© 2001 by Society
© 2001 by the Society forNeuro-Oncology
Pleomorphic xanthoastrocytoma: Favorable outcome after complete surgicalresection
Departments of Hematology-Oncology (M.F., R.H., S.T.,A.G.), Radiation Oncology (T.M., L.K.), Pathology and Laboratory Medicine (J.J.), and Diagnostic Imaging (J.L.), St. Jude Children's ResearchHospital, Memphis, TN 38105; Departments of Pediatrics(M.F., R.H., S.T., A.G.), Radiation Oncology(L.K.), Radiology (J.L.), and the Division of Pediatric Neurosurgery (A.S.), University ofTennessee, College of Medicine, Memphis, TN 38105; and the Department of Pathology, Johns Hopkins Hospital, Baltimore,MD 21287 (P.B.)
2 Address correspondence and reprint requests to Amar Gajjar, Department ofHematology-Oncology, St Jude Children's Research Hospital, 332 NorthLauderdale, Memphis, TN 38105-2794.
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Abstract |
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To describe the clinical features, histologic characteristics, andmanagement of patients with pleomorphic xanthoastrocytoma (PXA), we revieweddata on 13 children who had histologically confirmed PXA and were referred tothe neuro-oncology service between 1985 and 1999. Neuro-imaging with CT and/orMRI documented the anatomic location, tumor extent, and degree of resection.There were 3 males and 10 females; median age was 12.9 years (range, 8.2-17.2years). The most frequent presentations included seizures (n = 8) andheadache (n = 5). Tumor sites included temporal (n = 5),parietal (n = 3), frontal (n = 1), frontoparietal(n = 1), parietooccipital (n = 1), and temporoparietal(n = 1) lobes and the spinal cord (n = 1). CT/MRI revealed acystic component in 6 patients, with cyst wall enhancement in 3 patients. Thesolid component was uniformly enhancing in 11 patients. Vasogenic edema waspresent in 9 patients, and calcification was noted in 4 patients.Histopathologic findings included meningeal invasion in 12 patients,calcifications in 4, and necrosis in 2. Mitotic figures (1-12 per high-powerfield) were seen in 8 patients. Gross total resection was achieved in 8patients, near total resection in 1, and subtotal resection in 4. Ten patientswere alive with a median follow-up of 41 months at this writing. Two patientsdied of progressive disease, and 1 died of an unrelated cause. In conclusion,pleomorphic xanthoastrocytoma is a rare neoplasm in childhood, commonlypresenting with seizures. Gross total resection without adjuvant therapyprovides prolonged disease control, as seen in 6 of 7 patients (85%) in ourseries.
Received December 7, 2000; Accepted February 28, 2001
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