Advertisement

Springer Nature is making Coronavirus research free. View research | View latest news | Sign up for updates

Diffuse leptomeningeal glioneuronal tumor in a Chinese adult: a novel case report and review of literature

Abstract

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare glioma tumor classified by the World Health Organization as a central nervous system tumor in 2016. DLGNT is most common in children and adolescents but is rare in adults. A 25-year-old male patient was admitted due to recurrent seizures. Head magnetic resonance imaging revealed lesions in the right temporal lobe, which were considered to be intracranial tumors with variable properties. The patient was admitted for surgical treatment. After admission, it was confirmed that seizures were associated with right temporal lobe lesions. Right temporal epileptogenic focus resection was performed by craniotomy. Immunohistochemistry revealed that tumor cells were reactive for oligodendrocyte transcription factor 2, synaptophysin, S100 proteins, and α-thalassemia mental retardation X-linked; and partially reactive for neuronal nuclei, glial fibrillary acidic protein, and nestin. The vascular wall was reactive for vimentin, CD34, CD31, and smooth muscle actin. Ki-67 was 4%. Molecular detection demonstrated 1p36 deletion, O6-methylguanine-DNA-methyltransferase methylation, and positive v-raf murine sarcoma viral oncogene homolog B mutation. DLGNT. The patient recovered well after surgery and received 54 Gy/27f radiotherapy without neurological dysfunction and seizures. In this study, onset age, tumor site, tumor increment coefficient, molecular detection, treatment methods, and prognosis of 54 patients were summarized from 19 studies. DLGNT patients are characterized by more singular tumor site, smaller volume, lower increment coefficient, and longer stable disease period. Patients with DLGNT may also have a longer stable condition in cases of molecular detection of 1p/19q deletion, or BRAF fusion.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2
Fig. 3

Abbreviations

CNS:

Central nervous system

CT:

Computed tomography

CV:

Carboplatin/vincristine

DLGNT:

Diffuse leptomeningeal glioneuronal tumor

EEG:

Electroencephalogram

IHC:

Immunohistochemistry

MRI:

Magnetic resonance imaging

MSL:

Multiple site lesion

PG:

Poor prognosis group

SG:

Stable condition group

TMZ:

Temozolomide

VEEG:

Video electroencephalogram

WHO:

World Health Organization

Olig-2:

Oligodendrocyte transcription factor 2

ATRX:

α-Thalassemia mental retardation X-linked

NeuN:

Neuronal nuclei

GFAP:

Glial fibrillary acidic protein

SMA:

Smooth muscle actin

BRAF:

v-raf murine sarcoma viral oncogene homolog B

CSF:

Cerebrospinal fluid

MSLs:

Multiple site lesions

References

  1. 1.

    Louis DN, Perry A, Reifenberger G et al (2016) The 2016 world health organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. https://doi.org/10.1007/s00401-016-1545-1

  2. 2.

    Demir MK, Yapıcıer O, Yılmaz B et al (2018) Magnetic resonance imaging findings of mixed neuronal–glial tumors with pathologic correlation: a review. Acta Neurol Belg 118:379–386. https://doi.org/10.1007/s13760-018-0981-1

  3. 3.

    Li Y, Qian Z, Xu K et al (2017) Radiomic features predict Ki-67 expression level and survival in lower grade gliomas. J Neuro-Oncol 135:317–324. https://doi.org/10.1007/s11060-017-2576-8

  4. 4.

    Gardiman MP, Fassan M, Orvieto E et al (2010) Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain Pathol 20:361–366. https://doi.org/10.1111/j.1750-3639.2009.00285.x

  5. 5.

    Preuss M, Christiansen H, Merkenschlager A et al (2015) Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity. J Neuro-Oncol 124:65–74. https://doi.org/10.1007/s11060-015-1735-z

  6. 6.

    Fiaschi P, Badaloni F, Cagetti B et al (2018) Disseminated oligodendroglial-like leptomeningeal tumor in the adult: case report and review of the literature. World Neurosurg 114:53–57. https://doi.org/10.1016/j.wneu.2018.02.160

  7. 7.

    Kang JH, Buckley AF, Nagpal S, Fischbein N, Peters KB (2018) A diffuse leptomeningeal glioneuronal tumor without diffuse leptomeningeal involvement: detailed molecular and clinical characterization. J Neuropathol Exp Neurol 77:751–756. https://doi.org/10.1093/jnen/nly053

  8. 8.

    Chen WJ, He DS, Tang RX, Ren FH, Chen G (2015) Ki-67 is a valuable prognostic factor in gliomas: evidence from a systematic review and meta-analysis. Asian Pac J Cancer Prev 16:411–420. https://doi.org/10.7314/apjcp.2015.16.2.411

  9. 9.

    Kuriyama H, Lamborn KR, O’Fallon JR et al (2002) Prognostic significance of an apoptotic index and apoptosis/proliferation ratio for patients with high-grade astrocytomas. Neuro Oncol 4:179–186. https://doi.org/10.1093/neuonc/4.3.179

  10. 10.

    Torp SH (1997) Proliferative activity in human glioblastomas: evaluation of different Ki67 equivalent antibodies. Mol Pathol 50:198–200. https://doi.org/10.1136/mp.50.4.198

  11. 11.

    Vaquero J, Zurita M, Morales C, Oya S, Coca S (2000) Prognostic significance of endothelial surface score and MIB-1 labeling index in glioblastoma. J Neurooncol 46(1):11–16. https://doi.org/10.1023/a:1006347919565

  12. 12.

