What are the guideline sources, guidelines and references for Glioblastoma ?
Guideline sources The following summarized guidelines for the evaluation and management of glioblastoma are prepared by our editorial team based on guidelines from the American Association of Neurological Surgeons (AANS/CNS 2022; 2014), the European Association of Neuro-Oncology (EANO 2021; 2017), the European Association of Neuro-Oncology (EANO/SNO 2021), the Spanish Association of Medical Oncology (SEOM 2018), the American Society for Radiation Oncology (ASTRO 2016), and the European Society of Medical Oncology (ESMO 2014). 1 2 3 4 5 6 7 8 9 10 11 12 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Guidelines https://web.pathway.md/diseases/reckgYogVTBs6efiZ 1/8 6/24/23, 4:21 PM Glioblastoma Pathway 1. Screening and diagnosis Indications for screening: Recognize that screening and prevention have no major role for patients with gliomas. B Refer patients with relevant germline variants or suspected hereditary cancer syndromes for genetic counseling and, subsequently, for possible molecular genetic testing. B 2. Classification and risk stratification Classification: As per EANO 2021 guidelines, use the most recent WHO Classification of Tumors of the CNS for glioma classification, complemented by cIMPACT-NOW. B As per SEOM 2018 guidelines, classify glioblastomas into IDH-wild-type and IDH-mutated depending on the IDH gene mutation status. B 3. Diagnostic investigations Clinical assessment: assess the Karnofsky performance score and neurological function, as well as the patient's age, individual risks, and benefits for clinical decision-making. B Diagnostic imaging: As per EANO 2021 guidelines, obtain MRI without and with gadolinium contrast as the first choice diagnostic imaging modality. B As per SEOM 2018 guidelines: Obtain MRI without and with contrast enhancement as first-line imaging for diagnostic evaluation. B Consider obtaining advanced MRI sequences, including perfusion (cerebral blood volume/permeability), diffusion (DWI/apparent diffusion coefficient), and proton magnetic resonance spectroscopy, to provide information related to hemodynamic, cellular, and metabolism and help to identify glioma subtype and aggressiveness. C 4. Diagnostic procedures Tumor biopsy: As per EANO 2021 guidelines: Perform biopsy or resection as the standard of care in < 70 years old patients with IDH-wild- type glioblastoma and a Karnofsky performance status > 70. B Make clinical decisions without obtaining a tissue diagnosis only in very exceptional situations. B https://web.pathway.md/diseases/reckgYogVTBs6efiZ 2/8 6/24/23, 4:21 PM Glioblastoma Pathway As per ESMO 2014 guidelines, obtain histological confirmation of diagnosis and include sufficient tissue for molecular testing. E Molecular testing, IDH mutation, MGMT promoter methylation, and 1p/19q co-deletion: As per EANO 2021 guidelines, obtain routine immunohistochemistry for mutant IDH-1 R132H protein and nuclear expression of ATRX in the diagnostic evaluation of patients with diffuse gliomas. B Show 2 more As per SEOM 2018 guidelines, obtain MGMT promoter methylation status and IDH mutation testing. B As per ESMO 2014 guidelines, obtain MGMT promoter methylation status, IDH gene mutation, and 1p/19q co-deletion testing depending on the histological and clinical context. E Molecular testing (further testing): obtain combined chromosome 7 gain and chromosome 10 loss (+7/-10 signature), epidermal growth factor receptor amplification, and TERT promoter mutation testing in patients with IDH-wild-type diffuse gliomas lacking microvascular proliferation and necrosis as histological features of WHO grade 4 to allow for a diagnosis of IDH-wild-type glioblastoma. B Show 2 more Molecular testing (repeat testing): do not obtain repeat IDH mutation testing to derive new additional management or prognostic information beyond that derived from the tumor at the initial presentation in adult patients with progressive glioblastoma if the tumor is histologically similar to the primary tumor and the patient's clinical course is as expected. D Show 5 more 5. Medical management Management of initial disease, surgical resection: As per EANO 2021 guidelines: Perform surgical resection as feasible or biopsy followed as the standard of care in < 70 years old patients with IDH-wild-type glioblastoma and a Karnofsky performance score > 70. A Attempt to obtain complete resection B while prioritizing