|
|
|
|
|
|
||
|
|
||||
|
|
||||
|
||||
Pediatric Neuro-Oncology |
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 Research Hospital, 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 of Tennessee, 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 of Hematology-Oncology, St Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105-2794.
Abstract
To describe the clinical features, histologic characteristics, and management of patients with pleomorphic xanthoastrocytoma (PXA), we reviewed data on 13 children who had histologically confirmed PXA and were referred to the neuro-oncology service between 1985 and 1999. Neuro-imaging with CT and/or MRI documented the anatomic location, tumor extent, and degree of resection. There were 3 males and 10 females; median age was 12.9 years (range, 8.2-17.2 years). The most frequent presentations included seizures (n = 8) and headache (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 a cystic component in 6 patients, with cyst wall enhancement in 3 patients. The solid component was uniformly enhancing in 11 patients. Vasogenic edema was present 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-power field) were seen in 8 patients. Gross total resection was achieved in 8 patients, near total resection in 1, and subtotal resection in 4. Ten patients were alive with a median follow-up of 41 months at this writing. Two patients died of progressive disease, and 1 died of an unrelated cause. In conclusion, pleomorphic xanthoastrocytoma is a rare neoplasm in childhood, commonly presenting with seizures. Gross total resection without adjuvant therapy provides prolonged disease control, as seen in 6 of 7 patients (85%) in our series.
References
Allegranza, A., Ferraresi, S., Bruzzone, M., and Giombini, S. (1991) Cerebromeningeal pleomorphic xanthoastrocytoma: Report on four cases: Clinical, radiologic and pathologic features. (Including a case with malignant evolution). Neurosurg. Rev. 14, 43-49.[Web of Science][Medline]
Davies, K.G., Maxwell, R.E., Seljeskog, E., and Sung, J.H. (1994) Pleomorphic xanthoastrocytoma: Report of four cases, with MRI scan appearances and literature review. Br. J. Neurosurg. 8,681 -689.[Web of Science][Medline]
de Chadarevian, J.P., Pattisapu, J.V., and Faerber, E.N. (1990) Desmoplastic cerebral astrocytoma of infancy: Light microscopy, immunohistochemistry, and ultrastructure. Cancer 66,173 -179.[CrossRef][Web of Science][Medline]
Giannini, C., Scheithauer, B.W., Burger, P.C., Brat, D.J., Wollan, P.C., Lach, B., and O'Neill, B.P. (1999) Pleomorphic xanthoastrocytoma: What do we really know about it. Cancer 85,2033 -2045.[Web of Science][Medline]
Glasser, R.S., Rojiani, A.M., Mickle, J.P., and Eskin, T.A. (1995) Delayed occurrence of cerebellar pleomorphic xanthoastrocytoma after supratentorial pleomorphic xanthoastrocytoma removal: Case report. J. Neurosurg. 82,116 -118.[Web of Science][Medline]
Grant, J.W., and Gallagher, P.J. (1986) Pleomorphic xanthoastrocytoma: Immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am. J. Surg. Pathol. 10,336 -341.[CrossRef][Web of Science][Medline]
Herpers, M.J., Freling, G., and Beuls, E.A. (1994) Pleomorphic xanthoastrocytoma in the spinal cord: Case report. J. Neurosurg. 80,564 -569.[Web of Science][Medline]
Iwaki, T., Fukui, M., Kondo, A., Matsushima, T., and Takeshita, I. (1987) Epithelial properties of pleomorphic xanthoastrocytomas determined in ultrastructural and immunohistochemical studies. Acta Neuropathol. 74,142 -150.[CrossRef][Medline]
Jones, M.C., Drut, R., and Raglia, G. (1983) Pleomorphic xanthoastrocytoma: A report of two cases. Pediatr. Pathol. 1,459 -467.[Medline]
Kawano, N. (1992) Pleomorphic xanthoastrocytoma: Some new observations. Clin. Neuropathol. 11,323 -328.[Web of Science][Medline]
Kepes, J.J. (1993) Pleomorphic xanthoastrocytoma: The birth of a diagnosis and a concept. Brain Pathol. 3, 269-274.[Web of Science][Medline]
Kepes, J.J., Kepes, M., and Slowik, F. (1973) Fibrous xanthomas and xanthoastrocytomas of the meninges and the brain. Acta Neuropathol. 23,187 -199.[CrossRef][Medline]
Kepes, J.J., Rubinstein, L.J., and Eng, L.F. (1979) Pleomorphic xanthoastrocytoma: A distinctive meningocerebral glioma of young subjects with relatively favorable prognosis: A study of 12 cases. Cancer 44,1839 -1852.[CrossRef][Web of Science][Medline]
Kepes J.J., Rubinstein, L.J., Ansbacher, L., and Schreiber, D.J. (1989) Histopathological features of recurrent pleomorphic xanthoastrocytomas: Further corroboration of the glial nature of the neoplasm: A study of 3 cases. Acta Neuropathol. 78,585 -593.[CrossRef][Medline]
Kepes, J.J., Louis, D.N., and Paulus, W. (1997) Pleomorphic xanthoastrocytoma. In: Kleihues, P., and Cavenee, W.K. (Eds.), Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer. pp. 34-36.
