Primary lymphoma of the central nervous system

Clinical features of primary vitreoretinal lymphoma developing extra-central nervous system (CNS)/testicular lymphomas with or without CNS involvement: A single-centre study of 13 cases.

Neuronal-Glial and Immune Microenvironment Signatures Show Prognostic Effects in Primary CNS Lymphoma

A Multicenter, Real World Analysis of Primary Central Nervous System Lymphoma in Those with and without Human Immunodeficiency Virus

Primary central nervous system (CNS) lymphoma is an uncommon neoplasm. However, the incidence of primary CNS lymphoma has increased more than 10-fold over the past three decades, and continues to accelerate. Currently, primary CNS lymphoma represents 4 to 7 percent of all newly diagnosed primary CNS tumors. Primary CNS lymphoma may arise from different parts of the brain, with deep hemispheric periventricular white matter being the most common site of origin. The presenting symptoms in primary CNS lymphoma vary depending on the location of the mass. Involvement of the hypothalamic-pituitary axis may cause hypopituitarism, diabetes insipidus, headache, diplopia, and blurred vision. We experienced a case of a 58-year-old woman who developed central diabetes insipidus and panhypopituitarism secondary to primary CNS lymphoma. Hypothalamic and thalamic involvement were suspected based on brain MRI, and primary CNS lymphoma was confirmed by a CT-guided stereotactic biopsy. Through performing a water deprivation test and a combined pituitary stimulation test, we diagnosed complete type central diabetes insipidus and panhypopituitarism. Symptomatic relief was obtained with desmopressin, levothyroxine, hydrocortisone, and high-dose methotrexate-based chemotherapy. The thalamic and hypothalamic masses were significantly decreased in size after chemotherapy. We report the details of this case along with a review of the literature concerning primary CNS lymphom (J Korean Endocr Soc 23:260~265, 2008)

Is It Possible to Omit Bone Marrow Biopsy in Diagnostic Workup in Patients with Newly Diagnosed Primary CNS Lymphoma? a Retrospective Analysis in the PET/CT Era

A Phase II Trial of Nivolumab and Ibrutinib for Patients with Relapsed or Refractory Central Nervous System Lymphoma

Guidelines for the diagnosis and management of primary central nervous system diffuse large B-cell lymphoma.

A multicenter, real-world analysis of primary central nervous system lymphoma in those with and without human immunodeficiency virus.

Purpose: Primary central nervous system (CNS) lymphoma, a rare form of non-Hodgkin's lymphoma (NHL), is defined as high-grade B cell neoplasm of aggressive extranodal CNS. The incidence of CNS lymphoma has been increasing in recent years. In general, it has a poor prognosis. The aim of this study is to evaluate the clinical, pathological, immunohistochemical and radiological features of patients with primary CNS lymphoma in the light of literature. Materials and methods: Between May 2011 and January 2017, 10 patients with pathologic confirmation of primary CNS lymphoma were retrospectively reviewed. Results: Of 10 patients with CNS lymphoma, 7 (70%) were female and 3 (30%) were male. The mean age of all patients at diagnosis was 59.1±13.98 years. The most common settlements; eight patients (80%) had basal ganglia and 3 patients (30%) had multiple intracranial masses. Sterotaxic brain biopsy was performed in 8 patients (80%) and tumor resection was performed by craniotomy in 2 patients (20%). All of the patients with non-Hodgkin B cell lymphoma had diffuse large B cell lymphoma in 10 (100%). All cases had diffuse and strong positivity for CD20. None of the patients had immunodeficiency. Conclusion: Primary CNS lymphoma with a poor prognosis is frequently seen over 55 years of age. Diffuse large B-cell lymphoma is the most common subtype and basal ganglia is the most common site.

Keywords: Primary CNS lymphoma; Relapse; follow-up; magnetic resonance imaging; surveillance imaging

Using Targeted RNA Next Generation Sequencing and Differential Expression to Compare Primary Central Nervous System Lymphoma with CD+ DLBCL, Nodal DLBCL and EBV+ DLBCL

Trial in progress: Treatment of CNS lymphoma with zanubrutinib and tislelizumab: A phase ib/II investigator-initiated study