prevention of new permanent neurological deficits over the extent of resection. B As per SEOM 2018 guidelines: Perform surgical resection (maximum safe resection while preserving neurological function) as the first therapeutic approach to reduce the mass effect, obtain tissue for diagnosis, and improve survival in patients with glioblastoma. B Use neuronavigation systems, intraoperative imaging with MRI or ultrasound, and fluorescence dye 5-ALA to improve the extent of resection and improve progression-free survival. A As per ESMO 2014 guidelines, perform surgery to the extent feasible as the first-line therapeutic intervention in all patients with malignant glioma. E https://web.pathway.md/diseases/reckgYogVTBs6efiZ 3/8 6/24/23, 4:21 PM Glioblastoma Pathway Management of initial disease, radiotherapy: As per EANO 2021 guidelines, offer involved-field radiotherapy after resection or biopsy in < 70 years old patients with IDH-wild-type glioblastoma and a Karnofsky performance score > 70. A As per ASTRO 2016 guidelines, consider offering fractionated radiotherapy in individual patients depending on patient characteristics, such as performance status. (Strength of recommendation: Strong, HQE) Recognize that older age and poor performance status are associated with shorter survival in patients with glioblastoma. C Show 5 more Management of initial disease, concomitant chemoradiotherapy: As per EANO 2021 guidelines, offer concomitant radiotherapy and 6 cycles of maintenance temozolomide chemotherapy after resection or biopsy in < 70 years old patients with IDH-wild- type glioblastoma and a Karnofsky performance score > 70. A Show 2 more As per SEOM 2018 guidelines, offer combined treatment with radiotherapy (60 Gy in 30 fractions) and temozolomide (75 mg/m /day during radiotherapy) followed by adjuvant temozolomide (150-200 mg/m /day for 5 consecutive days every 28 days) for 6 cycles in < 70 years old adult patients with newly diagnosed glioblastoma. A Show 2 more As per ASTRO 2016 guidelines, offer fractionated radiotherapy with concurrent and adjuvant temozolomide as the standard of care following biopsy or resection in < 70 years old patients with newly diagnosed glioblastoma. A Show 2 more As per ESMO 2014 guidelines: Offer combined treatment with temozolomide and radiotherapy as the standard of care in patients with glioblastoma. E Consider offering temozolomide monotherapy in elderly patients with MGMT promoter- methylated tumors and hypofractionated radiotherapy alone in elderly patients with MGMT promoter-unmethylated tumors. E Management of initial disease (target volumes for radiotherapy): perform partial brain radiation therapy as the standard treatment paradigm for glioblastoma. A Show 2 more Management of initial disease (tumor-treating fields): consider offering tumor-treating fields in addition to adjuvant temozolomide after combined radiotherapy and temozolomide in patients with newly diagnosed supratentorial glioblastoma. B Management of progressive disease (repeat cytoreductive surgery): perform repeat cytoreductive surgery in symptomatic patients with progressive malignant glioma, recognizing that the expected median survival is 6-17 months following a second procedure. B Management of progressive disease, carmustine-impregnated wafers: As per SEOM 2018 guidelines, consider performing implantation of carmustine-impregnated wafers in patients with progressive glioblastoma. C https://web.pathway.md/diseases/reckgYogVTBs6efiZ 4/8 6/24/23, 4:21 PM Glioblastoma Pathway As per CNS/AANS 2014 guidelines, perform implantation of carmustine-impregnated biodegradable polymer wafers as a surgical adjunct when cytoreductive surgery is indicated in patients with progressive glioblastoma, taking into account the associated toxicities observed with this treatment. B Management of progressive disease (repeat radiotherapy): Offer re-irradiation (conventional fractionation radiotherapy, fractionated radiosurgery, or single fraction radiosurgery) for improved local tumor control in adult patients with progressive glioblastoma after first-line combined multimodality treatment with chemotherapy and radiotherapy if the target tumor is amenable to additional radiation. B Offer re-irradiation to maintain or improve the neurological status and quality of life before further tumor progression. B Management of progressive disease (chemotherapy): Offer temozolomide over procarbazine in patients with a first