Glioblastoma IDH wild-type (with primitive neuronal component)

Case contributed by A.Prof Frank Gaillard

Presentation

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Patient Data

Age: 40 years
Gender: Female

There is a 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

The patient went on to have surgery. 

Histology

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 with a large primitive neuronal component (WHO Grade IV).

 

Note: Although this tumour is entirely consistent with IDH wild-type molecular subtype, strictly speaking, to conclusively establish this, IDH would need to be sequenced to ensure that a non-IDH1 R132H mutation was present. In practice, an IDH1 R132H negative tumour in an individual over 55-years-of-age makes the possibility of this being IDH mutant remote (<1%), and sequencing is not felt to be necessary by many institutions, and not recommended by the WHO classification of CNS tumours (2016). In a younger patient (like this one) sequencing would be recommended. Thus the diagnostic certainty has been dropped to "almost certain".

 

Note: The current (2016) WHO classification of CNS tumours has made substantial changes to tumours previously considered to be PNET, now classified as embryonal tumours with multilayered rosettes (ETMR). In contrast, when a glioblastoma demonstrates similar histological features it is now referred to as a glioblastoma with primitive neuronal component

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Case information

rID: 33235
Case created: 5th Jan 2015
Last edited: 26th May 2017
Inclusion in quiz mode: Included

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