatr. 11(5):613, 2013
                                                                                          14. Bhargava D et al: Occurrence and distribution of pilomyxoid astrocytoma. Br J Neurosurg. 27(4):413-8, 2013
                                                                                          15. Lee IH et al: Imaging characteristics of pilomyxoid astrocytomas in comparison with pilocytic astrocytomas. Eur J Radiol. 79(2):311-6, 2011
                                                                                          16. Johnson MW et al: Spectrum of pilomyxoid astrocytomas: intermediate pilomyxoid tumors. Am J Surg Pathol. 34(12):1783-91, 2010