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Diffuse intrinsic pontine glioma

MedGen UID:
458884
Concept ID:
C2986658
Neoplastic Process
Synonyms: Brain Tumor, DIPG; Diffuse Intrinsic Pontine Glioma; DIPG Brain Tumor; DIPG Brain Tumors; DIPG, Diffuse Intrinsic Pontine Glioma
SNOMED CT: Diffuse intrinsic pontine glioma (11471000224106); DIPG - diffuse intrinsic pontine glioma (11471000224106); Diffuse intrinsic pontine glioma (1156451005)
 
Monarch Initiative: MONDO:0006033
Orphanet: ORPHA497188

Definition

A rare glial tumor characterized by a highly aggressive, diffusely infiltrative pontine lesion generally occurring in children, affecting local nerve fiber tracts and spreading contiguously to involve adjacent structures, but also metastasizing within the central nervous system. Patients mostly present with a short history of symptoms, typically including the classic triad of multiple cranial neuropathies, long tract signs, and ataxia. Signs and symptoms of increased intracranial pressure may present due to obstructive hydrocephalus. Prognosis is poor and not related to histological grade. [from ORDO]

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Professional guidelines

PubMed

Weisbrod LJ, Thiraviyam A, Vengoji R, Shonka N, Jain M, Ho W, Batra SK, Salehi A
Cancer Lett 2024 May 28;590:216876. Epub 2024 Apr 10 doi: 10.1016/j.canlet.2024.216876. PMID: 38609002Free PMC Article
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418

Recent clinical studies

Etiology

Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article
Venkatesh HS, Tam LT, Woo PJ, Lennon J, Nagaraja S, Gillespie SM, Ni J, Duveau DY, Morris PJ, Zhao JJ, Thomas CJ, Monje M
Nature 2017 Sep 28;549(7673):533-537. Epub 2017 Sep 20 doi: 10.1038/nature24014. PMID: 28959975Free PMC Article
Gholamin S, Mitra SS, Feroze AH, Liu J, Kahn SA, Zhang M, Esparza R, Richard C, Ramaswamy V, Remke M, Volkmer AK, Willingham S, Ponnuswami A, McCarty A, Lovelace P, Storm TA, Schubert S, Hutter G, Narayanan C, Chu P, Raabe EH, Harsh G 4th, Taylor MD, Monje M, Cho YJ, Majeti R, Volkmer JP, Fisher PG, Grant G, Steinberg GK, Vogel H, Edwards M, Weissman IL, Cheshier SH
Sci Transl Med 2017 Mar 15;9(381) doi: 10.1126/scitranslmed.aaf2968. PMID: 28298418
Wu G, Diaz AK, Paugh BS, Rankin SL, Ju B, Li Y, Zhu X, Qu C, Chen X, Zhang J, Easton J, Edmonson M, Ma X, Lu C, Nagahawatte P, Hedlund E, Rusch M, Pounds S, Lin T, Onar-Thomas A, Huether R, Kriwacki R, Parker M, Gupta P, Becksfort J, Wei L, Mulder HL, Boggs K, Vadodaria B, Yergeau D, Russell JC, Ochoa K, Fulton RS, Fulton LL, Jones C, Boop FA, Broniscer A, Wetmore C, Gajjar A, Ding L, Mardis ER, Wilson RK, Taylor MR, Downing JR, Ellison DW, Zhang J, Baker SJ
Nat Genet 2014 May;46(5):444-450. Epub 2014 Apr 6 doi: 10.1038/ng.2938. PMID: 24705251Free PMC Article
Grimm SA, Chamberlain MC
Curr Neurol Neurosci Rep 2013 May;13(5):346. doi: 10.1007/s11910-013-0346-3. PMID: 23512689

