Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls

Abstract

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.