Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Dec 21, 2020; 26(47): 7584-7592
Published online Dec 21, 2020. doi: 10.3748/wjg.v26.i47.7584
Multiple cerebral lesions in a patient with refractory celiac disease: A case report
Lena Horvath, Georg Oberhuber, Andreas Chott, Maria Effenberger, Herbert Tilg, Eberhard Gunsilius, Dominik Wolf, Sarah Iglseder
Lena Horvath, Eberhard Gunsilius, Dominik Wolf, Department of Internal Medicine V (Hematology and Medical Oncology), Medical University Innsbruck, Innsbruck 6020, Austria
Georg Oberhuber, InnPath GmbH, Institute of Pathology, Innsbruck 6020, Austria
Andreas Chott, Ottakring Clinic, Institute of Pathology and Microbiology, Vienna 1160, Austria
Maria Effenberger, Herbert Tilg, Department of Internal Medicine I (Gastroenterology, Hepatology, Endocrinology and Metabolism), Medical University Innsbruck, Innsbruck 6020, Austria
Sarah Iglseder, Department of Neurology, Medical University Innsbruck, Innsbruck 6020, Austria
Author contributions: Horvath L and Iglseder S wrote and corrected the manuscript; Oberhuber G, Gunsilius E, Wolf D, Effenberger M, Tilg H and Chott A reviewed and corrected the manuscript; Oberhuber G and Iglseder S provided the figures; Iglseder S was the patient´s neurologist; Effenberger M and Tilg H were the patient´s gastroenterologists; Horvath L, Gunsilius E and Wolf D were the patient´s haematologists; Oberhuber G and Chott A were the pathologists involved; all authors issued final approval for the version to be submitted.
Informed consent statement: Any and all details that might disclose the identity of the patient described in this report are anonymized. The patient has deceased at the time point of enrollment, writing and submission of this case report and therefore not informed written consent was provided.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Sarah Iglseder, MD, Academic Fellow, Department of Neurology, Medical University Innsbruck, Anichstrasse 35, Innsbruck 6020, Austria. sarah.iglseder@tirol-kliniken.at
Received: August 24, 2020
Peer-review started: August 24, 2020
First decision: October 18, 2020
Revised: October 31, 2020
Accepted: November 12, 2020
Article in press: November 12, 2020
Published online: December 21, 2020
Abstract
BACKGROUND

Enteropathy-associated T cell lymphoma (EATL) is an aggressive intestinal T cell lymphoma derived from intraepithelial lymphocytes, which occurs in individuals with celiac disease (CD). Cerebral involvement is an extremely rare condition and as described so far, lymphoma lesions may present as parenchymal predo-minantly supratentorial or leptomeningeal involvement. We describe a case of EATL with multifocal supra- and infratentorial brain involvement in a patient with refractory celiac disease (RCD).

CASE SUMMARY

A 58-years old man with known CD developed ulcerative jejunitis and was diagnosed with RCD type II. Six months later he presented with subacute cerebellar symptoms (gait ataxia, double vision, dizziness). Cranial magnetic resonance imaging (MRI) revealed multifocal T2 hyperintense supra- and infratentorial lesions. Laboratory studies of blood and cerebrospinal fluid were inconspicuous for infectious, inflammatory or autoimmune diseases. 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (18FDG-PET/CT) scan showed a suspect hypermetabolic lesion in the left upper abdomen and consequent surgical jejunal resection revealed the diagnosis of EATL. During the diagnostic work-up, neurological symptoms aggravated and evolved refractory to high-dosage cortisone. Recurrent MRI scans showed progressive cerebral lesions, highly suspicious for lymphoma and methotrexate chemotherapy was initiated. Unfortunately, clinically the patient responded only transiently. Finally, cerebral biopsy confirmed the diagnosis of cerebral involvement of EATL. Considering the poor prognosis and deterioration of the performance status, best supportive care was started. The patient passed away three weeks after diagnosis.

CONCLUSION

EATL with cerebral involvement must be considered as a possible differential diagnosis in patients with known RCD presenting with neurological symptoms.

Keywords: Enteropathy-associated T cell lymphoma, Brain neoplasm, Celiac disease, Cerebellar syndrome, Case report

Core Tip: Enteropathy-associated T cell lymphoma (EATL) is a rare type of peripheral T cell lymphoma frequently evolving from refractory celiac disease (RCD) type II. The prognosis is often dismal due to its aggressive clinical behavior. Extraintestinal manifestation may occur, but concerning cerebral involvement, only very few reports have been described so far, showing parenchymal lesions with predominantly supratentorial or leptomeningeal involvement. This case shows multifocal supratentorial, brainstem and cerebellar involvement of EATL in a patient with RCD type II, with a rarely observed gamma/delta T cell receptor immunophenotype. This report underlines the importance to consider EATL with cerebral involvement in patients with RCD who develop neurological symptoms.