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The role of neuropathology in the management of patients with diffuse low grade glioma

A systematic review and evidence-based clinical practice guideline

  • Topic Review & Clinical Guidelines
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Target population

Adult patients (age ≥18 years) who have suspected low-grade diffuse glioma.

Question

What are the optimal neuropathological techniques to diagnose low-grade diffuse glioma in the adult?

Recommendation

Level I

Histopathological analysis of a representative surgical sample of the lesion should be used to provide the diagnosis of low-grade diffuse glioma.

Level III

Both frozen section and cytopathologic/smear evaluation should be used to aid the intra-operative assessment of low-grade diffuse glioma diagnosis. A resection specimen is preferred over a biopsy specimen, to minimize the potential for sampling error issues.

Target population

Patients with histologically-proven WHO grade II diffuse glioma.

Question

In adult patients (age ≥18 years) with histologically-proven WHO grade II diffuse glioma, is testing for IDH1 mutation (R132H and/or others) warranted? If so, is there a preferred method?

Recommendation

Level II

IDH gene mutation assessment, via IDH1 R132H antibody and/or IDH1/2 mutation hotspot sequencing, is highly-specific for low-grade diffuse glioma, and is recommended as an additional test for classification and prognosis.

Target population

Patients with histologically-proven WHO grade II diffuse glioma.

Question

In adult patients (age ≥18 years) with histologically-proven WHO grade II diffuse glioma, is testing for 1p/19q loss warranted? If so, is there a preferred method?

Recommendation

Level III

1p/19q loss-of-heterozygosity testing, by FISH, array-CGH or PCR, is recommended as an additional test in oligodendroglial cases for prognosis and potential treatment planning.

Target population

Patients with histologically-proven WHO grade II diffuse glioma.

Question

In adult patients (age ≥18 years) with histologically-proven WHO grade II diffuse glioma, is MGMT promoter methylation testing warranted? If so, is there a preferred method?

Recommendation

There is insufficient evidence to recommend methyl-guanine methyl-transferase (MGMT) promoter methylation testing as a routine for low-grade diffuse gliomas. It is recommended that patients be enrolled in properly designed clinical trials to assess the value of this and related markers for this target population.

Target population

Patients with histologically-proven WHO grade II diffuse glioma.

Question

In adult patients (age ≥18 years) with histologically-proven WHO grade II diffuse glioma, is Ki-67/MIB1 immunohistochemistry warranted? If so, is there a preferred method to quantitate results?

Recommendation

Level III

Ki67/MIB1 immunohistochemistry is recommended as an option for prognostic assessment.

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Acknowledgments

We acknowledge the significant contributions of Laura Mitchell, Senior Manager of Clinical Practice Guidelines for the Congress of Neurological Surgeons, the AANS/CNS Joint Guidelines Committee (JGC) for their review, comments and suggestions, and Anne Woznica, and Mary Bodach for their assistance with the literature searches.

Disclosures

Dr. Kalkanis is a consultant for Arbor and Varian. Dr. Olson is a consultant for the American Cancer Society; has received research funding from the National Cancer Institute, Genentech, and Millenium; and has received investigational drug provision from Merck.

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Cahill, D.P., Sloan, A.E., Nahed, B.V. et al. The role of neuropathology in the management of patients with diffuse low grade glioma. J Neurooncol 125, 531–549 (2015). https://doi.org/10.1007/s11060-015-1909-8

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