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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

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
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
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Recent clinical studies

Etiology

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
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
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
Gelabert-Gonzalez M, Serramito-García R, Arcos-Algaba A
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Diagnosis

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
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
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
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
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

Therapy

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
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Dunnick NR
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Prognosis

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
Samkari A, Alzahrani F, Almehdar A, Algahtani H
Clin Neuropathol 2017 Jan/Feb;36 (2017)(1):31-40. doi: 10.5414/NP300945. PMID: 27668845
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
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
VandenBerg SR
Brain Pathol 1993 Jul;3(3):275-81. doi: 10.1111/j.1750-3639.1993.tb00754.x. PMID: 8293187

Clinical prediction guides

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
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
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
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
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

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
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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