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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

Métais A, Tauziède-Espariat A, Garcia J, Appay R, Uro-Coste E, Meyronet D, Maurage CA, Vandenbos F, Rigau V, Chiforeanu DC, Pallud J, Senova S, Saffroy R, Colin C, Edjlali M, Varlet P, Figarella-Branger D; Biopathology RENOCLIP-LOC network
Acta Neuropathol Commun 2023 Jan 16;11(1):14. doi: 10.1186/s40478-023-01506-z. PMID: 36647073Free PMC Article
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
Luzzi S, Elia A, Del Maestro M, Elbabaa SK, Carnevale S, Guerrini F, Caulo M, Morbini P, Galzio R
World Neurosurg 2019 Jul;127:255-265. Epub 2019 Apr 11 doi: 10.1016/j.wneu.2019.04.056. PMID: 30981794
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

Diagnosis

Arfuso M, Kuril S, Shah H, Hanson D
Pediatr Neurol 2024 Jul;156:139-146. Epub 2024 May 3 doi: 10.1016/j.pediatrneurol.2024.04.022. PMID: 38781722
Rahim S, Ud Din N, Abdul-Ghafar J, Chundriger Q, Khan P, Ahmad Z
J Med Case Rep 2023 Aug 1;17(1):327. doi: 10.1186/s13256-023-04062-1. PMID: 37525202Free PMC Article
Ahluwalia R, Miles L, Hayes L, Scherer A
Childs Nerv Syst 2020 Jun;36(6):1127-1130. Epub 2020 Apr 5 doi: 10.1007/s00381-020-04581-y. PMID: 32249358
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

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
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
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

Métais A, Tauziède-Espariat A, Garcia J, Appay R, Uro-Coste E, Meyronet D, Maurage CA, Vandenbos F, Rigau V, Chiforeanu DC, Pallud J, Senova S, Saffroy R, Colin C, Edjlali M, Varlet P, Figarella-Branger D; Biopathology RENOCLIP-LOC network
Acta Neuropathol Commun 2023 Jan 16;11(1):14. doi: 10.1186/s40478-023-01506-z. PMID: 36647073Free 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
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
Rashidi M, DaSilva VR, Minagar A, Rutka JT
Curr Neurol Neurosci Rep 2003 May;3(3):200-5. doi: 10.1007/s11910-003-0079-9. PMID: 12691624

Clinical prediction guides

Métais A, Tauziède-Espariat A, Garcia J, Appay R, Uro-Coste E, Meyronet D, Maurage CA, Vandenbos F, Rigau V, Chiforeanu DC, Pallud J, Senova S, Saffroy R, Colin C, Edjlali M, Varlet P, Figarella-Branger D; Biopathology RENOCLIP-LOC network
Acta Neuropathol Commun 2023 Jan 16;11(1):14. doi: 10.1186/s40478-023-01506-z. PMID: 36647073Free 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
Bund C, Heimburger C, Imperiale A, Lhermitte B, Chenard MP, Lefebvre F, Kremer S, Proust F, Namer IJ
Clin Nucl Med 2017 Apr;42(4):250-257. doi: 10.1097/RLU.0000000000001540. PMID: 28114224
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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