1/15/2008 Case 3: PXA with gangliogliotic differentiation, focal antiomatous areas and hemorrhage

Presented by: Dr. Rao - University Hospital SUNY HSCB

Clinical History:

Case of a 10 year old female with a partially cystic tumor in the frontal lobe, who underwent total resection in Jamaica.  The oncologist requested review of the slides prior to radiotherapy.  Slides showed a pleomorphic astrocytic tumor with vacuolated cells, EGB and obvious neoplastic neurons. 

Diagnostic Notes:

The diagnosis was pleomorphic xanthoastrocytoma with ganglionic differentiation, focal angiomatous areas and focal intratumoral hemorrhage (WHO II).  This is a “PXA-Plus.”  The entity may include composite tumors with elements of DNT, ganglioglioma and ATRT.  PXA may exist as a component of ganglioglioma, or with a glioneuronal malformation.  If a PXA becomes anaplastic, i.e has 5 or more mitoses per 10 HPF) and/or has necrosis, the proper term is “pleomorphic xanthoastrocytoma with anaplastic features” (WHO classification, 2007).


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