Pleomorphic xanthoastrocytoma and oligodendroglioma: collision of 2 morphologically and genetically distinct anaplastic components

Case report

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With the exception of oligoastrocytoma, mixed gliomas are rarely encountered, and the astrocytic component of mixed oligoastrocytoma is almost always fibrillary and diffusely infiltrative. Pleomorphic xanthoastrocytoma (PXA) has occasionally been described in conjunction with ganglioglioma, as well as in 1 case of oligodendroglioma. In this latter case, described by Perry et al., 1p/19q codeletions were not detected.

The authors report on a 25-year-old woman with a combined PXA/oligodendroglioma in which concurrent 1p/19q codeletions were detected in the oligodendroglial component only. The patient presented with a 1-month history of headaches. Neuroimaging revealed a heterogeneous left temporal mass with focal enhancement, cystic changes, hemorrhage, and left-to-right midline shift. The patient underwent a craniotomy and gross-total resection. Pathological examination revealed a glial tumor composed of 2 apparently distinct components. The largest component exhibited a prominent fascicular, reticulin-rich, spindle cell arrangement admixed with areas of highly pleomorphic cells, with bizarre cytological features reminiscent of PXA. A smaller component was composed of cellular sheets and lobules of oligodendroglial cells. Both components were characterized by anaplastic features. Dual-color fluorescence in situ hybridization for 1p/19q codeletions was performed. Only the oligodendroglial component showed the combined 1p/19q deletions.

This case represents the first instance in which PXA has been reported in conjunction with an oligodendroglioma exhibiting the “molecular signature” characteristic of oligodendroglial neoplasms. The different genetic alterations seen in the 2 components of this neoplasm argue in favor of a “collision tumor” rather than a mixed glioma of the same genotype.

Abbreviations used in this paper: CEP = centromeric enumeration probe; EGFR = epidermal growth factor receptor; FISH = fluorescence in situ hybridization; GFAP = glial fibrillary acidic protein; PXA = pleomorphic xanthoastrocytoma.

Article Information

Address correspondence to: Eyas M. Hattab, M.D., Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 350 West 11th Street, CPL 4014, Indianapolis, Indiana 46202. email: ehattab@iupui.edu.

Please include this information when citing this paper: published online January 7, 2011; DOI: 10.3171/2010.11.JNS10739.

© AANS, except where prohibited by US copyright law.

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Figures

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    Head MR imaging revealing a heterogeneous mass in the left temporal lobe. The tumor showed lobular, predominantly anterior enhancement and a cystic component. A–C: The T1-weighted sequences obtained with Gd contrast in axial, sagittal, and coronal sections, respectively. Note the compressed left lateral ventricle and the slight midline shift. D: An axial T2-weighted image showing extensive vasogenic edema extending to the left basal ganglia and internal capsule.

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    Photomicrographs showing the PXA component. This component was characterized by a fascicular arrangement of spindled cells (A) as well as aggregates of xanthomatous cells and large, highly pleomorphic cells with pseudonuclear inclusions (B). Note the remarkable ability of this tumor to infiltrate the subarachnoid and perivascular spaces (C). Eosinophilic granular bodies were numerous (D), and the tumor had foci of vascular proliferation (E) and palisading necrosis (F). The spindled cells were rich in pericellular reticulin (G) and showed intense GFAP positivity (H). The MIB-1 proliferative index highlighted approximately 5%–8% of the cells (I). H & E (A–F). Original magnification × 200 (F), × 250 (A–C and E), and × 500 (D and G–I).

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    Photomicrographs showing the oligodendroglioma component. This component demonstrated cellular sheets of tumor cells decorated by abundant “chicken-wire” vessels and microcysts (A and B). Note the relative uniformity of the tumor cells, their characteristically round nuclear contours, and mitotic figures (C). Scattered large, atypical, lipidized astrocytic cells like those seen in the PXA component were present (D). The cells demonstrated a total lack of pericellular reticulin (E), and showed a high MIB-1 proliferation index (F). H & E (A–D). Original magnification × 100 (A), × 250 (E), × 400 (D), × 500 (B and F), and × 800 (C).

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    Dual-color FISH analysis demonstrated loss of chromosomal arm 1p (red) (A) and loss of chromosomal arm 19q (red) (B) in the oligodendroglial component. The pleomorphic xanthoastrocytoma cells showed extensive 19q (red) polysomic change (C).

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