Rapid Diffuse Leptomeningeal Dissemination of an Anaplastic Pleomorphic Xanthoastrocytoma in an Adult Patient

Abstract

Although pleomorphic xanthoastrocytomas generally carry a fair prognosis, anaplastic transformation has been identified in a subset of cases.We present the case of a patient with primary anaplastic pleomorphic xanthoastrocytoma (APXA) that demonstrated rapid recurrence and diffuse leptomeningeal spread of disease three months postoperatively, causing severe visual impairment and acute ischemic strokes leading to death.We believe this is the fastest reported time to leptomeningeal dissemination and death from the initial diagnosis. Through this case, we show how anaplastic features can have a highly variable biological effect on disease progression. We believe earlier craniospinal imaging at the time of APXA diagnosis should be pursued to manage disease progression more aggressively with currently recommended adjuvant therapies.

Keywords: anaplastic; anaplastic pleomorphic xanthoastrocytoma; brain tumor; craniospinal dissemination; leptomeningeal dissemination; neurosurgery.