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Diffuse leptomeningeal glioneuronal tumor

MedGen UID:
1391318
•Concept ID:
C4329735
•
Neoplastic Process
Synonyms: Diffuse leptomeningeal glioneuronal neoplasm; Diffuse Leptomeningeal Glioneuronal Tumor; diffuse leptomeningeal glioneuronal tumor; Disseminated Oligodendroglial-Like Leptomeningeal Tumor of Childhood
SNOMED CT: Diffuse leptomeningeal glioneuronal neoplasm (733123006); Diffuse leptomeningeal glioneuronal tumor (733123006)
 
Monarch Initiative: MONDO:0858956

Definition

A relatively slow growing diffuse leptomeningeal neoplasm usually affecting children and adolescents. It is characterized by the presence of clear glial neoplastic cells reminiscent of oligodendroglioma. A neuronal component may be present. The prognosis is variable. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse leptomeningeal glioneuronal tumor

Professional guidelines

PubMed

Pediatric Diffuse Leptomeningeal Glioneuronal Tumors: Diagnosis, Follow-up, and Treatment Options.
Ozen A, Tanrikulu B, Danyeli AE, Ozek MM
Turk Neurosurg 2024;34(3):441-447. doi: 10.5137/1019-5149.JTN.43742-23.2. PMID: 38650559
Diffuse Leptomeningeal Glioneuronal Tumors: A Case Series of Five Patients with Parenchymal Forms and an Analysis of the Diagnostic Challenges, Treatment Options and Outcomes.
Demir MK, Yapıcıer O, Kılıc T, Celik S, Oven BB, Ozdamarlar U, Kundak NE, Kanan D, Dincer A, Ertem O, Yakut T, Yılmaz B, Safi S
Curr Med Imaging 2023;19(12):1415-1426. doi: 10.2174/1573405619666230213152949. PMID: 36788686
The histomolecular criteria established for adult anaplastic pilocytic astrocytoma are not applicable to the pediatric population.
Gareton A, Tauziède-Espariat A, Dangouloff-Ros V, Roux A, Saffroy R, Castel D, Kergrohen T, Fina F, Figarella-Branger D, Pagès M, Bourdeaut F, Doz F, Puget S, Dufour C, Lechapt E, Chrétien F, Grill J, Varlet P
Acta Neuropathol 2020 Feb;139(2):287-303. Epub 2019 Nov 1 doi: 10.1007/s00401-019-02088-8. PMID: 31677015Free PMC Article

Recent clinical studies

Etiology

Imaging of pediatric glioneuronal and neuronal tumors.
Pai V, Laughlin S, Ertl-Wagner B
Childs Nerv Syst 2024 Oct;40(10):3007-3026. Epub 2024 Jul 3 doi: 10.1007/s00381-024-06502-9. PMID: 38960918
Understanding diffuse leptomeningeal glioneuronal tumors.
Bajin IY, Levine A, Dewan MC, Bennett J, Tabori U, Hawkins C, Bouffet E
Childs Nerv Syst 2024 Aug;40(8):2359-2366. Epub 2024 May 25 doi: 10.1007/s00381-024-06432-6. PMID: 38795167
Pediatric Diffuse Leptomeningeal Glioneuronal Tumors: Diagnosis, Follow-up, and Treatment Options.
Ozen A, Tanrikulu B, Danyeli AE, Ozek MM
Turk Neurosurg 2024;34(3):441-447. doi: 10.5137/1019-5149.JTN.43742-23.2. PMID: 38650559
Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.
Wiśniewski K, Brandel MG, Gonda DD, Crawford JR, Levy ML
Childs Nerv Syst 2022 Sep;38(9):1663-1673. Epub 2022 Jul 22 doi: 10.1007/s00381-022-05600-w. PMID: 35867118
Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features.
Deng MY, Sill M, Chiang J, Schittenhelm J, Ebinger M, Schuhmann MU, Monoranu CM, Milde T, Wittmann A, Hartmann C, Sommer C, Paulus W, Gärtner J, Brück W, Rüdiger T, Leipold A, Jaunmuktane Z, Brandner S, Giangaspero F, Nozza P, Mora J, Morales la Madrid A, Cruz Martinez O, Hansford JR, Pietsch T, Tietze A, Hernáiz-Driever P, Stoler I, Capper D, Korshunov A, Ellison DW, von Deimling A, Pfister SM, Sahm F, Jones DTW
Acta Neuropathol 2018 Aug;136(2):239-253. Epub 2018 May 15 doi: 10.1007/s00401-018-1865-4. PMID: 29766299

Diagnosis

Understanding diffuse leptomeningeal glioneuronal tumors.
Bajin IY, Levine A, Dewan MC, Bennett J, Tabori U, Hawkins C, Bouffet E
Childs Nerv Syst 2024 Aug;40(8):2359-2366. Epub 2024 May 25 doi: 10.1007/s00381-024-06432-6. PMID: 38795167
Dural and Leptomeningeal Diseases: Anatomy, Causes, and Neuroimaging Findings.
Kurokawa R, Kurokawa M, Isshiki S, Harada T, Nakaya M, Baba A, Naganawa S, Kim J, Bapuraj J, Srinivasan A, Abe O, Moritani T
Radiographics 2023 Aug;43(9):e230039. doi: 10.1148/rg.230039. PMID: 37535461
Contemporary outcomes of diffuse leptomeningeal glioneuronal tumor in pediatric patients: A case series and literature review.
Lu VM, Di L, Gernsback J, Eichberg DG, Luther EM, Shah AH, Daniels DJ, Maher OM, Niazi TN
Clin Neurol Neurosurg 2022 Jul;218:107265. Epub 2022 Apr 30 doi: 10.1016/j.clineuro.2022.107265. PMID: 35567835
Emerging glioneuronal and neuronal tumors: case-based review.
Lim SD, Kim SI, Park JW, Won JK, Kim SK, Phi JH, Chung CK, Choi SH, Yun H, Park SH
Brain Tumor Pathol 2022 Apr;39(2):65-78. Epub 2022 Jan 20 doi: 10.1007/s10014-021-00420-3. PMID: 35048219
Diffuse Leptomeningeal Glioneuronal Tumor of Childhood.
Lakhani DA, Mankad K, Chhabda S, Feizi P, Patel R, Sarma A, Pruthi S
AJNR Am J Neuroradiol 2020 Nov;41(11):2155-2159. Epub 2020 Sep 10 doi: 10.3174/ajnr.A6737. PMID: 32912870Free PMC Article

