Pleomorphic xanthoastrocytoma is an indolent (WHO grade I) tumor usually diagnosed in children and young adults. Location is most often temporal, and chronic epilepsy is the most common presenting symptom. It typically appears on imaging as an enhancing lesion with a cystic component in a superficial location. Tissue pathology is very aggressive in appearance, which is in sharp contrast with the typical benign clinical course seen in most cases. Treatment is with resection; other treatments are rarely warranted. Dr. Mintzer, an epilepsy specialist at Thomas Jefferson University, describes the characteristics of this unusual neoplasm, which is of equal interest to both the epileptologist and neuro-oncologist. Although this tumor type is rare, its appropriate diagnosis has considerable bearing on patient prognosis and is, therefore, crucial to avoid inappropriate patient management.
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