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Refractory Low Grade Glioma

MedGen UID:
1791069
•Concept ID:
C5554628
•
Neoplastic Process

Definition

Low grade glioma that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Low Grade Glioma

Professional guidelines

PubMed

Trametinib therapy for children with neurofibromatosis type 1 and life-threatening plexiform neurofibroma or treatment-refractory low-grade glioma.
Ronsley R, Hounjet CD, Cheng S, Rassekh SR, Duncan WJ, Dunham C, Gardiner J, Ghag A, Ludemann JP, Wensley D, Rehmus W, Sargent MA, Hukin J
Cancer Med 2021 Jun;10(11):3556-3564. Epub 2021 May 3 doi: 10.1002/cam4.3910. PMID: 33939292Free PMC Article

Recent clinical studies

Etiology

Treatment of Pediatric Low-Grade Gliomas.
Sait SF, Giantini-Larsen AM, Tringale KR, Souweidane MM, Karajannis MA
Curr Neurol Neurosci Rep 2023 Apr;23(4):185-199. Epub 2023 Mar 7 doi: 10.1007/s11910-023-01257-3. PMID: 36881254Free PMC Article
Dabrafenib plus trametinib in patients with BRAF(V600E)-mutant low-grade and high-grade glioma (ROAR): a multicentre, open-label, single-arm, phase 2, basket trial.
Wen PY, Stein A, van den Bent M, De Greve J, Wick A, de Vos FYFL, von Bubnoff N, van Linde ME, Lai A, Prager GW, Campone M, Fasolo A, Lopez-Martin JA, Kim TM, Mason WP, Hofheinz RD, Blay JY, Cho DC, Gazzah A, Pouessel D, Yachnin J, Boran A, Burgess P, Ilankumaran P, Gasal E, Subbiah V
Lancet Oncol 2022 Jan;23(1):53-64. Epub 2021 Nov 24 doi: 10.1016/S1470-2045(21)00578-7. PMID: 34838156
A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway: TRAM-01.
Perreault S, Larouche V, Tabori U, Hawkin C, Lippé S, Ellezam B, Décarie JC, Théoret Y, Métras MÉ, Sultan S, Cantin É, Routhier MÈ, Caru M, Legault G, Bouffet É, Lafay-Cousin L, Hukin J, Erker C, Jabado N
BMC Cancer 2019 Dec 27;19(1):1250. doi: 10.1186/s12885-019-6442-2. PMID: 31881853Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Seizures in low- and high-grade gliomas: current management and future outlook.
Vecht CJ, Wilms EB
Expert Rev Anticancer Ther 2010 May;10(5):663-9. doi: 10.1586/era.10.48. PMID: 20469999

Diagnosis

Sustained Survival Benefit in Recurrent Medulloblastoma by a Metronomic Antiangiogenic Regimen: A Nonrandomized Controlled Trial.
Peyrl A, Chocholous M, Sabel M, Lassaletta A, Sterba J, Leblond P, Nysom K, Torsvik I, Chi SN, Perwein T, Jones N, Holm S, Nyman P, Mörse H, Öberg A, Weiler-Wichtl L, Leiss U, Haberler C, Schmook MT, Mayr L, Dieckmann K, Kool M, Gojo J, Azizi AA, André N, Kieran M, Slavc I
JAMA Oncol 2023 Dec 1;9(12):1688-1695. doi: 10.1001/jamaoncol.2023.4437. PMID: 37883081Free PMC Article
Identifying predictors of glioma evolution from longitudinal sequencing.
Mu Q, Chai R, Pang B, Yang Y, Liu H, Zhao Z, Bao Z, Song D, Zhu Z, Yan M, Jiang B, Mo Z, Tang J, Sa JK, Cho HJ, Chang Y, Chan KHY, Loi DSC, Tam SST, Chan AKY, Wu AR, Liu Z, Poon WS, Ng HK, Chan DTM, Iavarone A, Nam DH, Jiang T, Wang J
Sci Transl Med 2023 Oct 4;15(716):eadh4181. doi: 10.1126/scitranslmed.adh4181. PMID: 37792958
Treatment of Pediatric Low-Grade Gliomas.
Sait SF, Giantini-Larsen AM, Tringale KR, Souweidane MM, Karajannis MA
Curr Neurol Neurosci Rep 2023 Apr;23(4):185-199. Epub 2023 Mar 7 doi: 10.1007/s11910-023-01257-3. PMID: 36881254Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
EANO guidelines for the diagnosis and treatment of meningiomas.
Goldbrunner R, Minniti G, Preusser M, Jenkinson MD, Sallabanda K, Houdart E, von Deimling A, Stavrinou P, Lefranc F, Lund-Johansen M, Moyal EC, Brandsma D, Henriksson R, Soffietti R, Weller M
Lancet Oncol 2016 Sep;17(9):e383-91. Epub 2016 Aug 30 doi: 10.1016/S1470-2045(16)30321-7. PMID: 27599143

