U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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]

Professional guidelines

PubMed

Bernstock JD, Hoffman SE, Kappel AD, Valdes PA, Essayed W, Klinger NV, Kang KD, Totsch SK, Olsen HE, Schlappi CW, Filipski K, Gessler FA, Baird L, Filbin MG, Hashizume R, Becher OJ, Friedman GK
Oncoimmunology 2022;11(1):2124058. Epub 2022 Sep 26 doi: 10.1080/2162402X.2022.2124058. PMID: 36185807Free 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
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
Rashed WM, Maher E, Adel M, Saber O, Zaghloul MS
Cancer Metastasis Rev 2019 Dec;38(4):759-770. doi: 10.1007/s10555-019-09824-2. PMID: 31802357
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

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
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

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
Rashed WM, Maher E, Adel M, Saber O, Zaghloul MS
Cancer Metastasis Rev 2019 Dec;38(4):759-770. doi: 10.1007/s10555-019-09824-2. PMID: 31802357
Aziz-Bose R, Monje M
Curr Opin Oncol 2019 Nov;31(6):522-530. doi: 10.1097/CCO.0000000000000577. PMID: 31464759Free 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

Clinical prediction guides

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
Krishnatry R, Manjali JJ, Chinnaswamy G, Chatterjee A, Goda JS, Janu A, Sahu A, Jalali R, Gupta T
Jpn J Clin Oncol 2021 Apr 30;51(5):762-768. doi: 10.1093/jjco/hyab006. PMID: 33521824
Rashed WM, Maher E, Adel M, Saber O, Zaghloul MS
Cancer Metastasis Rev 2019 Dec;38(4):759-770. doi: 10.1007/s10555-019-09824-2. PMID: 31802357
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
Chua J, Nafziger E, Leung D
Curr Oncol Rep 2019 Mar 5;21(4):30. doi: 10.1007/s11912-019-0783-5. PMID: 30835007

Recent systematic reviews

van den Bent M, Saratsis AM, Geurts M, Franceschi E
Neuro Oncol 2024 May 3;26(Supplement_2):S110-S124. doi: 10.1093/neuonc/noad220. PMID: 38102230Free PMC Article
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...