Therapy

Vision treatment strategy for acute blindness in adolescent female with diffuse leptomeningeal glioneuronal tumor.
Zhou S, Zhang M, Wu H, Liu G, Wang Y, Bao Y
Int J Neurosci 2020 Nov;130(11):1170-1173. Epub 2020 Feb 25 doi: 10.1080/00207454.2020.1730364. PMID: 32067532

Prognosis

Understanding diffuse leptomeningeal glioneuronal tumors.
Bajin IY, Levine A, Dewan MC, Bennett J, Tabori U, Hawkins C, Bouffet E
Childs Nerv Syst 2024 Aug;40(8):2359-2366. Epub 2024 May 25 doi: 10.1007/s00381-024-06432-6. PMID: 38795167
Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.
Wiśniewski K, Brandel MG, Gonda DD, Crawford JR, Levy ML
Childs Nerv Syst 2022 Sep;38(9):1663-1673. Epub 2022 Jul 22 doi: 10.1007/s00381-022-05600-w. PMID: 35867118
Contemporary outcomes of diffuse leptomeningeal glioneuronal tumor in pediatric patients: A case series and literature review.
Lu VM, Di L, Gernsback J, Eichberg DG, Luther EM, Shah AH, Daniels DJ, Maher OM, Niazi TN
Clin Neurol Neurosurg 2022 Jul;218:107265. Epub 2022 Apr 30 doi: 10.1016/j.clineuro.2022.107265. PMID: 35567835
Pediatric midline H3K27M-mutant tumor with disseminated leptomeningeal disease and glioneuronal features: case report and literature review.
Navarro RE, Golub D, Hill T, McQuinn MW, William C, Zagzag D, Hidalgo ET
Childs Nerv Syst 2021 Jul;37(7):2347-2356. Epub 2020 Sep 28 doi: 10.1007/s00381-020-04892-0. PMID: 32989496
A Diffuse Leptomeningeal Glioneuronal Tumor Without Diffuse Leptomeningeal Involvement: Detailed Molecular and Clinical Characterization.
Kang JH, Buckley AF, Nagpal S, Fischbein N, Peters KB
J Neuropathol Exp Neurol 2018 Sep 1;77(9):751-756. doi: 10.1093/jnen/nly053. PMID: 29931222

Clinical prediction guides

Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.
Wiśniewski K, Brandel MG, Gonda DD, Crawford JR, Levy ML
Childs Nerv Syst 2022 Sep;38(9):1663-1673. Epub 2022 Jul 22 doi: 10.1007/s00381-022-05600-w. PMID: 35867118
Contemporary outcomes of diffuse leptomeningeal glioneuronal tumor in pediatric patients: A case series and literature review.
Lu VM, Di L, Gernsback J, Eichberg DG, Luther EM, Shah AH, Daniels DJ, Maher OM, Niazi TN
Clin Neurol Neurosurg 2022 Jul;218:107265. Epub 2022 Apr 30 doi: 10.1016/j.clineuro.2022.107265. PMID: 35567835
Diffuse leptomeningeal glioneuronal tumor: A review of diagnosis and management with an illustrative case.
Al-Ghanem R, Luque Barona R, Godoy-Hurtado A, Galicia Bulnes JM, El-Rubaidi O
Neurocirugia (Astur : Engl Ed) 2022 Nov-Dec;33(6):389-393. Epub 2022 Mar 7 doi: 10.1016/j.neucie.2022.02.006. PMID: 35272977
Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features.
Deng MY, Sill M, Chiang J, Schittenhelm J, Ebinger M, Schuhmann MU, Monoranu CM, Milde T, Wittmann A, Hartmann C, Sommer C, Paulus W, Gärtner J, Brück W, Rüdiger T, Leipold A, Jaunmuktane Z, Brandner S, Giangaspero F, Nozza P, Mora J, Morales la Madrid A, Cruz Martinez O, Hansford JR, Pietsch T, Tietze A, Hernáiz-Driever P, Stoler I, Capper D, Korshunov A, Ellison DW, von Deimling A, Pfister SM, Sahm F, Jones DTW
Acta Neuropathol 2018 Aug;136(2):239-253. Epub 2018 May 15 doi: 10.1007/s00401-018-1865-4. PMID: 29766299
Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple's disease: a case report and literature review.
Karlowee V, Kolakshyapati M, Amatya VJ, Takayasu T, Nosaka R, Sugiyama K, Kurisu K, Yamasaki F
Childs Nerv Syst 2017 Aug;33(8):1411-1414. Epub 2017 Apr 5 doi: 10.1007/s00381-017-3405-2. PMID: 28382437

Recent systematic reviews

Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.
Wiśniewski K, Brandel MG, Gonda DD, Crawford JR, Levy ML
Childs Nerv Syst 2022 Sep;38(9):1663-1673. Epub 2022 Jul 22 doi: 10.1007/s00381-022-05600-w. PMID: 35867118

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