Local intracerebral form of diffuse leptomeningeal glioneuronal tumor – a new entity of the group of epileptogenic neoplasms?
V. S. Khalilov,
A. N. Kislyakov,
A. A. Kholin,
U. A. Kukota,
N. A. Medvedeva,
A. S. Shapovalov,
A. E. Druy https://doi.org/10.17650/2073-8803-2024-19-2-64-71
Abstract
Diffuse leptomeningeal glioneuronal tumor was introduced into the World Health Organization classification of central nervous system tumors in 2016. According to the actual World Health Organization classification of central nervous system tumors emerged in 2021, its reliable verification requires the combination of specific pathomorphological and molecular-genetic features as well as data of the neuroimaging. Typically occurring in children and adolescents these tumors are characterized by widespread diffuse leptomeningeal dissemination along the neuraxis and demonstrate a tendency to abundant contrast enhancement resulting in a specific magnetic resonance imaging appearance. Despite this, and the rather rare incidence, a number of publications have reported an increasing number of atypical cases of diffuse leptomeningeal glioneuronal tumor suggesting that the spectrum of clinical manifestations, molecular-genetic and radiological criteria of this tumor is not fully disclosed and requiring further comprehensive investigations. The article presents the experience of complex, interdisciplinary diagnosis of diffuse leptomeningeal glioneuronal tumor with atypical radiological picture in a child with focal structural epilepsy.
About the Authors
V. S. Khalilov
Federal Research and Clinical Center for Children and Adolescents, Federal Medical and Biological Agency of Russia; Pirogov Russian National Research Medical University, Ministry of Health of Russia
Russian Federation
Russian Federation
Varis Sadrutdinovich Khalilov
20 Moskvorechye St., Moscow 115409
1 Ostrovityanova St., 117997 Moscow
A. N. Kislyakov
Morozov Children’s City Clinical Hospital, Moscow Healthcare Department
Russian Federation
Russian Federation
1/9 4<sup>th</sup> Dobryninskiy Pereulok, Moscow 119049
A. A. Kholin
Pirogov Russian National Research Medical University, Ministry of Health of Russia
Russian Federation
Russian Federation
1 Ostrovityanova St., 117997 Moscow
U. A. Kukota
V.A. Almazov National Medical Research Center, Ministry of Health of Russia
Russian Federation
Russian Federation
2 Akkuratova St., Saint Petersburg 197341
N. A. Medvedeva
Federal Research and Clinical Center for Children and Adolescents, Federal Medical and Biological Agency of Russia; I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University)
Russian Federation
Russian Federation
20 Moskvorechye St., Moscow 115409
Build. 2, 8 Trubetskaya St., Moscow 119991
A. S. Shapovalov
V.A. Almazov National Medical Research Center, Ministry of Health of Russia
Russian Federation
Russian Federation
2 Akkuratova St., Saint Petersburg 197341
A. E. Druy
Dmitry Rogachev Federal Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation
Russian Federation
1 Samory Mashela St., Moscow 117997
References
1. Valiakhmetova E.F., Papusha L.I., Artemov A.V. et al. Diffuse leptomeningeal glioneuronal tumor in children: MR characteristics, clinical features and outcome. Four clinical cases. Rossiyskiy bioterapevticheskiy zhurnal = Russian Journal of Biotherapy 2021;20(1): 42–55. (In Russ.). DOI: 10.17650/1726-9784-2021-20-1-42-55
2. Medvedeva N.А., Khalilov V.S., Kislyakov A.N. et al. Atypical results of MR-perfusion in the diagnosis of long-term epilepsy associated tumors. REJR 2022;12(3):94–108. (In Russ.). DOI: 10.21569/2222-7415-2022-12-3-94-108
3. Khalilov V.S., Kholin A.A., Kisyakov A.N. et al. Neuroradiological and pathomorphological features of epilepsy associated brain tumors. Luchevaya diagnostika i terapiya = Diagnostic Radiology and Radiotherapy 2021;12(2):7–21. (In Russ.). DOI: 10.22328/2079-5343-2021-12-2-7-21
4. Appay R., Pages M., Colin C. et al. Diffuse leptomeningeal glioneuronal tumor: A double misnomer? A report of two cases. Acta Neuropathol Commun 2020;8:95. DOI: 10.1186/s40478-020-00978-7
5. Beck D.J.K., Russel D.S. Oligodendrogliomatosis of the cerebrospinal pathway. Brain 1942;65:352–72.
6. Cambruzzi E., Medeiros M.S., Cardoso C.E. et al. Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: Case report and review of the literature. Childs Nerv Syst 2023;39(1):301–5. DOI: 10.1007/s00381-022-05625-1
7. Cheng W., He L., Zhu J. et al. Diffuse leptomeningeal glioneuronal tumor without KIAA1549-BRAF fusion and 1p detection: A case report and review of literature. Childs Nerv Syst 2022;38:279–85. DOI: 10.1007/s00381-021-05426-y
8. Deng M.Y., Sill M., Chiang J. et al. Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features. Acta Neuropathol 2018;2(136):239–53. DOI: 10.1007/s00401-018-1865-4
9. Kang J.H., Buckley A.F., Nagpal S. et al. A diffuse leptomeningeal glioneuronal tumor without diffuse leptomeningeal involvement: Detailed molecular and clinical characterization. J Neuropathol Exp Neurol 2018;77(9):751–6. DOI: 10.1093/jnen/nly053
10. Louis D.N., Wesseling P., Aldape K. et al. cIMPACT-NOW update 6: New entity and diagnostic principle recommendations of the cIMPACT-Utrecht meeting on future CNS tumor classification and grading. Brain Pathol 2020;30:844–56. DOI: 10.1111/bpa.12832
11. Louis D.N., Perry A., Wesseling P. et al. The 2021 WHO classification of tumors of the central nervous system: A summary. Neurooncology 2021;23:1231–51. DOI: 10.1093/neuonc/noab106
12. Rodriguez F.J., Perry A., Rosenblum M.K. et al. Disseminated oligodendroglial-like leptomeningeal tumor of childhood: A distinctive clinicopathologic entity. Acta Neuropathol 2012;124(5):627–41. DOI: 10.1007/s00401-012-1037-x
13. Xu H., Chen F., Zhu H. et al. Diffuse leptomeningeal glioneuronal tumor in a Chinese adult: A novel case report and review of literature. Acta Neurol Belg 2020;120(2):247–56. DOI: 10.1007/s13760-019-01262-9
Review
For citations:
Khalilov V.S., Kislyakov A.N., Kholin A.A., Kukota U.A., Medvedeva N.A., Shapovalov A.S., Druy A.E. Local intracerebral form of diffuse leptomeningeal glioneuronal tumor – a new entity of the group of epileptogenic neoplasms? Russian Journal of Child Neurology. 2024;19(2):64-71. (In Russ.) https://doi.org/10.17650/2073-8803-2024-19-2-64-71
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