Brain Pathology Case of the Month - March 2006
Ganglion cell tumors (GCT) are rather common low grade neoplasms found in patients with epilepsy and representing up to 1.3% of brain tumors (5). Over 80% of tumors are discovered during the first three decades of life with a male-to-female ratio of 1.1:1-1.9:1 (3), (7). Many of the tumors arise in the temporal lobe, but other sites are not spared (1). A classic cyst with an enhancing mural nodule appearance simplifies the diagnostic challenge (8), effectively limiting the differential diagnosis to such entities as pilocytic astrocytoma, pleomorphic xanthoastrocytoma (PXA), hemangioblastoma, extraventricular neurocytoma and ependymoma. PXA and GCT share not only desmoplasia, but also a variable proportion of a neuronal component (2). PXA differs in having mostly fascicular architecture, presence of xanthic cells and extreme nuclear pleomorphism. The prognosis of GCT is generally favorable, except for a fraction of neoplasms featuring a component of frankly anaplastic astrocytes. Even in such cases the outlook mostly depends on the extent of surgical removal (6). Both neural and astrocytic components are clonal (9), but the histogenesis of GCT is unclear. Frequent association of GCT with focal microdysgenesis suggests a dysembryoplastic component (4).
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Contributed by Jorge E. Dotto, Joachim Baehring, Joseph M. Piepmeier, Sergei I. Bannykh