relapse of glioblastoma after having received nitrosourea chemotherapy or no prior cytotoxic chemotherapy at the time of initial therapy. B Consider offering other cytotoxic chemotherapy agents of uncertain benefit in patients with progressive glioblastoma based on the judgment of the treating physician, taking into account the individual patient's prior treatment exposure, systemic health, and the likelihood of tolerance of the toxicities of any given agent. Encourage enrollment in available clinical trials for these cases. C Management of progressive disease (targeted therapy): offer bevacizumab to improve disease control (imaging response and progression-free survival at 6 months) in adult patients with progressive glioblastoma. B Management of symptoms (headache): Administer corticosteroids for the treatment of headaches in patients with glioma. B Consider administering analgesics and co-analgesics for the treatment of headaches in patients with gliomas (in accordance with the WHO cancer pain ladder). C Management of symptoms, seizure: As per EANO/SNO 2021 guidelines, do not administer prophylactic antiepileptic drugs to reduce the risk of seizures in patients with a newly diagnosed brain tumor and no history of seizures. D Show 2 more As per SEOM 2018 guidelines, do not administer prophylactic antiepileptic drugs outside the perioperative period. D As per EANO 2017 guidelines: Offer intranasal midazolam or buccal clonazepam for the management of seizures in the end- of-life phase where the oral route is not an option. B Consider using alternative routes for antiepileptic drug administration in patients with a history of epilepsy developing swallowing difficulties, with the preferred route depending on the local availability of antiepileptic drugs and the place of care. E https://web.pathway.md/diseases/reckgYogVTBs6efiZ 5/8 6/24/23, 4:21 PM Glioblastoma Pathway Management of symptoms (fatigue): insufficient evidence to recommend any pharmacological or nonpharmacological intervention for fatigue in patients with glioma. I Management of symptoms (psychological and cognitive disorders): insufficient evidence to recommend pharmacological interventions, including methylphenidate and donepezil, for mood disorders in patients with glioma. I Consider offering multimodal psychosocial interventions to improve depressive symptoms. I Show 3 more Management of symptoms (delirium): identify and address underlying causes of delirium by adequate symptom control or changes in doses or type of medication. B Show 2 more 6. Perioperative care Postoperative thromboprophylaxis: initiate VTE prophylaxis with LMWH postoperatively within 24 hours, as the perioperative risk of intracranial hypertension increases when prophylaxis is initiated before induction of anesthesia. B Show 2 more Postoperative imaging: Obtain postoperative MRI within 24-48 hours after tumor excision (II, B) to avoid radiological changes related to subacute hemorrhage, ischemia, and inflammation appearing beyond 72 hours, in order to assess the extent of resection and acquire a baseline image for follow-up. B Obtain MRI within 72 hours after complete or partial resection. B 7. Patient education Genetic counseling: refer patients with relevant germline variants or suspected hereditary cancer syndromes for genetic counseling and, subsequently, for possible molecular genetic testing. B 8. Follow-up and surveillance Rehabilitation: Consider offering early postoperative rehabilitation and rehabilitation after tumor-specific treatment. C Consider offering cognitive rehabilitation in patients with glioma, especially in young patients with relatively favorable prognoses. (III) Consider offering cognitive rehabilitation in patients with stable glioma and cognitive complaints and/or deficits. E Surveillance after curative-intent therapy, MRI/MRS: As per EANO 2021 guidelines, suspect pseudoprogression in patients with an increase of abnormalities on neuroimaging in the first months after local therapeutic interventions (including radiotherapy) and after experimental local treatments. B https://web.pathway.md/diseases/reckgYogVTBs6efiZ 6/8 6/24/23, 4:21 PM Glioblastoma Pathway As per SEOM 2018 guidelines, consider viewing an apparent increase of tumor volume on MRI in the first months after local therapeutic interventions (including radiotherapy and experimental local treatments) as pseudoprogression. C As per CNS/AANS 2014 guidelines: Obtain MRI with