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Desmoplastic infantile astrocytoma(DIA)

MedGen UID:
141595
Concept ID:
C0457179
Neoplastic Process
Synonym: DIA
SNOMED CT: Desmoplastic infantile astrocytoma (128787000)
 
Monarch Initiative: MONDO:0022963

Definition

A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as 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 astrocytoma

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
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

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
Li Z, Yu Y, Lu Z, Gong J
World Neurosurg 2020 Dec;144:e189-e194. Epub 2020 Aug 18 doi: 10.1016/j.wneu.2020.08.091. PMID: 32822952
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
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

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
Li Z, Yu Y, Lu Z, Gong J
World Neurosurg 2020 Dec;144:e189-e194. Epub 2020 Aug 18 doi: 10.1016/j.wneu.2020.08.091. PMID: 32822952
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368
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

Therapy

Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article
van Tilburg CM, Selt F, Sahm F, Bächli H, Pfister SM, Witt O, Milde T
Pediatr Blood Cancer 2018 Mar;65(3) Epub 2017 Dec 12 doi: 10.1002/pbc.26893. PMID: 29230924
Greer A, Foreman NK, Donson A, Davies KD, Kleinschmidt-DeMasters BK
Pediatr Blood Cancer 2017 Jun;64(6) Epub 2016 Nov 10 doi: 10.1002/pbc.26350. PMID: 27860162Free PMC Article
Al-Kharazi K, Gillis C, Steinbok P, Dunham C
Clin Neuropathol 2013 Mar-Apr;32(2):100-6. doi: 10.5414/NP300548. PMID: 23149336

Prognosis

Chiang J, Li X, Jin H, Wu G, Lin T, Ellison DW
Neuropathol Appl Neurobiol 2022 Jun;48(4):e12801. Epub 2022 Mar 1 doi: 10.1111/nan.12801. PMID: 35191090Free PMC Article
Li Z, Yu Y, Lu Z, Gong J
World Neurosurg 2020 Dec;144:e189-e194. Epub 2020 Aug 18 doi: 10.1016/j.wneu.2020.08.091. PMID: 32822952
Samkari A, Alzahrani F, Almehdar A, Algahtani H
Clin Neuropathol 2017 Jan/Feb;36 (2017)(1):31-40. doi: 10.5414/NP300945. PMID: 27668845
Isaacs H Jr
Childs Nerv Syst 2016 Nov;32(11):2085-2096. Epub 2016 Aug 27 doi: 10.1007/s00381-016-3215-y. PMID: 27568373Free PMC Article
Soffietti R, Rudà R, Reardon D
Handb Clin Neurol 2016;134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4. PMID: 26948368

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
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
Gessi M, Zur Mühlen A, Hammes J, Waha A, Denkhaus D, Pietsch T
J Neuropathol Exp Neurol 2013 Sep;72(9):807-15. doi: 10.1097/NEN.0b013e3182a033a0. PMID: 23965740
Uro-Coste E, Ssi-Yan-Kai G, Guilbeau-Frugier C, Boetto S, Bertozzi AI, Sevely A, Lolmede K, Delisle MB
J Neurooncol 2010 May;98(1):143-9. Epub 2009 Dec 11 doi: 10.1007/s11060-009-0075-2. PMID: 20012157

Recent systematic reviews

Andrews LJ, Thornton ZA, Saincher SS, Yao IY, Dawson S, McGuinness LA, Jones HE, Jefferies S, Short SC, Cheng HY, McAleenan A, Higgins JPT, Kurian KM
Neuro Oncol 2022 Apr 1;24(4):528-540. doi: 10.1093/neuonc/noab247. PMID: 34718782Free PMC Article
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

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