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Pleomorphic xanthoastrocytoma – a clinico-pathological review
Affiliations- Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, United States
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Abstract
Pleomorphic xanthoastrocytoma (PXA) is a rare primary brain tumour which occurs in children and young adults. PXA is designated as low-grade astrocytoma (WHO II), although an anaplastic variant and malignant potential have been described. Pathologically, cellular pleomorphism is the rule and includes spindle cells, mononucleated and multinucleated giant cells, and granular bodies in a reticulin-rich background with few mitoses; necrosis is usually absent. The tumour cells stain positive for glial fibrillary acidic protein (GFAP).
Surgery is the mainstay of treatment with gross surgical resection being accomplished due to its well-circumscribed nature and peripheral location. The role of adjuvant treatment remains yet to be clearly defined. To date, the majority of PXAs have been reported as single or small case series; consequently data of this rare brain tumour are fragmentary. The present paper reviews the pathogenesis, neuroradiological features, prognostic factors and treatment options for PXA.
Abstract
Pleomorphic xanthoastrocytoma (PXA) is a rare primary brain tumour which occurs in children and young adults. PXA is designated as low-grade astrocytoma (WHO II), although an anaplastic variant and malignant potential have been described. Pathologically, cellular pleomorphism is the rule and includes spindle cells, mononucleated and multinucleated giant cells, and granular bodies in a reticulin-rich background with few mitoses; necrosis is usually absent. The tumour cells stain positive for glial fibrillary acidic protein (GFAP).
Surgery is the mainstay of treatment with gross surgical resection being accomplished due to its well-circumscribed nature and peripheral location. The role of adjuvant treatment remains yet to be clearly defined. To date, the majority of PXAs have been reported as single or small case series; consequently data of this rare brain tumour are fragmentary. The present paper reviews the pathogenesis, neuroradiological features, prognostic factors and treatment options for PXA.
Keywords
brain tumour, pleomorphic xanthoastrocytoma, review
About this articleTitle
Pleomorphic xanthoastrocytoma – a clinico-pathological review
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
379-386
Page views
351
Article views/downloads
1049
DOI
10.1016/S0028-3843(14)60109-2
Bibliographic record
Neurol Neurochir Pol 2011;45(4):379-386.
Keywords
brain tumour
pleomorphic xanthoastrocytoma
review
Authors
Aman Sharma
Daya Nand Sharma
Parmod Kumar Julka
Goura Kishor Rath
