Abstract
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.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
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
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
Kros JM, Vecht CJ, Stefanko SZ (1991) The pleomorphic xanthoastrocytoma and its differential diagnosis:a study of five cases. Hum Pathol 22:1128–1135
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
Lindboe CF, Cappelen J, Kepes JJ (1992) Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report. Neurosurgery 31:353–355
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
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
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
Vajtai I, Varga Z, Aguzzi A (1997) Pleomorphic xanthoastrocytoma with gangliogliomatous component. Pathol Res Pract 193: 617–621
Hessler RB, Kfoury H, Al-Watban J, Hassounah M (1999) Angiomatous pleomorphic xanthoastrocytoma as a component of ganglioglioma. Ann Saudi Med 19:48–51
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
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
Yeh DJ, Hessler RB, Stevens EA, Lee MR (2003) Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report. Neurosurgery 52:1465–1469
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
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
Blümcke I, Wiestler OD (2002) Gangliogliomas: an intriguing tumor entity with focal epilepsies. J Neuropathol Exp Neurol 61:575–584
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
Hirose T, Giannini C, Scheithauer BW (2001) Ultrastructural features of pleomorphic xanthoastrocytoma: a comparative study with glioblastoma multiforme. Ultrastruct Pathol 25:469–478
Reifenberger G, Kaulich K, Wiestler OD, Blümcke I (2003) Expression of the CD34 antigen in pleomorphic xanthoastrocytomas. Acta Neuropathol 105:358–364
Author information
Authors and Affiliations
Corresponding author
Rights 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). https://doi.org/10.1007/s10014-009-0244-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10014-009-0244-1