Therapy

Efficacy and Safety of Trametinib Monotherapy or in Combination With Dabrafenib in Pediatric BRAF V600-Mutant Low-Grade Glioma.
Bouffet E, Geoerger B, Moertel C, Whitlock JA, Aerts I, Hargrave D, Osterloh L, Tan E, Choi J, Russo M, Fox E
J Clin Oncol 2023 Jan 20;41(3):664-674. Epub 2022 Nov 14 doi: 10.1200/JCO.22.01000. PMID: 36375115Free PMC Article
Dabrafenib plus trametinib in patients with BRAF(V600E)-mutant low-grade and high-grade glioma (ROAR): a multicentre, open-label, single-arm, phase 2, basket trial.
Wen PY, Stein A, van den Bent M, De Greve J, Wick A, de Vos FYFL, von Bubnoff N, van Linde ME, Lai A, Prager GW, Campone M, Fasolo A, Lopez-Martin JA, Kim TM, Mason WP, Hofheinz RD, Blay JY, Cho DC, Gazzah A, Pouessel D, Yachnin J, Boran A, Burgess P, Ilankumaran P, Gasal E, Subbiah V
Lancet Oncol 2022 Jan;23(1):53-64. Epub 2021 Nov 24 doi: 10.1016/S1470-2045(21)00578-7. PMID: 34838156
A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway: TRAM-01.
Perreault S, Larouche V, Tabori U, Hawkin C, Lippé S, Ellezam B, Décarie JC, Théoret Y, Métras MÉ, Sultan S, Cantin É, Routhier MÈ, Caru M, Legault G, Bouffet É, Lafay-Cousin L, Hukin J, Erker C, Jabado N
BMC Cancer 2019 Dec 27;19(1):1250. doi: 10.1186/s12885-019-6442-2. PMID: 31881853Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Mutational Landscape of Secondary Glioblastoma Guides MET-Targeted Trial in Brain Tumor.
Hu H, Mu Q, Bao Z, Chen Y, Liu Y, Chen J, Wang K, Wang Z, Nam Y, Jiang B, Sa JK, Cho HJ, Her NG, Zhang C, Zhao Z, Zhang Y, Zeng F, Wu F, Kang X, Liu Y, Qian Z, Wang Z, Huang R, Wang Q, Zhang W, Qiu X, Li W, Nam DH, Fan X, Wang J, Jiang T
Cell 2018 Nov 29;175(6):1665-1678.e18. Epub 2018 Oct 18 doi: 10.1016/j.cell.2018.09.038. PMID: 30343896

