Glioma-like proliferation within tissues excised as tubers in patients with tuberous sclerosis complex

We describe diffuse glioma-like infiltrates in excised tubers in five out of forty Tuberous sclerosis complex (TSC) patients undergoing excision of a tuber at our institution within the last 10 years. All patients presented with refractory seizures. Resection specimens from four patients had the pat...

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Veröffentlicht in:Acta neuropathologica 2008-07, Vol.116 (1), p.67-77
Hauptverfasser: Fischer, Ingeborg, Cunliffe, Clare, Bollo, Robert J., Weiner, Howard L., Devinsky, Orrin, Ruiz-Tachiquin, Martha-Eugenia, Venuto, Toni, Pearlman, Alexander, Chiriboga, Luis, Schneider, Robert J., Ostrer, Harry, Miller, Douglas C.
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Sprache:eng
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Zusammenfassung:We describe diffuse glioma-like infiltrates in excised tubers in five out of forty Tuberous sclerosis complex (TSC) patients undergoing excision of a tuber at our institution within the last 10 years. All patients presented with refractory seizures. Resection specimens from four patients had the pathognomonic histologic features of neuroglial hamartomas (tubers) and in one case there was cortical microdysgenesis lacking cells typical of TSC. All lesions were associated with an infiltrate of atypical, mostly elongate, glioma-like small cells, which were immunoreactive for GFAP in three, and pS6 (a marker for activity of the mTOR pathway), in two cases. MAP-2 and CD34, were negative and MIB-1 (Ki67) immunostains ranged from
ISSN:0001-6322
1432-0533
DOI:10.1007/s00401-008-0391-1