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Dysembryoplastic neuroepithelial tumor

MedGen UID:
266109
Concept ID:
C1266177
Neoplastic Process
Synonyms: DNET; DNT; Dysembryoplastic Neuroepithelial Neoplasm; dysembryoplastic neuroepithelial neoplasm; Dysembryoplastic Neuroepithelial Tumor; dysembryoplastic neuroepithelial tumor; dysembryoplastic neuroepithelial tumor (morphologic abnormality); dysembryoplastic neuroepithelial tumour (morphologic abnormality)
SNOMED CT: Dysembryoplastic neuroepithelial tumor (128788005); Dysembryoplastic neuroepithelial tumor (87211000119104)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0033703
Monarch Initiative: MONDO:0005505
Orphanet: ORPHA251946

Definition

Dysembryoplastic neuroepithelial tumor (DNT) is a benign glioneuronal neoplasm that most commonly occurs in children and young adults and may present with medically intractable, chronic seizures. Lesions vary in size from 10 to 25 mm, although occasionally larger tumors of up to 70 mm have been reported. Grossly, tumors appear as well-defined, solitary nodular masses or poorly demarcated lesions. On the cut section, most tumors are cortically located and may extend into the underlying subcortical white matter in larger tumors. Multi-nodular appearance or cystic changes are commonly found [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDysembryoplastic neuroepithelial tumor
Follow this link to review classifications for Dysembryoplastic neuroepithelial tumor in Orphanet.

Professional guidelines

PubMed

Wang Y, Wang L, Blümcke I, Zhang W, Fu Y, Shan Y, Piao Y, Zhao G
Brain Pathol 2022 Jan;32(1):e13011. Epub 2021 Aug 5 doi: 10.1111/bpa.13011. PMID: 34355449Free PMC Article
Cinalli G, Aguirre DT, Mirone G, Ruggiero C, Cascone D, Quaglietta L, Aliberti F, Santi SD, Buonocore MC, Nastro A, Spennato P
J Neurosurg Pediatr 2018 Mar;21(3):247-257. Epub 2017 Dec 22 doi: 10.3171/2017.7.PEDS16463. PMID: 29271729

Recent clinical studies

Etiology

Weaver KN, Gripp KW
Am J Med Genet C Semin Med Genet 2022 Dec;190(4):494-500. Epub 2022 Dec 1 doi: 10.1002/ajmg.c.32023. PMID: 36454176
Gupta P, Siraj F, Malik A, Shankar KB
J Cancer Res Ther 2021 Jul-Sep;17(4):912-916. doi: 10.4103/jcrt.JCRT_632_19. PMID: 34528541
Chiang JCH, Harreld JH, Tanaka R, Li X, Wen J, Zhang C, Boué DR, Rauch TM, Boyd JT, Chen J, Corbo JC, Bouldin TW, Elton SW, Liu LL, Schofield D, Lee SC, Bouffard JP, Georgescu MM, Dossani RH, Aguiar MA, Sances RA, Saad AG, Boop FA, Qaddoumi I, Ellison DW
Neuro Oncol 2019 Jun 10;21(6):800-808. doi: 10.1093/neuonc/noz037. PMID: 30726976Free PMC Article
Sánchez Fernández I, Loddenkemper T
Seizure 2017 Jan;44:98-107. Epub 2016 Dec 7 doi: 10.1016/j.seizure.2016.11.028. PMID: 28017579
Zhang JG, Hu WZ, Zhao RJ, Kong LF
J Child Neurol 2014 Nov;29(11):1441-7. Epub 2013 Jun 10 doi: 10.1177/0883073813490831. PMID: 23752069

Diagnosis

Vaz A, Cavalcanti MS, da Silva Junior EB, Ramina R, de Almeida Teixeira BC
AJNR Am J Neuroradiol 2022 Aug;43(8):1080-1089. Epub 2022 May 5 doi: 10.3174/ajnr.A7465. PMID: 35512827Free PMC Article
Gupta P, Siraj F, Malik A, Shankar KB
J Cancer Res Ther 2021 Jul-Sep;17(4):912-916. doi: 10.4103/jcrt.JCRT_632_19. PMID: 34528541
Slegers RJ, Blumcke I
Acta Neuropathol Commun 2020 Mar 9;8(1):27. doi: 10.1186/s40478-020-00904-x. PMID: 32151273Free 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
Prayson RA, Estes ML
Am J Clin Pathol 1992 Mar;97(3):398-401. doi: 10.1093/ajcp/97.3.398. PMID: 1543164

