7/10/2007 Case 1: Progenitor cell glioma


Clinical History:

A 24 yr old man presented with severe headache on April 19th 2006. Initial imaging revealed a left frontal hemorrhagic mass associated with edema and midline shift which was resected on April 25, 2006. Slides were evaluated at three medical centers with similar impressions. He was next seen at RWJUH on June 6th of 2006. His neurological exam was reported as unremarkable. He was treated with external beam radiation therapy and temozolomide in summer of 2006, but his tumor progressed and he developed increasing seizures and a right hemiparesis. He was seen again at RWJUH in December 2006 with headache, fatigue, seizures, and worsening memory and cognition. Imaging in late 2006 revealed a large left frontal mass with extension through the orbital roof into the left ethmoid sinus. An extra-axial mass was present over the left parietal lobe, with surrounding edema.

He had a biopsy of the "new" lesion on 12/22/06 at RWJUH. The surgeon reported tumor infiltration of the calvarium. Slide 1 from original biopsy. Slide 2 from second biopsy of the new "discrete" lesion.

Diagnostic Notes:

The first tumor was clearly a high grade glioma. Many present thought that a significant proportion of the cells resembled "oligodendroglioma", and apparently Dr Marc Rosenblum gave a diagnosis for this case of Anaplastic Oligodendroglioma; in contrast others were less sure this wasn't astrocytic, and Dr Peter Burger allegedly called it just "high grade glioma". The second specimen had much more sarcoma-like features, and in fact was so diagnosed. Some in the Neuroplex thought it was a gliosarcoma. Immunostains demonstrated at the conference showed at least focal positivity for vimentin, GFAP, EGFR, and p53 in both tumor specimens. An immunostain for S100 protein was positive only in the second specimen. Stains for desmin, myogenin, smooth muscle actin, neurofilament protein, synaptophysin, EMA, pan-cytokeratin, CD34, and CD57 were all negative (although my notes indicate that there were some few cells positive for synaptophysin and for EMA).

The ultimate diagnosis then was given officially as sarcoma but most in the Neuroplex seem to think this was a gliosarcoma arising in the previous anaplastic glioma.

The tumor from the spine in 2007 also had a majority component of small to medium-size cells with round nuclei centrally placed in the cell bodies, but this tumor was ~ar more cellular, with more nuclear hyperchromatism. There were also minigemistocytes. There was considerable pleiomorphism, and there were obvious mitotic figures. The tumor grew in the subarachnoid space "and infiltrated adjacent nerve roots. Immunostains showed a similar phenotype as with the brain tumor, with a few GFAP-positive cells and some Neu-N positive cells but no cells positive for synaptophysin or neurofilament protein. A MIBl now labeled at least 50% of cells. It was concluded that this was a metastatic deposit from the primary brain tumor, and the tumor was now an Anaplastic Glioneurocytoma. Some in the audience raised the differential diagnosis for this second lesion of PNET given the high cell density in foci, but most thought the cells too large and differentiated in appearance.

Slide Image:

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