Diffuse leptomeningeal glioneuronal tumor in an 8-year-old girl: case report and review of the literature

被引：4

作者：

Cambruzzi, Eduardo ^{[1
,2
,3
,4
,5
]}

Medeiros, Mateus Scarabelot ^{[3
]}

Cardoso, Carmo Eduardo ^{[3
]}

Silva, Guilherme Alberto Germano ^{[3
]}

Schlotte, Kelly ^{[3
]}

Kus, Willian Pegoraro ^{[3
]}

机构：

[1] Univ Fed Rio Grande do Sul, Porto Alegre, RS, Brazil

[2] Santa Rita Hosp, Dept Pathol, Santa Casa Hosp Complex,Rua Sarmento Leite 1870, Porto Alegre, RS, Brazil

[3] Conceicao Hosp Grp, Porto Alegre, RS, Brazil

[4] Univ Fdn Cardiol, Inst Cardiol, Porto Alegre, RS, Brazil

[5] Univ Vale Rio dos Sinos, Fac Med, Sao Leopoldo, RS, Brazil

来源：

CHILDS NERVOUS SYSTEM | 2023年 / 39卷 / 01期

关键词：

Diffuse leptomeningeal glioneuronal tumor; Leptomeningeal neoplasms; Pediatrics; Neurosurgery; DLGNT;

D O I：

10.1007/s00381-022-05625-1

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are rare central nervous system tumors of childhood that were recently described as a new entity. DLGNTs usually manifest with symptoms related to increased intracranial pressure or spinal cord compression. The classic radiological feature is a widespread leptomeningeal enhancement that may involve the entire neuroaxis. Microscopic examination demonstrates oligodendroglial-like cells that are positive for OLIG2, MAP2, and S100 and negative for IDH-1. Anaplastic features occur in some cases. Molecularly, DLGNTs are characterized by chromosome arm 1p deletion and alteration of a mitogen-activated protein kinase (MAPK) pathway gene, most commonly BRAF-KIAA1549 fusion. There is no established grading system for these tumors, which may have an indolent or aggressive behavior. Treatment usually involves chemotherapy and radiation therapy.

引用

页码：301 / 305

页数：5

共 12 条

[1] Primary diffuse leptomeningeal glioneuronal tumors of the central nervous system: Report of three cases and review of literature [J].

Abongwa, Chenue ;

Cotter, Jennifer ;

Tamrazi, Benita ;

Dhall, Girish ;

Davidson, Tom ;

Margol, Ashley .

PEDIATRIC HEMATOLOGY AND ONCOLOGY, 2020, 37 (03) :248-258

[2] Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy [J].

Aguilera, Dolly ;

Castellino, Robert Craig ;

Janss, Anna ;

Schniederjan, Mathew ;

McNall, Renee ;

MacDonald, Tobey ;

Mazewski, Claire .

CHILDS NERVOUS SYSTEM, 2018, 34 (02) :329-334

[3] Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features [J].

Deng, Maximilian Y. ;

Sill, Martin ;

Chiang, Jason ;

Schittenhelm, Jens ;

Ebinger, Martin ;

Schuhmann, Martin U. ;

Monoranu, Camelia-Maria ;

Milde, Till ;

Wittmann, Andrea ;

Hartmann, Christian ;

Sommer, Clemens ;

Paulus, Werner ;

Gaertner, Jutta ;

Brueck, Wolfgang ;

Ruediger, Thomas ;

Leipold, Alfred ;

Jaunmuktane, Zane ;

Brandner, Sebastian ;

Giangaspero, Felice ;

Nozza, Paolo ;

Mora, Jaume ;

Morales la Madrid, Andres ;

Cruz Martinez, Ofelia ;

Hansford, Jordan R. ;

Pietsch, Torsten ;

Tietze, Anna ;

Hernaiz-Driever, Pablo ;

Stoler, Iris ;

Capper, David ;

Korshunov, Andrey ;

Ellison, David W. ;

von Deimling, Andreas ;

Pfister, Stefan M. ;

Sahm, Felix ;

Jones, David T. W. .

ACTA NEUROPATHOLOGICA, 2018, 136 (02) :239-253

[4] Disseminated glioneuronal tumors occurring in childhood: treatment outcomes and BRAF alterations including V600E mutation [J].

Dodgshun, Andrew J. ;

SantaCruz, Nadine ;

Hwang, Jaeho ;

Ramkissoon, Shakti H. ;

Malkin, Hayley ;

Bergthold, Guillaume ;

Manley, Peter ;

Chi, Susan ;

MacGregor, Duncan ;

Goumnerova, Liliana ;

Sullivan, Michael ;

Ligon, Keith ;

Beroukhim, Rameen ;

Herrington, Betty ;

Kieran, Mark W. ;

Hansford, Jordan R. ;

Bandopadhayay, Pratiti .

JOURNAL OF NEURO-ONCOLOGY, 2016, 128 (02) :293-302

[5] Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple's disease: a case report and literature review [J].

Karlowee, Vega ;

Kolakshyapati, Manish ;

Amatya, Vishwa Jeet ;

Takayasu, Takeshi ;

Nosaka, Ryo ;

Sugiyama, Kazuhiko ;

Kurisu, Kaoru ;

Yamasaki, Fumiyuki .

CHILDS NERVOUS SYSTEM, 2017, 33 (08) :1411-1414

[6] Diffuse Leptomeningeal Glioneuronal Tumor of Childhood [J].

Lakhani, D. A. ;

Mankad, K. ;

Chhabda, S. ;

Feizi, P. ;

Patel, R. ;

Sarma, A. ;

Pruthi, S. .

AMERICAN JOURNAL OF NEURORADIOLOGY, 2020, 41 (11) :2155-2159

[7]

Lyle Megan R, 2015, Child Neurol Open, V2, p2329048X14567531, DOI [10.1177/2329048x14567531, 10.1177/2329048X14567531]

[8] Diffuse leptomeningeal glioneuronal tumour (DLGNT) in children: the emerging role of genomic analysis [J].

Manoharan, Neevika ;

Ajuyah, Pamela ;

Senapati, Akanksha ;

Wong, Marie ;

Mullins, Anna ;

Rodriguez, Michael ;

Doyle, Helen ;

McCowage, Geoff ;

Lau, Loretta M. S. ;

Ekert, Paul G. ;

Ziegler, David S. .

ACTA NEUROPATHOLOGICA COMMUNICATIONS, 2021, 9 (01)

[9] High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN) [J].

Rodriguez, Fausto J. ;

Schniederjan, Matthew J. ;

Nicolaides, Theo ;

Tihan, Tarik ;

Burger, Peter C. ;

Perry, Arie .

ACTA NEUROPATHOLOGICA, 2015, 129 (04) :609-610

[10] Diffuse Leptomeningeal Glioneuronal Tumour with 9-Year Follow-Up: Case Report and Review of the Literature [J].

Sarkinaite, Milda ;

Devyziene, Indre ;

Makstiene, Jurgita ;

Matukevicius, Algimantas ;

Gleizniene, Rymante .

DIAGNOSTICS, 2022, 12 (02)

← 1 2 →