How and why lymphoma cells home to the central nervous system and vitreoretinal compartment in primary diffuse large B-cell lymphoma of the central nervous system remain unknown. Our aim was to create an in vivo model to study lymphoma cell tropism to the central nervous system. We established a patient-derived central nervous system lymphoma xenograft mouse model and characterised xenografts derived from four primary and four secondary central nervous system lymphoma patients using immunohistochemistry, flow cytometry and nucleic acid sequencing technology. In reimplantation experiments, we analysed dissemination patterns of orthotopic and heterotopic xenografts and performed RNA sequencing of different involved organs to detect differences at the transcriptome level. We found that xenografted primary central nervous system lymphoma cells home to the central nervous system and eye after intrasplenic transplantation, mimicking central nervous system and primary vitreoretinal lymphoma pathology, respectively. Transcriptomic analysis revealed distinct signatures for lymphoma cells in the brain in comparison to the spleen as well as a small overlap of commonly regulated genes in both primary and secondary central nervous system lymphoma. This in vivo tumour model preserves key features of primary and secondary central nervous system lymphoma and can be used to explore critical pathways for the central nervous system and retinal tropism with the goal to find new targets for novel therapeutic approaches.

Multiple sclerosis (MS) is an autoimmune disease that targets the myelin of the brain, spinal cord, and optic nerves. The inciting trigger that activates this immune response is unknown. Primary central nervous system (CNS) lymphoma is usually a diffuse large B-cell non-Hodgkin's lymphoma that originates in the brain, spinal cord, leptomeninges, or eyes. We report a 33-year-old patient who was diagnosed to have multiple sclerosis initially and a CNS lymphoma was noted 38 months later. Primary CNS lymphoma is a potential complication of chronic immunosuppression. Due to the uses of steroid, immunosuppresants and beta-interferon for multiple sclerosis, our patient developed an immuno-compromised state. These agents might contribute to the occurrence of a primary CNS lymphoma. On the other hand, a demyelinating disease may have preceded the diagnosis of primary CNS lymphoma. A possibility of neoplastic transformation in CNS inflammatory diseases such as multiple sclerosis may occur. The association of coexistent primary CNS lymphoma and multiple sclerosis may be more than coincidental.

Diffuse large B-cell lymphoma (DLBCL) constitutes most primary central nervous system (CNS) lymphoma (PCNSL), whereas T-cell, low-grade and Burkitt’s lymphomas (BL) are rarely encountered. Due to the paucity of cases, little is known about the clinical features and treatment outcomes of PCNSL other than DLBCL. The objective of this study was to describe the clinical characteristics and outcomes for patients with PCNSL other than DLBCL. Fifteen patients, newly diagnosed with PCNSLs other than DLBCL between 2000 and 2010, were included. The male to female ratio was 0.67:1 with a median age of diagnosis of 31 years (range 18–59). Pathologic distributions were as follows: peripheral T-cell lymphoma (PTCL; n = 7), marginal zone B-cell lymphoma (MZBCL; n = 1), lymphoplasmacytic lymphoma (LPL; n = 2), Burkitt’s lymphoma (n = 1), other unspecified (T-cell lineage, n = 2; B-cell lineage, n = 2). Thirteen patients (87%) showed Eastern Cooperative Oncology Group performance score (ECOG PS) 1–2. The remaining two were one PTCL patient and one Burkitt’s lymphoma patient. Of the nine patients with T-cell lymphoma, five (56%) had multifocal lesions, and one (20%) with LPL of the five patients with B-cell lymphoma showed a single lesion. Leptomeningeal lymphomatosis was identified in two patients (one with Burkitt’s lymphoma and one with unspecified B-cell lymphoma). Two patients (22%) with T-cell lymphoma died 7.7 and 23.3 months later, respectively, due to disease progression, despite HD-MTX-based therapy. Six patients with T-cell lymphoma (6/9, 66.7%) and four patients with low-grade B-cell lymphoma (4/5, 80%) achieved complete response and have survived without relapse (Table 3). One patient with Burkitt’s lymphoma showed poor clinical features with ECOG PS 3, deep structure, multifocal, and leptomeningeal lymphomatosis, and died 7.6 months after the initiation of treatment. In comparison with previously reported DLBCLs (median OS 6.4 years, 95% CI 3.7–9.1 years), T-cell lymphoma showed equivocal or favorable clinical outcomes and low-grade B-cell lymphomas, such as MZBCL and LPL, had a good prognosis. However, primary CNS Burkitt’s lymphoma presented poor clinical outcomes and showed a comparatively aggressive clinical course. In conclusion, primary CNS lymphoma other than DLBCL occurred more in younger patients and showed a generally good prognosis, except for Burkitt’s lymphoma. Further research on treatment strategies for Burkitt’s lymphoma is needed.