BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is a rare clinicopathologic entity that occurs typically in young patients. Improved neuroradiologic techniques, especially gadolinium-enhanced magnetic resonance imaging (MRI), reveal a characteristic tumor appearance. METHODS: We present six cases of PXA operated on with unusual clinical course, elucidating different clinical implications. RESULTS: Two patients showed subsequent progression into a malignant glioma, one case was a primary anaplastic PXA. The latter case had not previously been reported in the literature. Increased mitotic activity seems to indicate a worse clinical course; whereas focal infiltration of the brain does not necessarily lead to malignant transformation. CONCLUSIONS: Surgical removal is the treatment of choice. A consequent follow-up is mandatory in order to detect a potentially malignant recurrence as early as possible and to select patients who need additional therapy.
BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is a rare clinicopathologic entity that occurs typically in young patients. Improved neuroradiologic techniques, especially gadolinium-enhanced magnetic resonance imaging (MRI), reveal a characteristic tumor appearance. METHODS: We present six cases of PXA operated on with unusual clinical course, elucidating different clinical implications. RESULTS: Two patients showed subsequent progression into a malignant glioma, one case was a primary anaplastic PXA. The latter case had not previously been reported in the literature. Increased mitotic activity seems to indicate a worse clinical course; whereas focal infiltration of the brain does not necessarily lead to malignant transformation. CONCLUSIONS: Surgical removal is the treatment of choice. A consequent follow-up is mandatory in order to detect a potentially malignant recurrence as early as possible and to select patients who need additional therapy.
Authors: Nawal Shaikh; Nupur Brahmbhatt; Tim J Kruser; Kwok L Kam; Christina L Appin; Nitin Wadhwani; James Chandler; Priya Kumthekar; Rimas V Lukas Journal: CNS Oncol Date: 2019-09-19