Pleomorphic xanthoastrocytoma

Case contributed by Dr Bahman Rasuli

Presentation

Patient presented with a few months of complex seizures. Otherwise exam is well.

Patient Data

Age: 14 years
Gender: Male
MRI

There is an intra-axial multilobulated mixed solid-cystic mass involving cortical/subcortical region of right temporal lobe causing expansion of the lateral aspect of the temporal lobe, with effacement of sulci and enlargement of gyral folds. The solid component is hypo to isointense on T1 and slightly hyperintense on T2 images. The cystic component is hypointense on T1 and hyperintense on T2.

On post-contrast images, the solid component of the mass is avidly enhanced. No evidence of blood products within the lesion is noted.

Local mass effect without evidence of midline shift or uncal herniation is seen.

Subtle bony remodelling and thinning of the adjacent skull also is noted. 

Case Discussion

PXAs are solid-cystic variants of WHO class II astrocytomas that most commonly appear in the temporal lobe of adult patients. The solid component enhances avidly after contrast administration. The adjacent edema is minimal. 

Pathology proven pleomorphic xanthoastrocytoma (PXA). 

Microscopic examination show brain tissue with a neoplastic growth composed mainly of intertwining bundles of spindle cells having uniform oval nuclei with low mitotic activity accompanied by nests of entrapped foamy histiocytes, foci of myxoid change, multivacuolated xanthomas cells and rare bizarre giant cells; no evidence of necrosis or endothelial proliferation.

IHC study results:

  • GFAP: diffuse and strongly positive
  • S100: diffuse and strongly positive
  • EMA: negative
  • Synaptophysin: positive in entrapped neurons
  • Ki-67: positive in up 8% of tumor cells
  • P53: focal 5% positively
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Case information

rID: 61187
Published: 22nd Jun 2018
Last edited: 23rd Jun 2018
Inclusion in quiz mode: Included
Institution: Jame Jam Imaging Center

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