EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood
- Study type
- Clinical guideline
- Study name
- EANO Glioma 2021
- Design
- Evidence-based clinical guideline of the European Association of Neuro-Oncology (EANO); multidisciplinary task force
- Source
- Nat Rev Clin Oncol · 2021
In plain words
This guideline from the European Association of Neuro-Oncology explains how adult glioma tumours should be diagnosed and treated. The key point is that today the type of tumour is not decided by the microscope alone: molecular tests (such as the IDH mutation, 1p/19q deletion and MGMT methylation) identify the exact tumour type and the best treatment. For glioblastoma, the standard treatment is surgery followed by radiotherapy with temozolomide tablets; for IDH-mutant gliomas, radiotherapy followed by chemotherapy (PCV or temozolomide depending on the tumour type) is recommended.
Key guideline recommendations
- C: I; L: A
The standard of care for patients with IDH-wildtype glioblastoma aged under 70 years with KPS above 70 consists of maximal safe resection or biopsy, followed by involved-field radiotherapy with concomitant temozolomide and six cycles of maintenance temozolomide chemotherapy (EORTC 26981-NCIC CE.3).
- C: II; L: B
Temozolomide is probably active only in patients with MGMT promoter-methylated tumours, whereas its activity in tumours without MGMT promoter methylation is probably minimal.
- C: II; L: B
Elderly patients who are not candidates for temozolomide chemoradiotherapy should be treated according to MGMT promoter methylation status (NOA-08 and Nordic trials) with radiotherapy alone (such as 15 fractions of 2.66 Gy) or temozolomide alone (5 of 28 days).
- C: I; L: B
MGMT promoter methylation status should be determined in glioblastoma — especially in elderly or frail patients — to help decide on the use of temozolomide.
- C: II; L: B
The standard of care for IDH-mutant grade 2 astrocytoma requiring further treatment consists of maximal safe resection or biopsy, followed by involved-field radiotherapy and maintenance PCV chemotherapy (RTOG 9802).
- C: II; L: B
The standard of care for IDH-mutant grade 3 astrocytoma consists of maximal safe resection or biopsy, followed by involved-field radiotherapy and maintenance temozolomide (CATNON).
- C: II; L: B
Patients with IDH-mutant, 1p/19q-codeleted grade 3 oligodendroglioma should be treated with radiotherapy followed by PCV chemotherapy (EORTC 26951, RTOG 9402). For grade 2 requiring further treatment, radiotherapy followed by PCV is also recommended (C: III; L: B).
- C: IV; L: B
The extent of resection is a prognostic factor, so an attempt at complete resection is justified in all glioma types; however, preventing new permanent neurological deficits takes priority over the extent of resection (C: IV; L: C).
- C: IV; L: B
Glioma classification should follow the latest WHO classification of central nervous system tumours together with the cIMPACT-NOW updates; immunohistochemistry for mutant IDH1 R132H and ATRX should be done routinely.
- C: II; L: B
At recurrence, standards of care are less well defined. Surgery and radiotherapy can be considered. Nitrosourea regimens, temozolomide rechallenge and — depending on each country's approval — bevacizumab are pharmacotherapy options, but their effect on overall survival has not been proven. Where possible, enrolment in a suitable clinical trial should be considered.
Study population
Adults with diffuse glioma, including IDH-mutant astrocytoma, IDH-mutant and 1p/19q-codeleted oligodendroglioma, IDH-wildtype glioblastoma and H3 K27M-mutant diffuse midline glioma. The diagnostic section is based on the WHO 2016 classification and the cIMPACT-NOW updates.
Clinical significance and place in guidelines
This guideline is the main European reference for managing adult glioma, and its recommendations are based on the Stupp (EORTC/NCIC), RTOG 9802, CATNON, EORTC 26951/RTOG 9402 and NOA-08/Nordic trials. On TTFields, the guideline considers their use during temozolomide maintenance "still controversial despite a positive phase 3 trial (EF-14)" and of limited availability in Europe. Note: this guideline was published before the WHO 2021 classification and before the approval of vorasidenib (FDA, 2024).
Limitations
The diagnostic section is based on WHO 2016 and cIMPACT-NOW and was written before the official publication of the WHO 2021 classification. Many of the underlying trials enrolled patients before molecular classification. Newer data (such as INDIGO and vorasidenib) are not included.
This summary was prepared by the editorial team from the published paper; the original paper is the final reference. Read the original abstract on PubMed.