Spinal cord glioneuronal tumor with neuropil-like islands with 1p/19q deletion in an adult with low-grade cerebral oligodendroglioma - PubMed (original) (raw)

Spinal cord glioneuronal tumor with neuropil-like islands with 1p/19q deletion in an adult with low-grade cerebral oligodendroglioma

Tyler J Fraum et al. J Neurooncol. 2012 Apr.

Abstract

Glioneuronal tumor with neuropil-like islands (GTNI) is considered a rare variant of astrocytoma, characterized by discrete aggregates of cells expressing neuronal markers that punctuate a GFAP-positive glial background. Of the 24 published GTNI cases, only two occurred in adult spinal cords; none occurred concurrent with another CNS tumor; and none of those tested exhibited the 1p/19q deletion typical of oligodendroglioma. A 48-year-old man without significant past medical history was diagnosed with a WHO grade II oligodendroglioma by stereotactic biopsy of a lesion discovered after the patient suffered a generalized tonic-clonic seizure. By FISH analysis, this tumor exhibited the 1p/19q deletion present in up to 80% of oligodendrogliomas. The patient received 14 monthly cycles of temozolomide, and his cerebral tumor had a minor response. When the patient subsequently reported progressive paresthesias of his lower extremities, an MRI revealed an enhancing, cystic tumor of the thoracic spinal cord that was diagnosed as GTNI by histological analysis. By FISH analysis, this lesion exhibited the same 1p/19q deletion present in the concurrent cerebral oligodendroglioma. This case of a spinal cord GTNI with 1p/19q deletions constitutes the third report of a spinal cord GTNI in an adult patient; the first report of a GTNI in an individual with a separate CNS neoplasm; and the first report of a GTNI with 1p/19q deletions. This case establishes a potential genetic kinship between GTNI and oligodendroglioma that warrants further investigation.

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Figures

Fig. 1

Fig. 1

Radiographic and histological characterization of the original cerebral lesion. T1-weighted post-gadolinium MRI a revealed a hypointense, nonenhancing mass of the right insula that appeared hyperintense on FLAIR images b, consistent with a low-grade glioma. Stereotactic biopsy of this lesion c revealed cells with round nuclei and perinuclear halos (‘fried-egg’ appearance), with a discrete amount of branching capillaries, consistent with a WHO grade II oligodendroglioma

Fig. 2

Fig. 2

Fluorescent in situ hybridization (FISH) showing presence of 1p/19q deletion in both cerebral and spinal cord lesions. Upper panel FISH probes targeting chromosome arms 1p (red) and 1q (green) show loss of one copy of the 1p arm among nuclei of tumor cells from the cerebral lesion. Similarly, probes targeting chromosome arms 19p (green) and 19q (red) show concurrent loss of one copy of the 19q arm among tumor cell nuclei from the same cerebral lesion. Lower panel FISH probes targeting chromosome arms 1p (red) and 1q (green) show loss of one copy of the 1p arm among nuclei of tumor cells from the spinal cord lesion. Similarly, probes targeting chromosome arms 19p (green) and 19q (red) show concurrent loss of one copy of the 19q arm among tumor cell nuclei from the same spinal cord lesion

Fig. 3

Fig. 3

Radiographic and histological characterization of spinal cord lesion. T1-weighted post-gadolinium MRI a revealed an intramedullary lesion at the T8-T9 level that demonstrated mild enhancement and a cystic component. The lesion was hyperintense on T2-weighted images b. Biopsy revealed a biphasic lesion consisting of a loose weave of fibrillary glial cells in association with neuropil-like islands c, with oligodendrocyte-like cells and mature-appearing neurons present around these islands d. The neuropil-like islands stained strongly for the neuronal marker synaptophysin (e), while immunohistochemistry for NeuN f labeled cells around the edges of these islands

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