Kleihues, P., Burger, P.C., and Scheithauer, B.W. (1993) Histological Typing of Tumours of the Central Nervous System. Second edition. World Health Organization. New York: Springer-Verlag. pp. 11-14.
Kleihues, P., and Cavenee W.K. (2000) Pathology and Genetics of Tumors of the Nervous System. Lyon, France: International Agency for Research on Cancer.
Kros, J.M., Vecht, C.J., and Stefanko, S.Z. (1991) The pleomorphic xanthoastrocytoma and its differential diagnosis: A study of five cases. Hum. Pathol. 22,1128 -1135.[CrossRef][Web of Science][Medline]
Kuhajda, F.P., Mendelsohn, G., Taxy, J.B., and Long, D.M. (1981) Pleomorphic xanthoastrocytoma: Report of a case with light and electron microscopy. Ultrastruct. Pathol. 2, 25-32.
Lach, B., Duggal, N., DaSilva, V.F., and Benoit, B.G. (1996) Association of pleomorphic xanthoastrocytoma with cortical dysplasia and neuronal tumors. Cancer 78,2551 -2563.[CrossRef][Web of Science][Medline]
Lindboe, C.F., Cappelen, J., and Kepes, J.J. (1992) Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma. Case report. J. Neurosurg. 31,353 -355.
Lipper, M.H., Eberhard, D.A., Phillips, C.D., Vezina, L.G., and Cail, W.S. (1993) Pleomorphic xanthoastrocytoma, a distinctive astroglial tumor: Neuroradiologic and pathologic features. AJNR Am. J. Neuroradiol. 14,1397 -1404.[Abstract]
Macaulay, R.J.B., Jay, V., Hoffman, H.J., and Becker, L.E. (1993) Increased mitotic activity as a negative prognostic indicator in pleomorphic xanthoastrocytoma. Case report. J. Neurosurg. 79,761 -768.[Web of Science][Medline]
Maleki, M., Robitaille, Y., and Bertrand, G. (1983) Atypical xanthoastrocytoma presenting as a meningioma. Surg. Neurol. 20,235 -238.[CrossRef][Web of Science][Medline]
Packer, R.J., Sutton, L.N., Patel, K.M., Duhaime, A.C., Schiff, S., Weinstein, S.R., Gaillard, W.D., Conry, J.A., and Schut, L. (1994) Seizure control following tumor surgery for childhood cortical low-grade gliomas. J. Neurosurg. 80,998 -1003.[Web of Science][Medline]
Papahill, P.A., Ramsay, D.A., and Del Maestro, R.F. (1996) Pleomorphic xanthoastrocytoma: Case report and analysis of the literature concerning the efficacy of resection and the significance of necrosis. Neurosurgery 38,822 -828.[Web of Science][Medline]
Paulus, W., and Peiffer, J. (1988) Does the pleomorphic xanthoastrocytoma exist? Problems in the application of immunological techniques to the classification of brain tumors. Acta Neuropathol. 76,245 -252.[CrossRef][Medline]
Paulus, W., and Peiffer, J. (1991) History, histology, histochemistry and histiocytic histogenesis of the pleomorphic xanthoastrocytoma. Brain Tumor Pathol. 8, 67-71.
Powell, S.Z., Yachnis, A.T., Rorke, L.B., Rojiani, A.M., and Eskin, T.A. (1996) Divergent differentiation in pleomorphic xanthoastrocytoma: Evidence for a neuronal element and possible relationship to ganglion cell tumors. Am. J. Surg. Pathol. 20, 80-85.[CrossRef][Web of Science][Medline]
Tien, R.D., Cardenas, C.A., and Rajagopalan, S. (1992)
Pleomorphic xanthoastrocytoma of the brain: MR findings in six patients.
AJR Am. J. Roentgenol.
159,1287
-1290.
Yoshino, M.T., and Lucio, R. (1992) Pleomorphic xanthoastrocytoma. AJNR Am. J. Neuroradiol. 13,1330 -1332.[Abstract]
Zorzi, F., Faccti, F., Baronchelli, C., and Cani, E. (1992) Pleomorphic xanthoastrocytoma: An immunohistochemical study of three cases. Histopathology 20,267 -269.[Web of Science][Medline]
CiteULike 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  T. Koga, A. Morita, K. Maruyama, M. Tanaka, Y. Ino, J. Shibahara, D. N. Louis, G. Reifenberger, J. Itami, R. Hara, et al. Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation Neuro-oncol, January 1, 2009; 11(4): 446 - 451. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Broniscer, S. J. Baker, A. N. West, M. M. Fraser, E. Proko, M. Kocak, J. Dalton, G. P. Zambetti, D. W. Ellison, L. E. Kun, et al. Clinical and Molecular Characteristics of Malignant Transformation of Low-Grade Glioma in Children J. Clin. Oncol., February 20, 2007; 25(6): 682 - 689. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|
|