Prognosis

Sustained Survival Benefit in Recurrent Medulloblastoma by a Metronomic Antiangiogenic Regimen: A Nonrandomized Controlled Trial.
Peyrl A, Chocholous M, Sabel M, Lassaletta A, Sterba J, Leblond P, Nysom K, Torsvik I, Chi SN, Perwein T, Jones N, Holm S, Nyman P, Mörse H, Öberg A, Weiler-Wichtl L, Leiss U, Haberler C, Schmook MT, Mayr L, Dieckmann K, Kool M, Gojo J, Azizi AA, André N, Kieran M, Slavc I
JAMA Oncol 2023 Dec 1;9(12):1688-1695. doi: 10.1001/jamaoncol.2023.4437. PMID: 37883081Free PMC Article
Treatment of Pediatric Low-Grade Gliomas.
Sait SF, Giantini-Larsen AM, Tringale KR, Souweidane MM, Karajannis MA
Curr Neurol Neurosci Rep 2023 Apr;23(4):185-199. Epub 2023 Mar 7 doi: 10.1007/s11910-023-01257-3. PMID: 36881254Free PMC Article
Seizures in glioma patients: An overview of incidence, etiology, and therapies.
Samudra N, Zacharias T, Plitt A, Lega B, Pan E
J Neurol Sci 2019 Sep 15;404:80-85. Epub 2019 Jul 19 doi: 10.1016/j.jns.2019.07.026. PMID: 31352293
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Mutational Landscape of Secondary Glioblastoma Guides MET-Targeted Trial in Brain Tumor.
Hu H, Mu Q, Bao Z, Chen Y, Liu Y, Chen J, Wang K, Wang Z, Nam Y, Jiang B, Sa JK, Cho HJ, Her NG, Zhang C, Zhao Z, Zhang Y, Zeng F, Wu F, Kang X, Liu Y, Qian Z, Wang Z, Huang R, Wang Q, Zhang W, Qiu X, Li W, Nam DH, Fan X, Wang J, Jiang T
Cell 2018 Nov 29;175(6):1665-1678.e18. Epub 2018 Oct 18 doi: 10.1016/j.cell.2018.09.038. PMID: 30343896

Clinical prediction guides

Identifying predictors of glioma evolution from longitudinal sequencing.
Mu Q, Chai R, Pang B, Yang Y, Liu H, Zhao Z, Bao Z, Song D, Zhu Z, Yan M, Jiang B, Mo Z, Tang J, Sa JK, Cho HJ, Chang Y, Chan KHY, Loi DSC, Tam SST, Chan AKY, Wu AR, Liu Z, Poon WS, Ng HK, Chan DTM, Iavarone A, Nam DH, Jiang T, Wang J
Sci Transl Med 2023 Oct 4;15(716):eadh4181. doi: 10.1126/scitranslmed.adh4181. PMID: 37792958
A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway: TRAM-01.
Perreault S, Larouche V, Tabori U, Hawkin C, Lippé S, Ellezam B, Décarie JC, Théoret Y, Métras MÉ, Sultan S, Cantin É, Routhier MÈ, Caru M, Legault G, Bouffet É, Lafay-Cousin L, Hukin J, Erker C, Jabado N
BMC Cancer 2019 Dec 27;19(1):1250. doi: 10.1186/s12885-019-6442-2. PMID: 31881853Free PMC Article
Seizures in glioma patients: An overview of incidence, etiology, and therapies.
Samudra N, Zacharias T, Plitt A, Lega B, Pan E
J Neurol Sci 2019 Sep 15;404:80-85. Epub 2019 Jul 19 doi: 10.1016/j.jns.2019.07.026. PMID: 31352293
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
EANO guidelines for the diagnosis and treatment of meningiomas.
Goldbrunner R, Minniti G, Preusser M, Jenkinson MD, Sallabanda K, Houdart E, von Deimling A, Stavrinou P, Lefranc F, Lund-Johansen M, Moyal EC, Brandsma D, Henriksson R, Soffietti R, Weller M
Lancet Oncol 2016 Sep;17(9):e383-91. Epub 2016 Aug 30 doi: 10.1016/S1470-2045(16)30321-7. PMID: 27599143

Recent systematic reviews

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples.
Baumgartner ME, Lang SS, Tucker AM, Madsen PJ, Storm PB, Kennedy BC
Childs Nerv Syst 2024 May;40(5):1361-1366. Epub 2023 Dec 27 doi: 10.1007/s00381-023-06256-w. PMID: 38150037
Factors associated with seizure freedom in the surgical resection of glioneuronal tumors.
Englot DJ, Berger MS, Barbaro NM, Chang EF
Epilepsia 2012 Jan;53(1):51-7. Epub 2011 Sep 20 doi: 10.1111/j.1528-1167.2011.03269.x. PMID: 21933181

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