and without gadolinium enhancement for imaging surveillance to detect the progression of previously diagnosed glioblastoma. B Obtain magnetic resonance spectroscopy as a diagnostic modality to differentiate true tumor progression from treatment-related imaging changes or pseudoprogression in patients with suspected progressive glioblastoma. B Surveillance after curative-intent therapy (PET/SPECT): Obtain single-photon emission CT imaging as a diagnostic modality to differentiate true tumor progression from treatment-related imaging changes or pseudoprogression in patients with suspected progressive glioblastoma. B Do not obtain routine positron emission tomography to identify the progression of glioblastoma. D Management of recurrent disease: As per EANO 2021 guidelines, consider offering surgery and radiotherapy in patients with recurrent IDH-wild-type glioblastoma. C Show 2 more As per SEOM 2018 guidelines, consider offering procarbazine, lomustine, and vincristine or single-agent nitrosourea therapy to achieve tumor control in patients with recurrent glioblastoma. C Show 4 more As per ASTRO 2016 guidelines, consider offering focal re-irradiation (stereotactic radiosurgery, hypofractionated stereotactic radiation therapy, or brachytherapy) in younger patients with recurrent glioblastoma and good performance status, taking into account tumor size and location. C As per AANS 2014 guidelines: Offer repeat cytoreductive surgery in symptomatic patients with locally recurrent malignant glioma, recognizing that the expected median survival is 6-17 months following a second procedure. B Take into account the location of recurrence in eloquent/critical brain regions, Karnofsky performance status, and tumor volume when evaluating patients for a repeat operation. B References 1. Alvin R Cabrera, John P Kirkpatrick, John B Fiveash et al. Radiation therapy for glioblastoma: Executive summary of an American Society for Radiation Oncology Evidence-Based Clinical Practice Guideline. Pract Radiat Oncol. 2016 Jul-Aug;6 4 217 225. Open https://web.pathway.md/diseases/reckgYogVTBs6efiZ 7/8 6/24/23, 4:21 PM Glioblastoma Pathway 2. Andrea Pace, Linda Dirven, Johan A F Koekkoek et al. European Association for Neuro-Oncology EANO guidelines for palliative care in adults with glioma. Lancet Oncol. 2017 Jun;18 6):e330-e340. Open 3. R Stupp, M Brada, M J van den Bent et al. High-grade glioma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep;25 Suppl 3:iii93 101. Open 4. Michael Weller, Martin van den Bent, Matthias Preusser et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021 Mar;18 3 170 186. Open 5. Jeffrey J Olson, Lakshmi Nayak, D Ryan Ormond et al. The role of cytotoxic chemotherapy in the management of progressive glioblastoma : a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2014 Jul;118 3 501 55. Open 6. M Mart nez-Garcia, J lvarez-Linera, C Carrato et al. SEOM clinical guidelines for diagnosis and treatment of glioblastoma 2017 . Clin Transl Oncol. 2018 Jan;20 1 22 28. Open 7. Timothy Charles Ryken, Steven N Kalkanis, John M Buatti et al. The role of cytoreductive surgery in the management of progressive glioblastoma : a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2014 Jul;118 3 479 88. Open 8. Timothy Charles Ryken, Nafi Aygun, Johnathan Morris et al. The role of imaging in the management of progressive glioblastoma : a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2014 Jul;118 3 435 60. Open 9. Tobias Walbert, Rebecca A Harrison, David Schiff et al. SNO and EANO practice guideline update: Anticonvulsant prophylaxis in patients with newly diagnosed brain tumors. Neuro Oncol. 2021 Nov 2;23 11 1835 1844. Open 10. Abigail L Goodman, Jos E Vel zquez Vega, Chad Glenn et al. Congress of neurological surgeons systematic review and evidence-based guidelines update on the role of neuropathology in the management of progressive glioblastoma in adults. J Neurooncol. 2022 Jun;158 2 179 224. Open 11. Jeffrey J Olson, Lakshmi Nayak, D Ryan Ormond et al. The role of targeted therapies in the management of progressive glioblastoma : a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2014 Jul;118 3 557 99. Open 12. Samuel Ryu, John M Buatti, Ann Morris et al. The role of radiotherapy in the management of progressive glioblastoma : a systematic review and evidence-based clinical practice guideline. J Neurooncol. 2014 Jul;118 3 489 99. Open https://web.pathway.md/diseases/reckgYogVTBs6efiZ 8/8