Clinical Manifestations of Primary CNS T-Cell Lymphoma
Year: 2026
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND OBJECTIVES: Primary CNS T-cell lymphoma (PCNSTL) is an extremely rare and aggressive form of non-Hodgkin lymphoma. Diagnosis is often challenging because of the nonspecific clinical presentation, which can lead to delays in treatment. This study aims to analyze the clinical, histopathologic, and neuroimaging characteristics of PCNSTL. METHODS: In this retrospective, multicentric cohort study, histologically confirmed PCNSTL cases without evidence of systemic disease were selected from pathology databases at 3 academic neuro-oncology centers (University Hospital of Munich, University Hospital of Heidelberg, Massachusetts General Hospital in Boston) during the period from 2008 to 2024. Retrospective data, including demographics, histopathology, immunophenotyping, multimodal MRI, and PET imaging, treatment lines, and outcome data were extracted from medical records and analyzed. RESULTS: < 0.0275). In 1 case of ALK1-positive PCNSTL, persistent complete remission was achieved after treatment with the ALK-inhibitor lorlatinib. DISCUSSION: 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.
Source: Neurology
Authors: Katharina Johanna Schmidbauer, Philipp Karschnia, Leon D. Kaulen, Jonas Reis, Georgios Gakis, Benjamin Englert, Felix Hinz, Benjamin-Leon Traub, Felix Ehret, Elizabeth C. Conner, Maria Martinez-Lage, Veit M. Stoecklein, Martin Dreyling, Oliver Weigert, Marion Subklewe, Martina Rudelius, Nathalie L. Albert, Andreas Straube, Robert Forbrig, Florian Schoeberl, Florian Ringel, Patrick N. Harter, Jorg Dietrich, Louisa Daniela Von Baumgarten
Institutions: Ludwig-Maximilians-Universität München, Friedrich-Alexander-Universität Erlangen-Nürnberg, Universitätsklinikum Erlangen, German Cancer Research Center, University Hospital Heidelberg, Deutsches Konsortium für Translationale Krebsforschung, LMU Klinikum, Institut für Diagnostische und Interventionelle Neuroradiologie, Heidelberg University, Institut für Neuropathologie, Harvard University, Humboldt-Universität zu Berlin, Massachusetts General Hospital, Charité - Universitätsmedizin Berlin, Pathologisches Institut
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W7138238764