Intracranial chondrosarcoma (myxoid type)

Case contributed by Royal Melbourne Hospital

Patient Data

Age: 60 years
Gender: Female

CT brain

Large rounded mainly hypotenuse mass lesion in the right frontoparietal region, with surrounding vasogenic deem and moderate mass effect, in the form of effacement of the ipsilateral sulci, lateral ventricle (partially) causing a degree of midline shift. 

  • Mixed solid-cystic lesion with necrosis/liquefaction seen within the solid component. Enhancing wall around the cystic component. Intraaxial location. Bright DWI signal within tumour reflects low ADC due to hypercellularity. No calcification seen (CT). The appearance is indistinguishable from high grade glioma/glioblastoma.

    During surgery, the margin is well-circumscribed and the tumour is rubbery/leathery.

Paraffin sections show a densely hypercellular tumour composed of small cells with round and oval hyperchromatic nuclei and a small amount of pale cytoplasm. These are arranged in diffuse sheets within a stroma containing myxoid/mucinous material. Poorly formed cartilage is noted in one area. Tumour cells also form perivascular pseudo-rosette like structures around viable blood vessels. Frequent mitotic figures are identified and there is extensive tumour necrosis.

Immunohistochemistry shows strong membrane staining in tumour cells for CD56, patchy staining for CD99 (MIC-2) in a membranous and peri-nuclear pattern, and patchy staining for desmin, smooth muscle actin (SMA) and neurone specific enolase (NSE). No staining for GFAP, synaptophysin, TTF-1, tyrosinase or a range of cytokeratins (AE1/AE3, CAM5.2; CK7, CK20, CK19, CK5&6) is seen in tumour cells.

The features are of an undifferentiated malignant tumour. The myxoid stromal material and focal poorly formed cartilage suggest myxoid chondrorsarcoma.  Immunostaining for CD56, NSE and CD99(MIC2) is consistent with this diagnosis.

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Case information

rID: 15336
Published: 9th Oct 2011
Last edited: 12th Jan 2016
Inclusion in quiz mode: Included

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