CNS tumor
Other astrocytic tumors
Pleomorphic xanthoastrocytoma


Topic Completed: 1 March 2012

Minor changes: 25 September 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Pleomorphic xanthoastrocytoma CNS

Eman Abdelzaher, M.D., Ph.D.
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Cite this page: Abdelzaher E. Pleomorphic xanthoastrocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorpleomorphicxanthoastrocytoma.html. Accessed October 28th, 2020.
Definition / general
  • Astrocytic neoplasm with relatively favorable prognosis, typically in children and young adults
  • First described in 1979 (Cancer 1979;44:1839)
  • WHO grade II
  • May be a developmental neuroglial tumor with prominent glioproliferative changes associated with focal cortical dysplasia (J Neurooncol 2004;66:17)
  • Lesions with significant mitotic activity (5+ mitoses per 10 HPF) or with areas of necrosis may be designated "pleomorphic xanthoastrocytoma with anaplastic features"
Epidemiology
  • Typically children and young adults but older patients may be affected
  • No gender predilection
Sites
  • Superficial meningocerebral location
  • 98% arise supratentorially, with preferential temporal lobe involvement
  • May arise in cerebellum, spinal cord and rarely in retina
Clinical features
  • Associated with intractable seizures
  • 15 - 20% progress to malignancy
Radiology description
  • Usually cystic with a frequent cyst mural nodule architecture
Radiology images

Images hosted on other servers:

Contrast enhanced axial CT image

Superficial tumor

Case reports
Treatment
  • Gross total resection usually eliminates seizures
Clinical images

Images hosted on other servers:

Surgical exposure

Gross description
  • Mainly superficial with leptomeningeal involvement
  • Usually cystic
Microscopic (histologic) description
  • Key histopathological features are usually seen in superficial compact component of tumor, including pleomorphic cells (mono or multinucleated with frequent nuclear inclusion and occasional cytoplasmic xanthomatous change), spindle cells arranged in fascicular pattern
  • Other features include perivascular lymphocytic cuffing, scattered eosinophilic granular bodies, reticulin rich network
  • Underlying cortex shows infiltrative astrocytic component
  • Variable hemorrhage and protein granular degeneration (similar to pilocytic astrocytoma)
  • No necrosis and no mitotic activity, except in tumors "with anaplastic features"
Positive stains
Negative stains
Electron microscopy description
  • Tumor cells are surrounded by basal lamina; neuronal features of microtubules, dense core granules
Differential diagnosis
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