Diagnosis

Duchatel RJ, Jackson ER, Parackal SG, Kiltschewskij D, Findlay IJ, Mannan A, Staudt DE, Thomas BC, Germon ZP, Laternser S, Kearney PS, Jamaluddin MFB, Douglas AM, Beitaki T, McEwen HP, Persson ML, Hocke EA, Jain V, Aksu M, Manning EE, Murray HC, Verrills NM, Sun CX, Daniel P, Vilain RE, Skerrett-Byrne DA, Nixon B, Hua S, de Bock CE, Colino-Sanguino Y, Valdes-Mora F, Tsoli M, Ziegler DS, Cairns MJ, Raabe EH, Vitanza NA, Hulleman E, Phoenix TN, Koschmann C, Alvaro F, Dayas CV, Tinkle CL, Wheeler H, Whittle JR, Eisenstat DD, Firestein R, Mueller S, Valvi S, Hansford JR, Ashley DM, Gregory SG, Kilburn LB, Nazarian J, Cain JE, Dun MD
J Clin Invest 2024 Feb 6;134(6) doi: 10.1172/JCI170329. PMID: 38319732Free PMC Article
Noll A, Myers C, Biery MC, Meechan M, Tahiri S, Rajendran A, Berens ME, Paine D, Byron S, Zhang J, Winter C, Pakiam F, Leary SES, Cole BL, Jackson ER, Dun MD, Foster JB, Evans MK, Pattwell SS, Olson JM, Vitanza NA
Neoplasia 2023 Sep;43:100921. Epub 2023 Aug 19 doi: 10.1016/j.neo.2023.100921. PMID: 37603953Free PMC Article
Gállego Pérez-Larraya J, Garcia-Moure M, Labiano S, Patiño-García A, Dobbs J, Gonzalez-Huarriz M, Zalacain M, Marrodan L, Martinez-Velez N, Puigdelloses M, Laspidea V, Astigarraga I, Lopez-Ibor B, Cruz O, Oscoz Lizarbe M, Hervas-Stubbs S, Alkorta-Aranburu G, Tamayo I, Tavira B, Hernandez-Alcoceba R, Jones C, Dharmadhikari G, Ruiz-Moreno C, Stunnenberg H, Hulleman E, van der Lugt J, Idoate MÁ, Diez-Valle R, Esparragosa Vázquez I, Villalba M, de Andrea C, Núñez-Córdoba JM, Ewald B, Robbins J, Fueyo J, Gomez-Manzano C, Lang FF, Tejada S, Alonso MM
N Engl J Med 2022 Jun 30;386(26):2471-2481. doi: 10.1056/NEJMoa2202028. PMID: 35767439
Wu G, Diaz AK, Paugh BS, Rankin SL, Ju B, Li Y, Zhu X, Qu C, Chen X, Zhang J, Easton J, Edmonson M, Ma X, Lu C, Nagahawatte P, Hedlund E, Rusch M, Pounds S, Lin T, Onar-Thomas A, Huether R, Kriwacki R, Parker M, Gupta P, Becksfort J, Wei L, Mulder HL, Boggs K, Vadodaria B, Yergeau D, Russell JC, Ochoa K, Fulton RS, Fulton LL, Jones C, Boop FA, Broniscer A, Wetmore C, Gajjar A, Ding L, Mardis ER, Wilson RK, Taylor MR, Downing JR, Ellison DW, Zhang J, Baker SJ
Nat Genet 2014 May;46(5):444-450. Epub 2014 Apr 6 doi: 10.1038/ng.2938. PMID: 24705251Free PMC Article
Grimm SA, Chamberlain MC
Curr Neurol Neurosci Rep 2013 May;13(5):346. doi: 10.1007/s11910-013-0346-3. PMID: 23512689

