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Desmoplastic infantile ganglioglioma(DIG)

MedGen UID:
231168
•Concept ID:
C1321878
•
Neoplastic Process
Synonym: DIG
SNOMED CT: Desmoplastic infantile ganglioglioma (128787000)
 
Monarch Initiative: MONDO:0022965

Definition

A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population of neoplastic astrocytes together with a variable neuronal component. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as a large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDesmoplastic infantile ganglioglioma

Professional guidelines

PubMed

A comprehensive analysis of infantile central nervous system tumors to improve distinctive criteria for infant-type hemispheric glioma versus desmoplastic infantile ganglioglioma/astrocytoma.
Tauziède-Espariat A, Beccaria K, Dangouloff-Ros V, Sievers P, Meurgey A, Pissaloux D, Appay R, Saffroy R, Grill J, Mariet C, Bourdeaut F, Hasty L, Métais A, Chrétien F, Blauwblomme T, Puget S, Boddaert N, Varlet P; RENOCLIP-LOC
Brain Pathol 2023 Sep;33(5):e13182. Epub 2023 Jun 22 doi: 10.1111/bpa.13182. PMID: 37349135Free PMC Article
Surgical treatment of brain tumors in infants younger than six months of age and review of the literature.
Lang SS, Beslow LA, Gabel B, Judkins AR, Fisher MJ, Sutton LN, Storm PB, Heuer GG
World Neurosurg 2012 Jul;78(1-2):137-44. Epub 2011 Nov 7 doi: 10.1016/j.wneu.2011.09.012. PMID: 22120270Free PMC Article
The pleomorphic xanthoastrocytoma and its differential diagnosis: a study of five cases.
Kros JM, Vecht CJ, Stefanko SZ
Hum Pathol 1991 Nov;22(11):1128-35. doi: 10.1016/0046-8177(91)90265-q. PMID: 1743696

Recent clinical studies

Etiology

A comprehensive analysis of infantile central nervous system tumors to improve distinctive criteria for infant-type hemispheric glioma versus desmoplastic infantile ganglioglioma/astrocytoma.
Tauziède-Espariat A, Beccaria K, Dangouloff-Ros V, Sievers P, Meurgey A, Pissaloux D, Appay R, Saffroy R, Grill J, Mariet C, Bourdeaut F, Hasty L, Métais A, Chrétien F, Blauwblomme T, Puget S, Boddaert N, Varlet P; RENOCLIP-LOC
Brain Pathol 2023 Sep;33(5):e13182. Epub 2023 Jun 22 doi: 10.1111/bpa.13182. PMID: 37349135Free PMC Article
Desmoplastic infantile astrocytoma and ganglioglioma: a series of 12 patients treated at a single institution.
Imperato A, Spennato P, Mazio F, Arcas E, Ozgural O, Quaglietta L, Errico ME, Cinalli G
Childs Nerv Syst 2021 Jul;37(7):2187-2195. Epub 2021 Jan 28 doi: 10.1007/s00381-021-05057-3. PMID: 33507362
Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution's experience.
Naylor RM, Wohl A, Raghunathan A, Eckel LJ, Keating GF, Daniels DJ
J Neurosurg Pediatr 2018 Oct;22(4):397-403. Epub 2018 Jul 6 doi: 10.3171/2018.4.PEDS17638. PMID: 29979130Free PMC Article
Radiological features of infantile glioblastoma and desmoplastic infantile tumors: British Columbia's Children's Hospital experience.
Bader A, Heran M, Dunham C, Steinbok P
J Neurosurg Pediatr 2015 Aug;16(2):119-25. Epub 2015 May 8 doi: 10.3171/2014.10.PEDS13634. PMID: 25955808
Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature.
Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A
Neurosurg Rev 2010 Apr;34(2):151-8. Epub 2011 Jan 19 doi: 10.1007/s10143-010-0303-4. PMID: 21246390

