7/10/2004 Case 3: Anaplastic ganglioneurocytoma with focal granulomas

Clinical History:

This was a recurrent tumor in a man first diagnosed in 1994 with a right parieto-temporal lesion. He was asymptomatic when this was first discovered, and he was followed by CT and MRI scans. In 1995 he developed seizures and was started on Tegretol; ultimately in 1998 he had surgery, a stereotactic volumetric subtotal resection of a 6 x 5 x 6 cm tumor. The occipital portion of the tumor had recurrent growth requiring a second excision in 2000; now in 2004 he has required resection of a temporal lobe recurrence.

Diagnostic Notes:

The slides showed a neuroepithelial tumor with anaplastic features (vascular hyperplasia, necrosis, mitotic activity) but with an overall "oligodendroglioma"-like cytomorphology. Immunostains demonstrated some neuronal differentiation, as had been seen in the two earlier resection specimens as well. The diagnosis was one of "Anaplastic Glioneurocytoma", but this was not why the case was presented. Scattered throughout the specimen were mu~tiple necrotizing granulomas, complete with epithelioid histiocytes and multinucleated giant cells. Starns for organisms were all negative. A re-examination of the earlier specimens showed at least one such granuloma in the original 1998 resection specimen. Dr Miller asked if anyone else had cases of intra-tumoral granulomas in a glioma or similar CNS parenchymal neoplasm. He noted that he had a case of a man with multiple Schwannomatosis, in whom each resected Schwannoma also had such granulomas, also with negative stains for organisms .

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