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Rapid progression of pleomorphic xanthoastrocytoma into grade IV glioma
Author(s) -
Kaimaktchiev Vassil,
Korcheva Veselina,
Hughes Samuel,
Grafe Marjorie
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a394-c
Subject(s) - pleomorphic xanthoastrocytoma , pathology , medicine , glioma , temozolomide , pilocytic astrocytoma , astrocytoma , cancer research
We describe a case of unusually rapid progression of PXA into grade IV glioma. The patient was a 17 year old woman who underwent craniotomy for a fronto‐parietal tumor, diagnosed as PXA. She presented 6 months later with slurred speech, confusion, and radiographic progression. Repeat resection revealed recurrent/residual PXA and grade IV glioma with necrosis with pseudopalisading and endothelial proliferation. Ki‐67 labeling index was 10–15% in tumor that was morphologically PXA and > 80% in the grade IV areas. There was extensive reticulin deposition in PXA areas, but little in high grade areas. She underwent a 6 week course of radiation and temozolomide therapy, but subsequently developed headache, back pain, cranial nerve palsies and bilateral leg paresis. MRI showed extensive leptomeningeal enhancement along the spine. She died <1 yr after her initial presentation. Autopsy demonstrated a large necrotic, left fronto‐parietal tumor. No gross tumor was observed in spinal or cranial meninges. Microscopic examination revealed biphasic morphology with distinct areas of PXA architecture and glioblastoma. There was extensive microscopic tumor dissemination, including in subarachnoid spaces throughout bilateral cerebral hemispheres, cerebellum, brain stem, spinal cord and cauda equina. Molecular mechanisms associated with accelerated progression of PXA to high grade glioma are not yet known.