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International Journal of Advances In Case Reports

Volume 11, Issue 1, 2024
Mcmed International
International Journal of Advances In Case Reports
Issn
XXX-XXXX (Print), 2349 - 8005 (Online)
Frequency
bi-annual
Email
editorijacr@mcmed.us
Journal Home page
http://mcmed.us/about/ijacr
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Abstract
Title
PLEOMORPHIC XANTHOASTROCYTOMA IN A PATIENT WITH NEUROFIBROMATOSIS TYPE I
Author
Umesh Phalke, Sarang Gotecha*, Mangesh Sagale, Pravin Ganjare, Prashant Punia, Shrikant Sharma
Email
dr.sarangsgotecha@gmail.com
keyword
Pleomorphic Xanthoastrocytoma, neurofibromatosis, Left temporal lesion, India.
Abstract
Pleomorphic xanthoastrocytoma (PXA), a new addition to the recent 1993 World Health Organization (WHO) classification of tumors of the central nervous system, was first described in 1973 is a rare neoplasm that accounts for 1% of all astrocytic tumors. Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with complete penetrance involving the chromosomal 17q11.2 gene locus. The first case of cerebral PXA-NF1, that is, PXA in association with NF1, was reported in 1993. Since then, only very few other cases have been documented, only 9 cases were reported till 2012. 40 year old male patient presented with progressive right sided weakness, slurred speech and severe headache since 10 days. Examination revealed multiple subcutaneous neurofibromas and café au lait spots all over the body. Neurological examination was also consistent with right sided weakness with no other neurological deficit. Computed tomography of the brain revealed large hypodense lesion in left frontal lobe. Left frontal craniotomy and excision of the mass lesion was done. Histopathological examination and Immunohistochemistry were suggestive of Pleomorphic xanthoastrocytoma. The first association of PXA with NF1 was reported in 1993 and 9 previously well documented cases of PXA-NF1 have been reported in the global literature of which only 2 cases have been reported in Asia and this is the first reported case in India. In a slow growing tumor such as PXA, longer period of follow up will be essential. Post-operative adjuvant therapy is required in patients with plastic changes in histopathology.
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