Glioblastoma IDH wild-type

Case contributed by Bruno Di Muzio
Diagnosis certain

Presentation

Seizure.

Patient Data

Age: 65 years
Gender: Male

MRI Brain

mri

Technique: Multiplanar, multisequence imaging including pre and post contrast, perfusion and spectroscopy. No previous imaging available for comparison. Stereotaxis also performed for surgical planning.

Findings: There is a peripherally enhancing mass in the left parietal lobe corona radiata extending to the ventricle. There is irregular peripheral enhancement with central necrosis and increased cerebral blood volume and evidence of hemorrhage into the lesion. There are patchy areas of low ADC in the lesion and spectroscopy shows relatively increased choline and lactate and reduced NAA in keeping with a glioma trace (Spectroscopy not shown above). Non enhancing tumor extends into the splenium of the corpus callosum and left cuneus as well as into the fornices that are thickened. There is mild mass effect on the left lateral ventricle without midline shift.

Superior frontal gyrus high T2 signal is suggestive of further disease, although gliosis is also a possibility (low CBV).

Conclusion: Findings are those of a high grade glioma (WHO IV) with extensive extension across the midline and with a possible second non-enhancing focus in the frontal lobe (gliomatosis).

pathology

MICROSCOPIC DESCRIPTION: Sections show fragments of a moderately hypercellular astrocytic glioma. Tumor cells show moderate nuclear and cellular pleomorphism. Scattered mitotic figures are identified. There is prominent microvascular proliferation with multilayering of atypical cells around vessel lumena. Areas of palisaded tumor necrosis are also identified.

Immunohistochemistry results show tumor cells stain:

  • GFAP Positive
  • Nestin Positive (high)
  • IDH-1 R132H Negative (not mutated)
  • ATRX Positive (not mutated)
  • MGMT Negative (likely methylated)
  • p53 Positive
  • p16 CDKN2A Negative

Topoisomerase labeling index: Approximately 30%.

DIAGNOSIS: IDH-1 wild type Glioblastoma (WHO Grade IV).

Case Discussion

An old adult presenting with the first episode of seizure were assessed by a CT scan (not available) that depicted the cerebral lesion better evaluated on MRI, with typical features of a high-grade glioma

Surgery resection confirmed the diagnosis of a glioblastoma, specifically a wild-type, based on its immunohistochemistry. 

  • IDH-1 negative (not mutated) = 'wild type' = poor prognosis
  • ATRX Positive (not mutated) 
  • MGMT Negative (likely methylated)

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