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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Integrated genotype-phenotype analysis of long-term epilepsy-associated ganglioglioma.
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
Surgical treatment of thalamic tumors in children.
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

Clinico-pathological and epigenetic heterogeneity of diffuse gliomas with FGFR3::TACC3 fusion.
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
Central nervous system involvement in individuals with RASopathies.
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
Clinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.
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
Dysembryoplastic Neuroepithelial Tumors: What You Need to Know.
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
Dysembryoplastic neuroepithelial tumor: a clinical, neuroradiological, and pathological study of 15 cases.
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

Pediatric Neuroglial Tumors: A Review of Ependymoma and Dysembryoplastic Neuroepithelial Tumor.
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
Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series.
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
Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update.
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
Low-grade developmental and epilepsy associated brain tumors: a critical update 2020.
Slegers RJ, Blumcke I
Acta Neuropathol Commun 2020 Mar 9;8(1):27. doi: 10.1186/s40478-020-00904-x. PMID: 32151273Free PMC Article
Rare glial tumors.
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

Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
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
SURGICAL AND SEIZURE TREATMENT OUTCOMES IN ADULT DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMORS: A CASE SERIES.
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
Utility of Stereoelectroencephalography in Children with Dysembryoplastic Neuroepithelial Tumor and Cortical Malformation.
Park JT, Baca Vaca GF, Avery J, Miller JP
Neurodiagn J 2017;57(3):191-210. doi: 10.1080/21646821.2017.1326270. PMID: 28898173
Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy.
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
Dysembryoplastic neuroepithelial tumor: a curable cause of seizures.
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

Epileptogenic focal lesions in Dravet syndrome: A warning to investigators.
Ventura R, Beltrán-Corbellini Á, Toledano R, Román IS, García-Morales I, Gil-Nagel A
Epileptic Disord 2024 Apr;26(2):173-180. Epub 2023 Dec 28 doi: 10.1002/epd2.20191. PMID: 38116874
Clinico-pathological and epigenetic heterogeneity of diffuse gliomas with FGFR3::TACC3 fusion.
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
Clinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.
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
Rare glial tumors.
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
Nonmalignant pediatric brain tumors.
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

Clinico-pathological and epigenetic heterogeneity of diffuse gliomas with FGFR3::TACC3 fusion.
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
Clinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.
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
FDOPA PET-CT of Nonenhancing Brain Tumors.
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
Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors.
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
Dysembryoplastic neuroepithelial tumor, a pure glial tumor? Immunohistochemical and morphometric studies.
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

Epileptic versus neuro-oncological focus of management in pediatric patients with concurrent primary brain lesion and seizures: a systematic review.
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
Preoperative MRI for postoperative seizure prediction: a radiomics study of dysembryoplastic neuroepithelial tumor and a systematic review.
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
Prevalence of BRAFV600 in glioma and use of BRAF Inhibitors in patients with BRAFV600 mutation-positive glioma: systematic review.
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
Epilepsy in NF1: a systematic review of the literature.
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
Preoperative evaluation and surgical management of infants and toddlers with drug-resistant epilepsy.
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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