Therapy

Vitanza NA, Wilson AL, Huang W, Seidel K, Brown C, Gustafson JA, Yokoyama JK, Johnson AJ, Baxter BA, Koning RW, Reid AN, Meechan M, Biery MC, Myers C, Rawlings-Rhea SD, Albert CM, Browd SR, Hauptman JS, Lee A, Ojemann JG, Berens ME, Dun MD, Foster JB, Crotty EE, Leary SES, Cole BL, Perez FA, Wright JN, Orentas RJ, Chour T, Newell EW, Whiteaker JR, Zhao L, Paulovich AG, Pinto N, Gust J, Gardner RA, Jensen MC, Park JR
Cancer Discov 2023 Jan 9;13(1):114-131. doi: 10.1158/2159-8290.CD-22-0750. PMID: 36259971Free PMC Article
Shan S, Chen J, Sun Y, Wang Y, Xia B, Tan H, Pan C, Gu G, Zhong J, Qing G, Zhang Y, Wang J, Wang Y, Wang Y, Zuo P, Xu C, Li F, Guo W, Xu L, Chen M, Fan Y, Zhang L, Liang XJ
Adv Sci (Weinh) 2022 Jul;9(21):e2200353. Epub 2022 May 18 doi: 10.1002/advs.202200353. PMID: 35585670Free PMC Article
Majzner RG, Ramakrishna S, Yeom KW, Patel S, Chinnasamy H, Schultz LM, Richards RM, Jiang L, Barsan V, Mancusi R, Geraghty AC, Good Z, Mochizuki AY, Gillespie SM, Toland AMS, Mahdi J, Reschke A, Nie EH, Chau IJ, Rotiroti MC, Mount CW, Baggott C, Mavroukakis S, Egeler E, Moon J, Erickson C, Green S, Kunicki M, Fujimoto M, Ehlinger Z, Reynolds W, Kurra S, Warren KE, Prabhu S, Vogel H, Rasmussen L, Cornell TT, Partap S, Fisher PG, Campen CJ, Filbin MG, Grant G, Sahaf B, Davis KL, Feldman SA, Mackall CL, Monje M
Nature 2022 Mar;603(7903):934-941. Epub 2022 Feb 7 doi: 10.1038/s41586-022-04489-4. PMID: 35130560Free PMC Article
Perrone MG, Ruggiero A, Centonze A, Carrieri A, Ferorelli S, Scilimati A
Curr Med Chem 2021;28(17):3287-3317. doi: 10.2174/0929867327666200806110206. PMID: 32767913
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article

Prognosis

Weisbrod LJ, Thiraviyam A, Vengoji R, Shonka N, Jain M, Ho W, Batra SK, Salehi A
Cancer Lett 2024 May 28;590:216876. Epub 2024 Apr 10 doi: 10.1016/j.canlet.2024.216876. PMID: 38609002Free PMC Article
Arrillaga-Romany I, Lassman A, McGovern SL, Mueller S, Nabors B, van den Bent M, Vogelbaum MA, Allen JE, Melemed AS, Tarapore RS, Wen PY, Cloughesy T
Neuro Oncol 2024 May 3;26(Supplement_2):S173-S181. doi: 10.1093/neuonc/noae031. PMID: 38445964Free PMC Article
Gállego Pérez-Larraya J, Garcia-Moure M, Labiano S, Patiño-García A, Dobbs J, Gonzalez-Huarriz M, Zalacain M, Marrodan L, Martinez-Velez N, Puigdelloses M, Laspidea V, Astigarraga I, Lopez-Ibor B, Cruz O, Oscoz Lizarbe M, Hervas-Stubbs S, Alkorta-Aranburu G, Tamayo I, Tavira B, Hernandez-Alcoceba R, Jones C, Dharmadhikari G, Ruiz-Moreno C, Stunnenberg H, Hulleman E, van der Lugt J, Idoate MÁ, Diez-Valle R, Esparragosa Vázquez I, Villalba M, de Andrea C, Núñez-Córdoba JM, Ewald B, Robbins J, Fueyo J, Gomez-Manzano C, Lang FF, Tejada S, Alonso MM
N Engl J Med 2022 Jun 30;386(26):2471-2481. doi: 10.1056/NEJMoa2202028. PMID: 35767439
Perrone MG, Ruggiero A, Centonze A, Carrieri A, Ferorelli S, Scilimati A
Curr Med Chem 2021;28(17):3287-3317. doi: 10.2174/0929867327666200806110206. PMID: 32767913
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article