Therapy

Sajko T, Rotim K, Jurilj M, Rotim A, Splavski B, Jurčić Čulina I
Acta Clin Croat 2021 Dec;60(4):627-631. doi: 10.20471/acc.2021.60.04.09. PMID: 35734497Free PMC Article
Park JT, Baca Vaca GF, Avery J, Miller JP
Neurodiagn J 2017;57(3):191-210. doi: 10.1080/21646821.2017.1326270. PMID: 28898173
Bird-Lieberman G, Sethi K, Childs AM, Chumas P, Crimmins D, Ismail A, Livingston J
J Neurosurg Pediatr 2011 Apr;7(4):416-20. doi: 10.3171/2011.1.PEDS10258. PMID: 21456916
Rushing EJ, Thompson LD, Mena H
Ann Diagn Pathol 2003 Aug;7(4):240-4. doi: 10.1016/s1092-9134(03)00070-4. PMID: 12913847
Weissman Z, Michowitz S, Shuper A, Kornreich L, Amir J
Pediatr Hematol Oncol 1996 Sep-Oct;13(5):463-8. doi: 10.3109/08880019609030859. PMID: 10897819

Prognosis

Sajko T, Rotim K, Jurilj M, Rotim A, Splavski B, Jurčić Čulina I
Acta Clin Croat 2021 Dec;60(4):627-631. doi: 10.20471/acc.2021.60.04.09. PMID: 35734497Free PMC Article
Gupta P, Siraj F, Malik A, Shankar KB
J Cancer Res Ther 2021 Jul-Sep;17(4):912-916. doi: 10.4103/jcrt.JCRT_632_19. PMID: 34528541
Chiang JCH, Harreld JH, Tanaka R, Li X, Wen J, Zhang C, Boué DR, Rauch TM, Boyd JT, Chen J, Corbo JC, Bouldin TW, Elton SW, Liu LL, Schofield D, Lee SC, Bouffard JP, Georgescu MM, Dossani RH, Aguiar MA, Sances RA, Saad AG, Boop FA, Qaddoumi I, Ellison DW
Neuro Oncol 2019 Jun 10;21(6):800-808. doi: 10.1093/neuonc/noz037. PMID: 30726976Free 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
Zhang JG, Hu WZ, Zhao RJ, Kong LF
J Child Neurol 2014 Nov;29(11):1441-7. Epub 2013 Jun 10 doi: 10.1177/0883073813490831. PMID: 23752069

Clinical prediction guides

Wang J, Luo X, Chen C, Deng J, Long H, Yang K, Qi S
Neurosurg Focus 2022 Oct;53(4):E7. doi: 10.3171/2022.7.FOCUS2254. PMID: 36183180
Gupta P, Siraj F, Malik A, Shankar KB
J Cancer Res Ther 2021 Jul-Sep;17(4):912-916. doi: 10.4103/jcrt.JCRT_632_19. PMID: 34528541
Onishi S, Amatya VJ, Kolakshyapati M, Takano M, Yonezawa U, Taguchi A, Kaichi Y, Takeshima Y, Awai K, Sugiyama K, Kurisu K, Yamasaki F
Eur J Radiol 2020 May;126:108924. Epub 2020 Mar 2 doi: 10.1016/j.ejrad.2020.108924. PMID: 32193035
Rivera B, Gayden T, Carrot-Zhang J, Nadaf J, Boshari T, Faury D, Zeinieh M, Blanc R, Burk DL, Fahiminiya S, Bareke E, Schüller U, Monoranu CM, Sträter R, Kerl K, Niederstadt T, Kurlemann G, Ellezam B, Michalak Z, Thom M, Lockhart PJ, Leventer RJ, Ohm M, MacGregor D, Jones D, Karamchandani J, Greenwood CM, Berghuis AM, Bens S, Siebert R, Zakrzewska M, Liberski PP, Zakrzewski K, Sisodiya SM, Paulus W, Albrecht S, Hasselblatt M, Jabado N, Foulkes WD, Majewski J
Acta Neuropathol 2016 Jun;131(6):847-63. Epub 2016 Feb 26 doi: 10.1007/s00401-016-1549-x. PMID: 26920151Free PMC Article
Komori T, Arai N
Neuropathology 2013 Aug;33(4):459-68. Epub 2013 Mar 27 doi: 10.1111/neup.12033. PMID: 23530928

Recent systematic reviews

Gruber MD, Pindrik J, Damante M, Schulz L, Shaikhouni A, Leonard JR
J Neurosurg Pediatr 2023 Nov 1;32(5):576-583. Epub 2023 Aug 11 doi: 10.3171/2023.6.PEDS22440. PMID: 37728409
Wang J, Luo X, Chen C, Deng J, Long H, Yang K, Qi S
Neurosurg Focus 2022 Oct;53(4):E7. doi: 10.3171/2022.7.FOCUS2254. PMID: 36183180
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
Bernardo P, Cinalli G, Santoro C
Childs Nerv Syst 2020 Oct;36(10):2333-2350. Epub 2020 Jul 1 doi: 10.1007/s00381-020-04710-7. PMID: 32613422
Pindrik J, Hoang N, Smith L, Halverson M, Wojnaroski M, McNally K, Gedela S, Ostendorf AP
Neurosurg Focus 2018 Sep;45(3):E3. doi: 10.3171/2018.7.FOCUS18220. PMID: 30173613

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