Pleomorphic xanthoastrocytoma

Scott Mintzer MD (Dr. Mintzer of Thomas Jefferson University has no relevant financial relationships to disclose.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released February 16, 1999; last updated February 17, 2015; expires February 17, 2018

This article includes discussion of pleomorphic xanthoastrocytoma, xanthoastrocytoma, PXA. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Dr. Mintzer, an epilepsy specialist, 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.

Key points

 

• 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.

Historical note and terminology

Pleomorphic xanthoastrocytoma is a rare variant of low-grade astrocytoma. The first cases were reported in 1973 as meningocerebral fibrous xanthomas, which were presumed to be of mesenchymal origin (Kepes et al 1973). In 1978, the discovery of the astrocytic marker GFAP allowed Kepes and coworkers to reevaluate these previously reported cases, along with several new ones, and to confirm their astrocytic origin. Their subsequent report of 12 patients with pleomorphic xanthoastrocytoma was the first to systematically describe its features and to name this new entity (a rather cumbersome name that reflects its most salient pathologic characteristics) (Kepes et al 1979). An evaluation of a tumor excised in 1930 from a patient who survived for 40 years has yielded what is apparently the earliest known example of this tumor (Geddes and Swash 1999). It is a rare neoplasm, with just over 200 cases reported in the medical literature to date. Nonetheless, the recognition of this tumor is critical for patient care because of its relatively benign prognosis and the consequent implications for management.

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