ArticleThe American journal of surgical pathology2015
Primary CNS T-cell Lymphomas: A Clinical, Morphologic, Immunophenotypic, and Molecular Analysis.
Article in The American journal of surgical pathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
What it found
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Who cites it
36 citing papers in PubMed, 68 citations in OpenAlex.
- Primary central nervous system T cell lymphoma involving multiple brain and spinal cord lesions with SETD2 mutation.International journal of hematology · 2026Article
- Advances in molecular pathobiology of PCNSL - towards clinical implementation of novel diagnostics and therapeutics.Biomarker research · 2026Review
- A 13-Year Review of Non-Diffuse Large B Cell Lymphomas of the Central Nervous System at a Tertiary Hospital: A Case Series Study.Neuropathology : official journal of the Japanese Society of Neuropathology · 2026Article
- Case Report: Cerebellar ALK+ ALCL: diagnostic challenges and therapeutic innovation.Frontiers in oncology · 2026Article
- Article
- Successful Treatment of Primary Central Nervous System T-Cell Lymphoma With Induction Chemotherapy Followed by Consolidation With High-Dose Chemotherapy and Autologous Stem Cell Rescue.Journal of medical cases · 2025Article
- Case Report and review of the literature: A rare cut - surgical resection of primary CNS EBV- positive T-cell lymphoma.Frontiers in oncology · 2025Article
- Flow cytometry identifies changes in peripheral and intrathecal lymphocyte patterns in CNS autoimmune disorders and primary CNS malignancies.Journal of neuroinflammation · 2024Article
- Multiple intra-axial lesions in a 57-year-old male with a history of B-cell chronic lymphocytic leukemia.Brain pathology (Zurich, Switzerland) · 2024Article
- Article
- Absence of enhancement in a lesion does not preclude primary central nervous system T-cell lymphoma: A case report.World journal of clinical cases · 2024Article
- Primary CNS ALK-negative anaplastic large cell lymphoma: A case report and review of the literature.Radiology case reports · 2024Article
- Imaging of Lymphomas Involving the CNS: An Update-Review of the Full Spectrum of Disease with an Emphasis on the World Health Organization Classifications of CNS Tumors 2021 and Hematolymphoid Tumors 2022.AJNR. American journal of neuroradiology · 2023Review
- Rare central nervous system lymphomas.British journal of haematology · 2022Review
- Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement.Brain tumor pathology · 2022Article
- Peripheral T-Cell Lymphomas Involving the Central Nervous System: A Report From the Czech Lymphoma Study Group Registry.Frontiers in oncology · 2022Article
- Case Report: Primary Indolent Epstein-Barr Virus-Positive T-Cell Lymphoproliferative Disease Involving the Central Nervous System.Frontiers in surgery · 2022Article
- Primary Central Nervous System T-cell Lymphoma as Methotrexate-associated Lymphoproliferative Disorders: Case Report.NMC case report journal · 2021Article
- Primary peripheral T-cell central nervous system lymphoma.Surgical neurology international · 2021Article
- Primary Central Nervous System Involvement in Peripheral T-Cell Lymphoma: A Case Report.Taehan Yongsang Uihakhoe chi · 2021Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 1 country.
Funding
Abstract
Primary central nervous system (CNS) lymphomas are relatively rare with the most common subtype being diffuse large B-cell lymphoma. Primary CNS T-cell lymphomas (PCNSTL) account for <5% of CNS lymphomas. We report the clinical, morphologic, immunophenotypic, and molecular characteristics of 18 PCNSTLs. Fifteen cases were classified as peripheral T-cell lymphoma, not otherwise specified, 2 of which were of γδ T-cell derivation and 1 was TCR silent; there was 1 anaplastic large cell lymphoma, ALK-positive and 2 anaplastic large cell lymphoma, ALK-negative. Median age was 58.5 years (range, 21 to 81 y), with an M:F ratio of 11:7. Imaging results showed that 15 patients had supratentorial lesions. Regardless of subtype, necrosis and perivascular cuffing of tumor cells were frequently observed (11/18 cases). CD3 was positive in all cases but 1; 10/17 were CD8-positive, and 5/17 were CD4-positive. Most cases studied had a cytotoxic phenotype with expression of TIA1 (13/15) and granzyme-B (9/13). Polymerase chain reaction analysis of T-cell receptor γ rearrangement confirmed a T-cell clone in 14 cases with adequate DNA quality. Next-generation sequencing showed somatic mutations in 36% of cases studied; 2 had >1 mutation, and none showed overlapping mutations. These included mutations in DNMT3A, KRAS, JAK3, STAT3, STAT5B, GNB1, and TET2 genes, genes implicated previously in other T-cell neoplasms. The outcome was heterogenous; 2 patients are alive without disease, 4 are alive with disease, and 6 died of disease. In conclusion, PCNSTLs are histologically and genomically heterogenous with frequent phenotypic aberrancy and a cytotoxic phenotype in most cases.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.