Skip to main content
Log in

Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Pleomorphic xanthoastrocytoma (PXA) is a rare brain glial tumour found in young patients. Most cases are reported as evolving low-grade neoplasms associated with a long survival after complete surgical resection. Some PXAs, however, can demonstrate secondary malignant transformation or progress with a short survival. Anaplastic histological features at first presentation or secondary meningeal dissemination have rarely been reported.

Case report

We describe the case of a cerebral PXA in a 7-year-old girl presenting with anaplastic histological features and craniospinal meningeal dissemination that progressed rapidly with a poor outcome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Bucciero A, Caro MD, Stefano VD, Tedeschi E, Monticelli A, Siciliano A, Cappabianca P, Vizioli L, Cerillo A (1997) Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases. Clin Neurol Neurosurg 99:40–45

    Article  CAS  PubMed  Google Scholar 

  2. Charbel FT (1998) Pleomorphic xanthoastrocytoma with malignant progression. Surg Neurol 50:385–386

    Article  CAS  PubMed  Google Scholar 

  3. Etzl MM Jr, Kaplan AM, Moss SD, Spataro J, Bonstelle CT, Lawson MA, Bandy DJ (2002) Positron emission tomography in three children with pleomorphic xanthoastrocytoma. J Child Neurol 17:522–527

    PubMed  Google Scholar 

  4. Fouladi M, Jenkins J, Burger P, Langston J, Merchant T, Heideman R, Thompson S, Sanford A, Kun L, Gajjar (2001) A pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. Neurooncology 3:184–192

    CAS  Google Scholar 

  5. Giannini C, Scheithauer BW (1997) Classification and grading of low-grade astrocytic tumors in children. Brain Pathol 7:785–798

    CAS  PubMed  Google Scholar 

  6. Giannini C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, Lach B, O’Neill BP (1999) Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer 85:2033–2045

    CAS  PubMed  Google Scholar 

  7. Giannini C, Hebrink D, Scheithauer BW, Dei Tos AP, James CD (2001) Analysis of p53 mutation and expression in pleomorphic xanthoastrocytoma. Neurogenetics 3:159–162

    CAS  PubMed  Google Scholar 

  8. Kaulich K, Blaschke B, Numann A, von Deimling A, Wiestler OD, Weber RG, Reifenberger G (2002) Genetic alterations commonly found in diffusely infiltrating cerebral gliomas are rare or absent in pleomorphic xanthoastrocytomas. J Neuropathol Exp Neurol 61:1092–1099

    CAS  PubMed  Google Scholar 

  9. Kepes JJ (1993) Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. Brain Pathol 3:269–274

    CAS  PubMed  Google Scholar 

  10. Kepes JJ, Kepes M, Slowik F (1973) Fibrous xanthomas and xanthosarcomas of the meninges and the brain. Acta Neuropathol 23:187–199

    CAS  PubMed  Google Scholar 

  11. Kepes JJ, Rubinstein LJ, Eng LF (1979) Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. Cancer 44:1839–1852

    CAS  PubMed  Google Scholar 

  12. Kleihues P, Burger PC, Scheithauer BW (1993) The new WHO classification of brain tumours. Brain Pathol 3:255–268

    CAS  PubMed  Google Scholar 

  13. Leonard N, Alcutt DA, Farrell MA (1998) Fatal pleomorphic xanthoastrocytoma with meningeal gliomatosis. Histopathology 32:375–378

    Article  CAS  PubMed  Google Scholar 

  14. Lopes MBS, VandenBerg SR, Scheithauer BW (1993) The World Health Organization classification of nervous system tumors in experimental neuro-oncology. In: Levine AJ, Schmidek HH (eds) Molecular genetics of nervous system tumors, Wiley-Liss, New York, pp 1–36

  15. Pahapill PA, Ramsay DA, Del Maestro RF (1996) Pleomorphic xanthoastrocytoma: case report and analysis of the literature concerning the efficacy of resection and the significance of necrosis. Neurosurgery 38:822–829

    CAS  PubMed  Google Scholar 

  16. Paulus W, Lisle DK, Tonn JC, Wolf HK, Roggendorf W, Reeves SA, Louis DN (1996) Molecular genetic alterations in pleomorphic xanthoastrocytoma. Acta Neuropathol 91:293–297

    Article  CAS  PubMed  Google Scholar 

  17. Prayson RA, Morris HH (1998) Anaplastic pleomorphic xanthoastrocytoma. Arch Pathol Lab Med 122:1082–1086

    Google Scholar 

  18. Tsuyuguchi N, Matsuoka Y, Sunada I, Matsusaka Y, Haque M (2001) Evaluation of pleomorphic xanthoastrocytoma by use of positron emission tomography with. Am J Neuroradiol 22:311–313

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Benoit Pirotte.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lubansu, A., Rorive, S., David, P. et al. Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation. Childs Nerv Syst 20, 119–122 (2004). https://doi.org/10.1007/s00381-003-0854-6

Download citation

  • Revised:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-003-0854-6

Keywords

Navigation