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.

Recurrent Pleomorphic Xanthoastrocytoma Presenting With Diffuse Leptomeningeal Spread

“…Although PXA is often associated with a favorable prognosis, recurrence with anaplastic transformation has been associated with leptomeningeal dissemination and worse outcomes. 1,2 Among gliomas, both delayed recurrence and leptomeningeal spread of disease are phenomena to be aware of, with recent estimates for the incidence of leptomeningeal metastases to be as high as 4.7% among patients with glioma. 3 This case highlights key clinical, CSF, and imaging findings of recurrent PXA with leptomeningeal spread.…”

Intravenous Fluorescein Sodium for Resection of Pleomorphic Xanthoastrocytoma: 2-Dimensional Operative Video

“…1 They are characterized by heterogeneous morphology and molecular alterations, conferring high potential to recur and suffer malignant transformation. 2 Although standardized treatment is not well established, gross total resection (GTR) is associated with increased overall survival (OS) and progression-free survival (PFS), making maximal extent of resection crucial. 3 Recently, fluorescence-guided surgery has become widely used to maximize extent of resection.…”