Skip to main content
Log in

Pleomorphic xanthoastrocytoma as a component of a temporal lobe cystic ganglioglioma: a case report

  • Case Report
  • Published:
Brain Tumor Pathology Aims and scope Submit manuscript


We report a case of pleomorphic xanthoastrocytoma (PXA) as a component of a ganglioglioma in a 13-year-old Japanese boy. Magnetic resonance imaging showed a large cystic lesion with an enhanced mural nodule of the left temporal lobe. Microscopic examination of the tumor showed that it was composed of two distinct neoplastic components: dysplastic ganglion cells and a PXA. There were gradual transitions between the two neoplastic components, and the PXA constituted the gliomatous component of the ganglioglioma. The PXA component showed spindle-shaped and pleomorphic large cells with lipidized cytoplasm. The tumor cells were surrounded by numerous reticulin fibers. Immunohistochemically, the ganglion cells were negative for glial fibrillary acidic protein (GFAP), but showed positive staining for a 70-kDa neurofilament protein, synaptophysin, and NeuN. In contrast, PXA cells were positive for GFAP but negative for neuronal markers. Our case is therefore histologically classified as ganglioglioma with PXA as the glial component. These results suggested that PXA and ganglioglioma share a common origin and that the combination of PXA-ganglioglioma would be positioned along the spectrum between PXA and ganglioglioma. Alternatively, these results may support the hypothesis that PXA originates from glioneuronal progenitor cells capable of generating astrocytic and neuronal cell types.

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.

Similar content being viewed by others


  1. 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 (Phila) 44:1839–1852

    Article  CAS  Google Scholar 

  2. Giannini C, Scheithauer BW, Burger PC, Brat DJ, Wollan PC, Lach B, O’Neill BP (1998) Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer (Phila) 85:2033–2045

    Google Scholar 

  3. Kros JM, Vecht CJ, Stefanko SZ (1991) The pleomorphic xanthoastrocytoma and its differential diagnosis:a study of five cases. Hum Pathol 22:1128–1135

    Article  PubMed  CAS  Google Scholar 

  4. Furuta A, Takahashi H, Ikuta F, Onda K, Takeda N, Tanaka R (1992) Temporal lobe tumor demonstrating ganglioglioma and pleomorphic xanthoastrocytoma components. J Neurosurg 77: 143–147

    Article  PubMed  CAS  Google Scholar 

  5. Lindboe CF, Cappelen J, Kepes JJ (1992) Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report. Neurosurgery 31:353–355

    Article  PubMed  CAS  Google Scholar 

  6. Kordek R, Biernat W, Sapieja W, Alwasiak J, Liberski PP (1995) Pleomorphic xanthoastrocytoma with a gangliomatous component: an immunohistochemical and ultrastructural study. Acta Neuropathol 89:194–197

    Article  PubMed  CAS  Google Scholar 

  7. Lach B, Duggal N, DaSilva VF, Benoit BG (1996) Association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. a report of three cases. Cancer (Phila) 78:2551–2563

    Article  CAS  Google Scholar 

  8. Perry A, Giannini C, Scheithauer BW, Rojiani A, Yachnis A, Seo S, Johnson P, Kho J, Shapiro S (1997) Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature. Am J Surg Pathol 21:763–771

    Article  PubMed  CAS  Google Scholar 

  9. Vajtai I, Varga Z, Aguzzi A (1997) Pleomorphic xanthoastrocytoma with gangliogliomatous component. Pathol Res Pract 193: 617–621

    PubMed  CAS  Google Scholar 

  10. Hessler RB, Kfoury H, Al-Watban J, Hassounah M (1999) Angiomatous pleomorphic xanthoastrocytoma as a component of ganglioglioma. Ann Saudi Med 19:48–51

    PubMed  CAS  Google Scholar 

  11. Evans AJ, Fayaz I, Cusimano MD, Laperriere N, Bilbao JM (2000) Combined pleomorphic xanthoastrocytoma and ganglioglioma of the cerebellum. Arch Pathol Lab Med 124:1707–1709

    PubMed  CAS  Google Scholar 

  12. Ebato M, Tsunoda A, Maruki C, Ikeya F, Okada M (2002) Distinctive pleomorphic xanthoastrocytoma-like tumor with exclusive abortive or aberrant neuronal differentiation and repeated recurrence: case report. Neurol Med Chir (Tokyo) 42:399–405

    Article  Google Scholar 

  13. Yeh DJ, Hessler RB, Stevens EA, Lee MR (2003) Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report. Neurosurgery 52:1465–1469

    Article  PubMed  Google Scholar 

  14. Giannini C, Paulus W, Louis DN, Liberski P (2007) Pleomorphic xanthoastrocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) Pathology and genetics of tumours of the nervous system. International Agency for Research on Cancer Press, Lyon, pp 22–24

    Google Scholar 

  15. Becker AL, Wiestler OD, Figarella-Branger D, Blümcke I (2007) Ganglioglioma and gangliocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) Pathology and genetics of tumours of the nervous system. International Agency for Research on Cancer Press, Lyon, pp 103–105

    Google Scholar 

  16. Blümcke I, Wiestler OD (2002) Gangliogliomas: an intriguing tumor entity with focal epilepsies. J Neuropathol Exp Neurol 61:575–584

    PubMed  Google Scholar 

  17. Powell S, Yachnis A, Rorke LB, Rojiani AM, Eskin T (1996) Divergent differentiation in pleomorphic xanthoastrocytoma: evidence for a neuronal element and possible relation to ganglion cell tumors. Am J Surg Pathol 20:80–85

    Article  PubMed  CAS  Google Scholar 

  18. Hirose T, Giannini C, Scheithauer BW (2001) Ultrastructural features of pleomorphic xanthoastrocytoma: a comparative study with glioblastoma multiforme. Ultrastruct Pathol 25:469–478

    Article  PubMed  CAS  Google Scholar 

  19. Reifenberger G, Kaulich K, Wiestler OD, Blümcke I (2003) Expression of the CD34 antigen in pleomorphic xanthoastrocytomas. Acta Neuropathol 105:358–364

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Yasuo Sugita.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sugita, Y., Irie, K., Ohshima, K. et al. Pleomorphic xanthoastrocytoma as a component of a temporal lobe cystic ganglioglioma: a case report. Brain Tumor Pathol 26, 31–36 (2009).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI:

Key words