Clinical Manifestations of Primary CNS T-Cell Lymphoma

Although PCNSTL is exceptionally rare, we identified distinct neuroimaging patterns showing highly aggressive features on MRI but hypometabolic PET imaging, which may assist in identifying future PCNSTL cases. Despite the limited cohort size, our findings suggest that MTX-based chemotherapy with ASCT may translate into favorable outcome. We report on an ALK1-positive PCNSTL case with sustained complete remission after targeted therapy with lorlatinib.

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Publication Details

Journal
Neurology
Published
2026-03-17
DOI
https://doi.org/10.1212/wnl.0000000000214744
Primary Topic
CNS Lymphoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
0.00
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article

Clinical Manifestations of Primary CNS T-Cell Lymphoma

Robert Forbrig, E S Conner, Benjamin Englert, Leon D. Kaulen et al.
Neurology
CNS Lymphoma Diagnosis and Treatment
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Clinical Manifestations of Primary CNS T-Cell Lymphoma

Robert Forbrig, E S Conner, Benjamin Englert, Leon D. Kaulen, Andreas Straube, Nathalie L. Albert, Felix Hinz, Veit M. Stoecklein, Felix Ehret, Jonas Reis, Florian Ringel, Georgios Gakis, Katharina Müller, Benjamin-Leon Traub, Florian Schoeberl, Maria Martinez-Lage, M Subklewe, P Harter, Louisa Daniela Von Baumgarten, Jorg Dietrich, Martin Dreyling, Martina Rudelius, Oliver Weigert, Philipp Karschnia
article en

Abstract

Although PCNSTL is exceptionally rare, we identified distinct neuroimaging patterns showing highly aggressive features on MRI but hypometabolic PET imaging, which may assist in identifying future PCNSTL cases. Despite the limited cohort size, our findings suggest that MTX-based chemotherapy with ASCT may translate into favorable outcome. We report on an ALK1-positive PCNSTL case with sustained complete remission after targeted therapy with lorlatinib.

NeurologyVol. 106(7)
Harvard University (US), German Cancer Research Center (DE), Friedrich-Alexander-Universität Erlangen-Nürnberg (DE), Heidelberg University (DE), University Hospital Heidelberg (DE), LMU Klinikum (DE), Humboldt-Universität zu Berlin (DE), Massachusetts General Hospital (US), German Cancer Society (DE), German Centre for Cardiovascular Research (DE), Ludwig-Maximilians-Universität München (DE)
Zero hunger
Openalex Percentile: Top 27%
CNS Lymphoma Diagnosis and Treatment
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