    Lyle MR, Dolia JN, Fratkin J, Nichols TA, Herrington BL (2015) Newly identified characteristics and suggestions for diagnosis and treatment of diffuse leptomeningeal glioneuronal/neuroepithelial tumors: a case report and review of the literature. Child Neurol Open 2015:1–7. https://doi.org/10.1177/2329048x14567531

  13. 13.

    Lee JK, Ko HC, Choi JG, Lee YS, Son BC (2018) A case of diffuse leptomeningeal glioneuronal tumor misdiagnosed as chronic tuberculous meningitis without brain biopsy. Case Rep Neurol Med 2018:1–7. https://doi.org/10.1155/2018/1391943

  14. 14.

    Aguilera D, Castellino RC, Janss A et al (2018) Clinical responses of patients with diffuse leptomeningeal glioneuronal tumors to chemotherapy. Child’s Nervous System 34:329–334. https://doi.org/10.1007/s00381-017-3584-x

  15. 15.

    Nambirajan A, Suri V, Kedia S et al (2018) Paediatric diffuse leptomeningeal tumor with glial and neuronal differentiation harbouring chromosome 1p/19q co-deletion and H3.3 K27M mutation: unusual molecular profile and its therapeutic implications. Brain Tumor Pathol 35:186–191. https://doi.org/10.1007/s10014-018-0325-0

  16. 16.

    Karlowee V, Kolakshyapati M, Amatya VJ et al (2017) Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple’s disease: a case report and literature review. Child’s Nerv Syst 33:1411–1414. https://doi.org/10.1007/s00381-017-3405-2

  17. 17.

    Peerboccus M, Beltran-Marin M, Sariban E, Fontanges Q, Ziereisen F (2017) Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive entity revised and correlated with pathology. J BELG SOC RADIOL 101:1–5. https://doi.org/10.5334/jbr-btr.1012

  18. 18.

    Schwetye KE, Kansagra AP, McEachern J et al (2017) Unusual high-grade features in pediatric diffuse leptomeningeal glioneuronal tumor: comparison with a typical low-grade example. Hum Pathol 70:105–112. https://doi.org/10.1016/j.humpath.2017.06.004

  19. 19.

    Chiang J, Dalton J, Upadhyaya SA et al (2019) Chromosome arm 1q gain is an adverse prognostic factor in localized and diffuse leptomeningeal glioneuronal tumors with BRAF gene fusion and 1p deletion. Acta Neuropathol 137:179–181. https://doi.org/10.1007/s00401-018-1940-x

  20. 20.

    Dodgshun AJ, SantaCruz N, Hwang J et al (2016) Disseminated glioneuronal tumors occurring in childhood: treatment outcomes and BRAF alterations including V600E mutation. J Neuro-Oncol 128:293–302. https://doi.org/10.1007/s11060-016-2109-x

  21. 21.

    Acar NP, Oguz KK, Kurne AT et al (2015) Disseminated cystic lesions: a case of disseminated oligodendroglial-like leptomeningeal tumor. Neurology 84:1382–1383. https://doi.org/10.1212/wnl.0000000000001410

  22. 22.

    Kessler BA, Bookhout C, Jaikumar S, Hipps J, Lee YZ (2015) Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report. CLIN IMAG 39:300–304. https://doi.org/10.1016/j.clinimag.2014.11.018

  23. 23.

    Ruppert B, Welsh CT, Hannah J et al (2011) Glioneuronal tumor with neuropil-like islands of the spinal cord with diffuse leptomeningeal neuraxis dissemination. J Neuro-Oncol 104:529–533. https://doi.org/10.1007/s11060-010-0505-1

  24. 24.

    Wei J, Xi-rui H, Qiong W, Qiu-ping G (2018) Mi man xing ruan nao mo jiao zhi shen jing yuan zhong liu [Diffuse leptomeningeal glioneuronal tumor]. Chinese Journal of Contemporary Neurology and Neurosurgery 18:527–534. https://doi.org/10.3969/j.issn.1672-6731.2018.07.010

  25. 25.

    Dabiao D, Jun G, Hainan L, Shaoqiang X, Wensheng W (2019) Mi man ruan nao mo jiao zhi shen jing yuan zhong liu yi li [Diffuse leptomeningeal glioneuronal tumor: a case report]. Chinese Journal of Radiology 53:145–146. https://doi.org/10.3760/cma.j.issn.1005-1201.2019.02.012

  26. 26.

    Shanshan C, Xueyong L, Yupeng C et al (2019) Mi man xing ruan nao mo jiao zhi shen jing yuan zhong liu yi li [Diffuse leptomeningeal glioneuronal tumor: report of a case]. Chin J Pathol 48:253–255. https://doi.org/10.3760/cma.j.issn.0529-5807.2019.03.019

  27. 27.

    Schniederjan MJ, Alghamdi S, Castellano-Sanchez A et al (2013) Diffuse leptomeningeal neuroepithelial tumor: 9 pediatric cases with chromosome 1p/19q deletion status and IDH1 (R132H) immunohistochemistry. Am J Surg Pathol 37:763–771. https://doi.org/10.1097/pas.0b013e31827bf4cc

Download references

Acknowledgements

This study was supported by grants from Nanjing medical science and technology development project (Grant No. YKK17136 to Haitao Zhu).

Author information

Correspondence to Rui Zhang.

Ethics declarations

Conflict of interest

All authors disclose no conflicts of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

For this type of study, formal consent is not required.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

The original article was revised to withdraw the open access.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

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 (2019). https://doi.org/10.1007/s13760-019-01262-9

Download citation

Keywords

  • Cerebral tumor
  • Glioneuronal
  • Leptomeningeal
  • Prognosis