Diffuse astrocytoma IDH mutant

Case contributed by A.Prof Frank Gaillard

Presentation

Seizure

Patient Data

Age: 40 years
MRI

A high T2  signal mass in the posterior part of the left superior frontal gyrus, abutting and involving the precentral gyrus has low T1 signal and partial FLAIR suppression and peripheral restricted diffusion. No enhancement or increased perfusion. Spectroscopy demonstrates elevated choline and reduced NAA. No abnormal meningeal enhancement identified. The dural venous sinuses are patent.

Conclusion: The overall appearances of the medial posterior left frontal lobe mass would most favor a low-grade astrocytoma (previously classified as a protoplasmic astrocytoma).

Case Discussion

The patient went on to have surgery.

Histology

MICROSCOPIC DESCRIPTION:

Sections show a moderately cellular tumor composed of moderately pleomorphic cells containing round to oval, hyperchromatic, vesicular nuclei with inconspicuous nucleoli. Tumor cells are arranged in diffuse sheets with a "protoplasmic" morphology. No mitotic figures are identified. No necrosis or microvascular proliferation are seen.

IMMUNOHISTOCHEMISTRY:

  • GFAP Positive
  • Nogo A Positive
  • Nestin Positive (intermediate)
  • IDH-1 R132H Positive (mutated)
  • ATRX Equivocal
  • MGMT Negative (likely methylated)
  • p53 Equivocal
  • p16 Negative
  • Topoisomerase labeling index: Approximately 1-2%. 

FISH For Chromosome 1p/19q Deletion: No 1p/19q loss detected.

FINAL DIAGNOSIS: Diffuse astrocytoma, IDH-1 mutant (WHO Grade II).

 

This case is typical of the entity previously known as protoplasmic astrocytoma, which is no longer recognized as a distinct entity in the current (2016) WHO classification of CNS tumors. This is a shame as it is a recognizable tumor and is probably highly predictive of intact 1p19q status. 

 

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Case information

rID: 53901
Published: 6th Jul 2017
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included

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