CNS tumor
Astrocytic tumors
Pleomorphic xanthoastrocytoma

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 28 August 2017, last major update March 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Pleomorphic xanthoastrocytoma CNS

Cite this page: Abdelzaher, E. Pleomorphic xanthoastrocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorpleomorphicxanthoastrocytoma.html. Accessed October 22nd, 2017.
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"
Microscopic (histologic) images

Images hosted on other servers:

Various images of pleomorphic cells and cells with lipid


GFAP+

Staining vs. glioblastoma giant cell variant

Various images / stains in pigmented tumor


1: Eosinophilic granular bodies (arrows); 2: Rosenthal fibers; 3: atypical cells with granular or foamy cytoplasm and atypical nuclei with pseudoinclusions (arrowhead) and mitotic figures (arrows); 4: multinuclear giant cells with tumor necrosis, nuclear palisading and focal vascular proliferation (arrows)

Positive stains
Negative stains
Electron microscopy description
  • Tumor cells are surrounded by basal lamina; neuronal features of microtubules, dense core granules
Differential diagnosis