Diagnosis

Recurrent TRAK1::RAF1 Fusions in pediatric low-grade gliomas.
Benhamida JK, Harmsen HJ, Ma D, William CM, Li BK, Villafania L, Sukhadia P, Mullaney KA, Dewan MC, Vakiani E, Karajannis MA, Snuderl M, Zagzag D, Ladanyi M, Rosenblum MK, Bale TA
Brain Pathol 2023 Sep;33(5):e13185. Epub 2023 Jul 3 doi: 10.1111/bpa.13185. PMID: 37399073Free PMC Article
Desmoplastic infantile ganglioglioma.
Kamoun S, Azouz H, Zemmali M, Haouet S, Kchir N
Pan Afr Med J 2019;32:113. Epub 2019 Mar 11 doi: 10.11604/pamj.2019.32.113.12669. PMID: 31223403Free PMC Article
Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature.
Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A
Neurosurg Rev 2010 Apr;34(2):151-8. Epub 2011 Jan 19 doi: 10.1007/s10143-010-0303-4. PMID: 21246390
Desmoplastic infantile ganglioglioma.
Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C
Childs Nerv Syst 2003 Jun;19(5-6):292-7. Epub 2003 May 16 doi: 10.1007/s00381-003-0743-z. PMID: 12750935
Desmoplastic infantile ganglioglioma.
Rothman S, Sharon N, Shiffer J, Toren A, Pollak L, Mandel M, Kenet G, Neumann Y, Nass D
Acta Oncol 1997;36(6):655-7. doi: 10.3109/02841869709001332. PMID: 9408160

Therapy

NTRK-fused central nervous system tumours: clinicopathological and genetic insights and response to TRK inhibitors.
Kim EE, Park CK, Kim SK, Phi JH, Paek SH, Choi JY, Kang HJ, Lee JH, Won JK, Yun H, Park SH
Acta Neuropathol Commun 2024 Jul 16;12(1):118. doi: 10.1186/s40478-024-01798-9. PMID: 39014476Free PMC Article
Desmoplastic infantile ganglioglioma: a potentially malignant tumor?
De Munnynck K, Van Gool S, Van Calenbergh F, Demaerel P, Uyttebroeck A, Buyse G, Sciot R
Am J Surg Pathol 2002 Nov;26(11):1515-22. doi: 10.1097/00000478-200211000-00015. PMID: 12409729
Molecular evidence of apoptotic death in malignant brain tumors including glioblastoma multiforme: upregulation of calpain and caspase-3.
Ray SK, Patel SJ, Welsh CT, Wilford GG, Hogan EL, Banik NL
J Neurosci Res 2002 Jul 15;69(2):197-206. doi: 10.1002/jnr.10265. PMID: 12111801
Image interpretation session: 1999. Desmoplastic infantile ganglioglioma.
Dunnick NR
Radiographics 2000 Jan-Feb;20(1):276-8. PMID: 10682792
Second malignancies in young children with primary brain tumors following treatment with prolonged postoperative chemotherapy and delayed irradiation: a Pediatric Oncology Group study.
Duffner PK, Krischer JP, Horowitz ME, Cohen ME, Burger PC, Friedman HS, Kun LE
Ann Neurol 1998 Sep;44(3):313-6. doi: 10.1002/ana.410440305. PMID: 9749596

Prognosis

Predictors of mortality and tumor recurrence in desmoplastic infantile ganglioglioma and astrocytoma-and individual participant data meta-analysis (IPDMA).
Wang S, Sun MZ, Abecassis IJ, Weil AG, Ibrahim GM, Fallah A, Ene C, Leary SES, Cole BL, Lockwood CM, Olson JM, Geyer JR, Ellenbogen RG, Ojemann JG, Wang AC
J Neurooncol 2021 Nov;155(2):155-163. Epub 2021 Oct 6 doi: 10.1007/s11060-021-03860-1. PMID: 34613581Free PMC Article
Desmoplastic infantile astrocytoma and ganglioglioma: a series of 12 patients treated at a single institution.
Imperato A, Spennato P, Mazio F, Arcas E, Ozgural O, Quaglietta L, Errico ME, Cinalli G
Childs Nerv Syst 2021 Jul;37(7):2187-2195. Epub 2021 Jan 28 doi: 10.1007/s00381-021-05057-3. PMID: 33507362
Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature.
Samkari A, Alzahrani F, Almehdar A, Algahtani H
Clin Neuropathol 2017 Jan/Feb;36 (2017)(1):31-40. doi: 10.5414/NP300945. PMID: 27668845
Desmoplastic infantile ganglioglioma.
Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C
Childs Nerv Syst 2003 Jun;19(5-6):292-7. Epub 2003 May 16 doi: 10.1007/s00381-003-0743-z. PMID: 12750935
Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy.
VandenBerg SR
Brain Pathol 1993 Jul;3(3):275-81. doi: 10.1111/j.1750-3639.1993.tb00754.x. PMID: 8293187

