{
  "abstract": "Background Primary central nervous system T-cell lymphoma (PCNSTL) is an exceptionally rare central nervous system lymphoma with limited clinical data. We present a large case review series of PCNSTL to summarise the clinical characteristics of this disease.Methods This study integrated 4 new cases of PCNSTL from our centre with 132 previously reported cases identified through a systematic search of PubMed, Cochrane Library and Web of Science databases.Results A total of 136 PCNSTL cases were identified, with a median age of 41 years (range 2–89 years), and a male-to-female ratio of 1.8:1. Peripheral T-cell lymphoma-not otherwise specified was the most prevalent pathological subtype. Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALK+ALCL) predominated in men relative to the various subtypes. The cerebral hemispheres are the most frequent anatomical region involved (71.3%), followed by cerebellum (16.2%), basal ganglia (14.7%), brainstem (14.7%), meninges (11.8%) and corpus callosum (2.9%). Meningeal involvement was observed in nearly 50% of ALCL cases. Histopathologically, 31.8% of tumour cells (29/91) were small cell-dominated, 79.2% of the cases presented angiocentric growth pattern (57/72), and half of cases had a positive finding of cerebrospinal fluid (CSF) flow cytometry (7/14).Conclusions PCNSTL has a male predilection, particularly in ALK+ALCL. PCNSTL shows reduced basal ganglia and corpus callosum involvement relative to primary central nervous system B-cell lymphoma, but displays heightened meningeal tropism, especially in ALCL cases. Histopathological examination typically reveals prominent perivascular lymphocytic cuffing in PCNSTL. CSF flow cytometry could be considered a preferred method for a definite diagnosis of PCNSTL when brain biopsy is not possible.",
  "authors": [
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Yi Chen"
    },
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Xiaoyang Lei"
    },
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Shipeng Guo"
    },
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Yuan Shi"
    },
    {
      "affiliations": [
        "Emergency Department, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Guofeng Wu"
    },
    {
      "affiliations": [
        "Emergency Department, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Likun Wang"
    },
    {
      "affiliations": [
        "Department of Pathology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Po Li"
    },
    {
      "affiliations": [
        "Department of Radiology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Hui Yu"
    },
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Zhu Xu"
    },
    {
      "affiliations": [
        "Department of Neurology, The Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China"
      ],
      "name": "Dian He"
    }
  ],
  "title": "Primary central nervous system T-cell lymphoma: a single-centre retrospective study and literature review",
  "uid": "ef551b72-acb1-52da-80e5-5dfb31f6aa58"
}