Possible differentiation of cerebral glioblastoma into pleomorphic xanthoastrocytoma: an unusual case in an infant

Case report

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The authors describe an infant girl who, at 10 months of age, presented with a large right parietooccipital tumor causing increased intracranial pressure, mass effect, and midline shift. The tumor was completely resected, and the entirety of the histology was consistent with glioblastoma. She was subsequently placed on adjuvant high-dose chemotherapy consisting of carboplatin, vincristine, and temozolomide, according to Head Start III, Regimen C. Three months after the complete resection, tumor recurrence was noted on MR imaging, during the third cycle of chemotherapy, and biopsy revealed malignant astrocytoma. Given the recurrence and the patient's intolerance to chemotherapy, a palliative course was pursued. Unexpectedly, the patient was alive and had made significant developmental improvements 18 months into palliation. Subsequently, however, signs of increased intracranial pressure developed and imaging demonstrated a very large new tumor growth at the site of prior resection. The recurrence was again fully resected, but microscopy surprisingly revealed pleomorphic xanthoastrocytoma throughout. The clinicopathological and genetic features of this girl's unusual neoplasm are detailed and potential pathogenic hypotheses are explored in this report.

Abbreviations used in this paper:EGB = eosinophilic granular body; GFAP = glial fibrillary acidic protein; GTR = gross-total resection; MVP = microvascular proliferation; PCR = polymerase chain reaction; PPN = pseudopallisading necrosis; PXA = pleomorphic xanthoastrocytoma.
Article Information

Contributor Notes

Address correspondence to: Ash Singhal, M.D., BC Children's Hospital, 4480 Oak Street, Room K3-159, Vancouver, British Columbia V6H 3V4, Canada. email: ash.singhal@cw.bc.ca.Please include this information when citing this paper: DOI: 10.3171/2012.1.PEDS11326.

© AANS, except where prohibited by US copyright law.

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