Glioblastoma with PNET

Case contributed by A.Prof Frank Gaillard

Presentation

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Patient Data

Age: 40
Gender: Female
Modality: MRI

There is a 4.8 x 5.9 x 5.3 mm right frontal lesion, with mixed cystic and solid components. Intense irregular rim-like enhancement pattern is seen, as well as enhancement of the solid component. Areas of moderate restriction diffusion are seen within the lesion, corresponding to the solid components (not the fluid / non-enhancing component).

Multiple flow voids are seen within the lesion.

There is a significant amount of surrounding flair signal abnormality, with 13 mm leftward midline shift at the level of the septum pellucidum.

Conclusion:

Findings are most likely in keeping with a high grade astrocytoma (WHO grade IV).

Case Discussion

MICROSCOPIC DESCRIPTION:

Paraffin sections show a densely hypercellular tumour. This is composed almost entirely of cells with features of primitive neuroectoderm with overlapping angulated hyperchromatic nuclei and a paucity of processes. These are arranged in diffuse sheets. In some very small areas, fibrillary astrocytic and oligodendroglial morphologies are discernible. Frequent mitotic figures are identified and there is prominent microvascular proliferation with florid endothelial cell hyperplasia. Many areas of palisaded and confluent necrosis are also identified. Several of these incorporate thin-walled necrotic and thrombosed blood vessels. The features are of glioblastoma multiforme with a large primitive neuroectodermal tumour (PNET) component (WHO Grade IV).

IMMUNOHISTOCHEMISTRY:

  • GFAP positive
  • Synaptophysin patchy positive
  • Nestin positive
  • IDH-1 R132H negative (not mutated)
  • MGMT negative (likely methylated)
  • p53 negative
  • p63 negative
  • Topoisomerase labelling index: Approximately 35%

DIAGNOSIS:

Glioblastoma multiforme with a large primitive neuroectodermal tumour (PNET) component (WHO Grade IV).

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Case Information

rID: 33235
Case created: 5th Jan 2015
Last edited: 29th Mar 2017
Inclusion in quiz mode: Included

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