Abstract
Monomorphic epitheliotropic T-cell lymphoma (MEITL) of gastrointestinal tract is an aggressive T-cell lymphoma that can rarely involve the brain. We present detailed descriptions of clinical and autopsy neuropathological findings of a rare case of an elderly woman who had surgery and chemotherapy for MEITL of the small intestine. Following her surgery, she progressively exhibited neurologic decline towards the end of her treatment. The patient eventually succumbed to her illness and was found to have MEITL with intracranial involvement on autopsy. Brain autopsy was performed and examination of tissues with hematoxylin–eosin staining under optical microscopy with 100 X magnification. Immunostaining for CD3, CD4, CD5, CD7, CD8, CD56, CD20, beta-amyloid, c-Myc, TCR-beta, TCR-delta, and EBER-ish was conducted on the formalin-fixed paraffin-embedded (FFPE) brain tissues. A neuropathological exam revealed multifocal friable necrotic and hemorrhagic areas in the supratentorial region. Histologically, monotonous small to medium-sized atypical lymphocytes infiltrated the brain parenchyma, prominently around the vessels. The immunophenotype of the atypical lymphocytes was positive for CD-3, CD-7, and CD-56 and negative for CD-5, CD-4, CD-8, CD-20, and c-Myc. EBER-ish was negative. The histology and immunophenotype confirmed the MEITL brain involvement. Neurologic decline and cognitive changes in patients with known MEITL can be the first clue of brain involvement upon which prompt evaluation is warranted.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Data availability
The authors confirm that all data generated or analyzed during this study are included in this published article.
References
Chan J et al (n.d.) Monomorphic epitheliotropic intestinal T-cell lymphoma. Hematolymphoid Tumours (5th ed.). WHO Classification of Tumours online. https://tumourclassification.iarc.who.int/chaptercontent/63/225. Accessed 11 Oct 2022
Gandhi S et al (2019) Central nervous system involvement in peripheral T-cell lymphoma. Blood 134(Supplement_1):5293. https://doi.org/10.1182/blood-2019-131965
Chuah YY et al (2020) Enteropathy-associated T-cell Lymphoma (EATL) with intracranial metastasis: a rare and dismal condition. Acta Gastro-Enterolofica Belgica 83:77–80
Nishida H et al (2015) Isolated central nervous system relapse in type II enteropathy-associated T cell lymphoma. Rinsho Ketsueki 55(6):692–698. https://doi.org/10.11406/rinketsu.56.692
Morimoto A et al (2021) Monomorphic epitheliotropic intestinal T-cell lymphoma invades the brain. Internal Med 60(5):815–816. https://doi.org/10.2169/internalmedicine.5925-20
Kubota, Y et al (2018) Monomorphic epitheliotropic intestinal T-cell lymphoma involving the central nervous system. American Society of Hematology. Image Bank. https://imagebank.hematology.org/image/61420/monomorphic-epitheliotropic-intestinal-tcell-lymphoma-involving-the-central-nervous-system. Accessed 14 Nov 2022
Suzuki Y et al (2020) Monomorphic epitheliotropic intestinal T-cell lymphoma involving the lung and brain: a rare case stud. Internal Med 59(20):2559–2563. https://doi.org/10.2169/internalmedicine.4710-20
Chen C et al (2021) Clinicopathological and molecular genomic features of monomorphic epitheliotropic intestinal T-cell lymphoma in the Chinese population: a study of 20 cases. Diagnostic Pathol 16:114. https://doi.org/10.1186/s13000-021-01173-5
Fisher A et al (2019) Truth lies below: a case report and literature review of typical appearing polyps yet with an atypical diagnosis. World J Gastrointest Endosc 11(1):54–60. https://doi.org/10.4253/wjge.v11.i1.54
Hall W et al (2001) Improving diagnostic yield in brain biopsy: coupling spectroscopic targeting with real-time needle placement. J Magn Reson Imaging. 13 (1). https://doi.org/10.1002/1522-2586(200101)13:1<12::AID-JMRI1002>3.0.CO;2-Q
Eng JA et al (2004) Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 55:250–325
Funding
This study was not supported by any funding.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Ethical approval
This is an observational study. The SUNY Downstate Research Ethics Committee has confirmed that no ethical approval is required.
Informed consent
For this type of study, formal consent is not required.
Consent for publication
Consent for publication was obtained for every individual person’s data included in the study.
Conflict of interest
The authors declare no competing interests.
Additional information
Publisher's note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Talabong, D.J.A., Laguerre, E., Chen, N.N. et al. Monomorphic epitheliotropic intestinal T-cell lymphoma involving the central nervous system: a rare case report with comprehensive autopsy neuropathological examinations. J Hematopathol 16, 155–160 (2023). https://doi.org/10.1007/s12308-023-00557-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12308-023-00557-4