A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report

Kristof Babarczy; Zita Reisz; Elza Szabo; Cecilia Rajda; Laszlo Vecsei; Istvan Bodi; Peter Klivenyi; Tibor Hortobagyi; Levente Szalardy

doi:10.5114/fn.2020.102440

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

Current issue Archive Manuscripts accepted About the journal Special Issues Editorial board Reviewers Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures

Editorial System

Submit your Manuscript

Editorial Policies

Sarajevo Declaration on Integrity and Visibility of Scholarly Publications

4/2020
vol. 58

Send email

Copy url:

abstract:

Case report

A longitudinally extensive H3 K27M-mutant diffuse midline glioma in an elderly patient clinically mimicking central nervous system inflammation: a case report

Kristof Babarczy

¹

,

Zita Reisz

^{2, 3}

,

Elza Szabo

¹

,

Cecilia Rajda

¹

,

Laszlo Vecsei

^{1, 4}

,

Istvan Bodi

³

,

Peter Klivenyi

¹

,

Tibor Hortobagyi

²

,

Levente Szalardy

¹

Department of Neurology, Faculty of Medicine, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary
Department of Pathology, Faculty of Medicine, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary
Department of Clinical Neuropathology, King’s College Hospital, London, United Kingdom
MTA-SZTE Neuroscience Research Group, Szeged, Hungary

Folia Neuropathol 2020; 58 (4): 377-385

DOI: https://doi.org/10.5114/fn.2020.102440

Online publish date: 2021/01/11

View full text Get citation

EndNote

Papers, Reference Manager, RefWorks, Zotero

AMA

Babarczy K, Reisz Z, Szabo E, et al. A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report. Folia Neuropathologica. 2020;58(4):377-385. doi:10.5114/fn.2020.102440.

APA

Babarczy, K., Reisz, Z., Szabo, E., Rajda, C., Vecsei, L.,  & Bodi, I. et al. (2020). A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report. Folia Neuropathologica, 58(4), 377-385. https://doi.org/10.5114/fn.2020.102440

Chicago

Babarczy, Kristof, Zita Reisz, Elza Szabo, Cecilia Rajda, Laszlo Vecsei, Istvan Bodi,  and Peter Klivenyi et al. 2020. "A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report". Folia Neuropathologica 58 (4): 377-385. doi:10.5114/fn.2020.102440.

Harvard

Babarczy, K., Reisz, Z., Szabo, E., Rajda, C., Vecsei, L., Bodi, I., Klivenyi, P., Hortobagyi, T.,  and Szalardy, L. (2020). A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report. Folia Neuropathologica, 58(4), pp.377-385. https://doi.org/10.5114/fn.2020.102440

MLA

Babarczy, Kristof et al. "A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report." Folia Neuropathologica, vol. 58, no. 4, 2020, pp. 377-385. doi:10.5114/fn.2020.102440.

Vancouver

Babarczy K, Reisz Z, Szabo E, Rajda C, Vecsei L, Bodi I et al. A longitudinally extensive H3 K27M-mutant diffuse midline
glioma in an elderly patient clinically mimicking central nervous
system inflammation: a case report. Folia Neuropathologica. 2020;58(4):377-385. doi:10.5114/fn.2020.102440.

PlumX metrics:

Diffuse midline gliomas, H3 K27M-mutant, World Health Organization (WHO) grade IV represent a distinct glioma entity with a predominantly paediatric presentation and remarkably poor prognosis. This report presents a case of a 73-year-old woman with a diffuse midline glioma, H3 K27M-mutant, WHO grade IV with a remarkable longitudinal extension, extending from the cervical myelon to the basal ganglia. On imaging, the lesion was predominantly suggestive of inflammatory oedema, and it was clinically associated with progressive hemi- and later tetraparesis with severe autonomic and bulbar symptoms. Laboratory examinations suggested a generalized inflammatory process; however, neither infectious nor autoimmune aetiology could be confirmed. Biopsy was deemed unfeasible given the critical localization. Presuming a seronegative autoimmune encephalomyelitis, high-dose corticosteroid therapy and plasma exchanges were conducted, resulting in a modest but transient relief. The patient passed away two months after hospitalization. Neuropathological examination of the lesion revealed a high-grade diffuse glioma with H3 K27M mutation (grade IV). Although originally considered as a paediatric entity, our case confirms reports from recent years that diffuse midline gliomas, H3 K27M-mutant, WHO grade IV can occur in adults, even among the elderly, and can mimic inflammatory alterations, posing diagnostic difficulty. Our case is one of the oldest patients reported with this pathology, the oldest with an extensive diffusely infiltrating growth pattern, and with the most extensive lesion reported in adulthood.

keywords:

A longitudinally extensive H3 K27M-mutant diffuse midline glioma in an elderly patient clinically mimicking central nervous system inflammation: a case report

Kristof Babarczy 1 , Zita Reisz 2, 3 , Elza Szabo 1 , Cecilia Rajda 1 , Laszlo Vecsei 1, 4 , Istvan Bodi 3 , Peter Klivenyi 1 , Tibor Hortobagyi 2 , Levente Szalardy 1

diffuse intrinsic pontine glioma, diffuse midline glioma, elderly, histone, K27M

Kristof Babarczy

¹

,

Zita Reisz

^{2, 3}

,

Elza Szabo

¹

,

Cecilia Rajda

¹

,

Laszlo Vecsei

^{1, 4}

,

Istvan Bodi

³

,

Peter Klivenyi

¹

,

Tibor Hortobagyi

²

,

Levente Szalardy

¹