Clinical prediction guides

Tzaridis T, Wechsler-Reya RJ
J Clin Invest 2024 Mar 15;134(6) doi: 10.1172/JCI179144. PMID: 38488006Free PMC Article
Noll A, Myers C, Biery MC, Meechan M, Tahiri S, Rajendran A, Berens ME, Paine D, Byron S, Zhang J, Winter C, Pakiam F, Leary SES, Cole BL, Jackson ER, Dun MD, Foster JB, Evans MK, Pattwell SS, Olson JM, Vitanza NA
Neoplasia 2023 Sep;43:100921. Epub 2023 Aug 19 doi: 10.1016/j.neo.2023.100921. PMID: 37603953Free PMC Article
Guo X, Chang M, Wang Y, Xing B, Ma W
Int J Biol Sci 2023;19(12):3762-3780. Epub 2023 Jul 24 doi: 10.7150/ijbs.85813. PMID: 37564196Free PMC Article
Majzner RG, Ramakrishna S, Yeom KW, Patel S, Chinnasamy H, Schultz LM, Richards RM, Jiang L, Barsan V, Mancusi R, Geraghty AC, Good Z, Mochizuki AY, Gillespie SM, Toland AMS, Mahdi J, Reschke A, Nie EH, Chau IJ, Rotiroti MC, Mount CW, Baggott C, Mavroukakis S, Egeler E, Moon J, Erickson C, Green S, Kunicki M, Fujimoto M, Ehlinger Z, Reynolds W, Kurra S, Warren KE, Prabhu S, Vogel H, Rasmussen L, Cornell TT, Partap S, Fisher PG, Campen CJ, Filbin MG, Grant G, Sahaf B, Davis KL, Feldman SA, Mackall CL, Monje M
Nature 2022 Mar;603(7903):934-941. Epub 2022 Feb 7 doi: 10.1038/s41586-022-04489-4. PMID: 35130560Free PMC Article
Anastas JN, Zee BM, Kalin JH, Kim M, Guo R, Alexandrescu S, Blanco MA, Giera S, Gillespie SM, Das J, Wu M, Nocco S, Bonal DM, Nguyen QD, Suva ML, Bernstein BE, Alani R, Golub TR, Cole PA, Filbin MG, Shi Y
Cancer Cell 2019 Nov 11;36(5):528-544.e10. Epub 2019 Oct 17 doi: 10.1016/j.ccell.2019.09.005. PMID: 31631026

Recent systematic reviews

Dalmage M, LoPresti MA, Sarkar P, Ranganathan S, Abdelmageed S, Pagadala M, Shlobin NA, Lam S, DeCuypere M
J Neurosurg Pediatr 2023 Dec 1;32(6):665-672. Epub 2023 Sep 8 doi: 10.3171/2023.7.PEDS22462. PMID: 37724839
Parenrengi MA, Suryaningtyas W, Al Fauzi A, Hafid Bajamal A, Kusumastuti K, Utomo B, Muslim Hidayat Thamrin A, Sulistiono B
Cancer Control 2022 Jan-Dec;29:10732748211053927. doi: 10.1177/10732748211053927. PMID: 35191733Free PMC Article
Park J, Yea JW, Park JW
Medicine (Baltimore) 2020 Oct 16;99(42):e22721. doi: 10.1097/MD.0000000000022721. PMID: 33080729Free PMC Article
Lu VM, Welby JP, Mahajan A, Laack NN, Daniels DJ
Childs Nerv Syst 2019 May;35(5):739-746. Epub 2019 Mar 16 doi: 10.1007/s00381-019-04118-y. PMID: 30879125
Freese C, Takiar V, Fouladi M, DeWire M, Breneman J, Pater L
Pract Radiat Oncol 2017 Mar-Apr;7(2):86-92. Epub 2016 Nov 23 doi: 10.1016/j.prro.2016.11.005. PMID: 28274399

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