Clinical prediction guides

Predictors of mortality and tumor recurrence in desmoplastic infantile ganglioglioma and astrocytoma-and individual participant data meta-analysis (IPDMA).
Wang S, Sun MZ, Abecassis IJ, Weil AG, Ibrahim GM, Fallah A, Ene C, Leary SES, Cole BL, Lockwood CM, Olson JM, Geyer JR, Ellenbogen RG, Ojemann JG, Wang AC
J Neurooncol 2021 Nov;155(2):155-163. Epub 2021 Oct 6 doi: 10.1007/s11060-021-03860-1. PMID: 34613581Free PMC Article
Desmoplastic Infantile Ganglioglioma: A MAPK Pathway-Driven and Microglia/Macrophage-Rich Neuroepithelial Tumor.
Blessing MM, Blackburn PR, Krishnan C, Harrod VL, Barr Fritcher EG, Zysk CD, Jackson RA, Milosevic D, Nair AA, Davila JI, Balcom JR, Jenkins RB, Halling KC, Kipp BR, Nageswara Rao AA, Laack NN, Daniels DJ, Macon WR, Ida CM
J Neuropathol Exp Neurol 2019 Nov 1;78(11):1011-1021. doi: 10.1093/jnen/nlz086. PMID: 31562743
Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIG/DIA) Are Distinct Entities with Frequent BRAFV600 Mutations.
Wang AC, Jones DTW, Abecassis IJ, Cole BL, Leary SES, Lockwood CM, Chavez L, Capper D, Korshunov A, Fallah A, Wang S, Ene C, Olson JM, Geyer JR, Holland EC, Lee A, Ellenbogen RG, Ojemann JG
Mol Cancer Res 2018 Oct;16(10):1491-1498. Epub 2018 Jul 13 doi: 10.1158/1541-7786.MCR-17-0507. PMID: 30006355Free PMC Article
Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review.
Bianchi F, Tamburrini G, Massimi L, Caldarelli M
Childs Nerv Syst 2016 Oct;32(10):1833-8. Epub 2016 Sep 20 doi: 10.1007/s00381-016-3149-4. PMID: 27659826
Desmoplastic infantile ganglioglioma.
Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C
Childs Nerv Syst 2003 Jun;19(5-6):292-7. Epub 2003 May 16 doi: 10.1007/s00381-003-0743-z. PMID: 12750935

Recent systematic reviews

Predictors of mortality and tumor recurrence in desmoplastic infantile ganglioglioma and astrocytoma-and individual participant data meta-analysis (IPDMA).
Wang S, Sun MZ, Abecassis IJ, Weil AG, Ibrahim GM, Fallah A, Ene C, Leary SES, Cole BL, Lockwood CM, Olson JM, Geyer JR, Ellenbogen RG, Ojemann JG, Wang AC
J Neurooncol 2021 Nov;155(2):155-163. Epub 2021 Oct 6 doi: 10.1007/s11060-021-03860-1. PMID: 34613581Free PMC Article
Resolving mystery behind autonomous retrogression of low-grade gliomas: A systematic review.
Ahmed SI, Bareeqa SB, Samar SS, Jilanee SDA
J Pak Med Assoc 2020 Dec;70(12(B)):2441-2448. doi: 10.47391/JPMA.581. PMID: 33475560

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