scholarly journals Primary Central Nervous System Lymphomas: A Diagnostic Overview of Key Histomorphologic, Immunophenotypic, and Genetic Features

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1076
Author(s):  
Marietya I. S. Lauw ◽  
Calixto-Hope G. Lucas ◽  
Robert S. Ohgami ◽  
Kwun Wah Wen
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Molecular Genetic ◽  
Central Nervous System Lymphoma ◽  
Great Majority ◽  
B Cell Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Genetic Features ◽  
The Central Nervous System ◽  
The Brain

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that primarily arises in the brain, spinal cord, leptomeninges, and vitreoretinal compartment of the eye. The term is sometimes used interchangeably with primary central nervous system diffuse large B-cell lymphoma (PCNS DLBCL) because DLBCL comprises a great majority (90–95%) of PCNSL. Although rare, other types of lymphomas can be seen in the central nervous system (CNS), and familiarity with these entities will help their recognition and further workup in order to establish the diagnosis. The latter is especially important in the case of PCNSL where procurement of diagnostic specimen is often challenging and yields scant tissue. In this review, we will discuss the most common types of primary lymphomas that can be seen in the CNS with emphasis on the diagnostic histomorphologic, immunophenotypic, and molecular genetic features. The differential diagnostic approach to these cases and potential pitfalls will also be discussed.

scholarly journals Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuchen Wu ◽  
Xuefei Sun ◽  
Xueyan Bai ◽  
Jun Qian ◽  
Hong Zhu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

scholarly journals Primary Central Nervous System Lymphoma in an Immunocompetent Patient Presenting as Multiple Cerebellar Lesions: A Case Report and Review of Literature

2019 ◽  
Vol 7 ◽  
pp. 232470961989354
Author(s):  
Gliceida M. Galarza Fortuna ◽  
Kathrin Dvir ◽  
Christopher Febres-Aldana ◽  
Michael Schwartz ◽  
Ana Maria Medina
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Lesion ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Immunocompetent Patient ◽  
Cns Lymphoma ◽  
High Dose ◽  
Older Woman ◽  
Non Hodgkin Lymphoma

Primary central nervous system (CNS) lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma often presenting as a single brain lesion within the CNS. On histopathological evaluation of PCNSL a positive CD10, which is frequently observed in systemic diffuse large B-cell lymphoma, is present in approximately 10% of PCNSL. We describe a case of CD10-positive PCNSL presenting with multiple posterior fossa enhancing lesions in an immunocompetent older woman with a history of breast cancer successfully treated by the RTOG 0227 protocol consisting of pre-irradiation chemotherapy with high-dose methotrexate, rituximab, and temozolomide for 6 cycles, followed by low-dose whole-brain radiation and post-irradiation temozolomide.

Identification of microRNAs in the cerebrospinal fluid as marker for primary diffuse large B-cell lymphoma of the central nervous system

Blood ◽  
2011 ◽  
Vol 117 (11) ◽  
pp. 3140-3146 ◽  
Author(s):  
Alexander Baraniskin ◽  
Jan Kuhnhenn ◽  
Uwe Schlegel ◽  
Andrew Chan ◽  
Martina Deckert ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Diagnostic Accuracy ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Diagnostic Value ◽  
Rna Molecules ◽  
Noninvasive Biomarker ◽  
The Central Nervous System

Abstract The diagnosis of primary central nervous system lymphoma (PCNSL) depends on histopathology of brain biopsies, because disease markers in the cerebrospinal fluid (CSF) with sufficient diagnostic accuracy are not available yet. MicroRNAs (miRNAs) are regulatory RNA molecules that are deregulated in many disease types, including cancer. Recently, miRNAs have shown promise as markers for cancer diagnosis. In this study, we demonstrate that miRNAs are present in the CSF of patients with PCNSL. With a candidate approach and miRNA quantification by reverse transcription polymerase chain reaction, miRNAs with significant levels in the CSF of patients with PCNSL were identified. MiR-21, miR-19, and miR-92a levels in CSF collected from patients with PCNSL and from controls with inflammatory CNS disorders and other neurologic disorders indicated a significant diagnostic value of this method. Receiver-operating characteristic analyses showed area under the curves of 0.94, 0.98, and 0.97, respectively, for miR-21, miR-19, and miR-92a CSF levels in discriminating PCNSL from controls. More importantly, combined miRNA analyses resulted in an increased diagnostic accuracy with 95.7% sensitivity and 96.7% specificity. We also demonstrated a remarkable stability of miRNAs in the CSF. In conclusion, CSF miRNAs are potentially useful tools as novel noninvasive biomarker for the diagnosis of PCNSL.

scholarly journals Secondary Central Nervous System Lymphoma as a Complication from a Systemic Non-Hodgkin Lymphoma

2021 ◽  
pp. 1-4
Author(s):  
Rodríguez-Leyva Ildefons ◽  
◽  
Rodríguez-Rivas Ricardo ◽  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hodgkin Lymphoma ◽  
Systemic Disease ◽  
Brain Magnetic Resonance Imaging ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Peripheral Nerve Involvement ◽  
Secondary Central Nervous System

Secondary central nervous system lymphoma is a complication from non-Hodgkin lymphoma not very well known. NHL can present in a variety of neurological clinical presentations varying from primary central nervous system lymphoma to complications from a systemic disease like metastasis or peripheral nerve involvement. We present a case of a 78-year-old male with Diffuse large B-cell lymphoma, treated with Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone. Six months later of the diagnosis, the patient presented to the emergency department, complaining of five days with progressive weakness in the left upper limb, and two days before the weakness developed in the left lower limb. On the neurologic examination with inattention, dysarthria, left central facial nerve palsy, severe weakness on the left hemibody with hyperreflexia, and left extensor plantar response. Brain Magnetic resonance imaging reported three metastatic lesions, with a final diagnosis of secondary central nervous system lymphoma.

scholarly journals Ibrutinib in Refractory or Relapsing Primary Central Nervous System Lymphoma: A Systematic Review

2022 ◽  
Vol 14 (1) ◽  
pp. 99-108
Author(s):  
Gaurav Nepal ◽  
Mahika Khurana ◽  
Domenica Herrera Bucheli ◽  
Siddhartha Bhandari ◽  
Utsav Joshi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Central Nervous System ◽  
Nervous System ◽  
Significant Proportion ◽  
Central Nervous System Lymphoma ◽  
The Novel ◽  
Non Hodgkin Lymphoma ◽  
Frequent Relapses ◽  
Btk Inhibitor ◽  
The Brain

Primary Central Nervous System Lymphoma (PCNSL) is a rare variant of Non-Hodgkin Lymphoma (NHL) representing 1–2% of all NHL cases. PCNSL is defined as a lymphoma that occurs in the brain, spinal cord, leptomeninges, or eyes. Efforts to treat PCNSL by traditional chemotherapy and radiotherapy have generally been unsuccessful as a significant proportion of patients have frequent relapses or are refractory to treatment. The prognosis of patients with Refractory or Relapsed (R/R) PCNSL is abysmal. The optimal treatment for R/R PCNSL is poorly defined as there are only a limited number of studies in this setting. Several studies have recently shown that ibrutinib, a Bruton tyrosine kinase (BTK) inhibitor, has promising results in the treatment of R/R PCNSL. However, these are preliminary studies with a limited sample size. In this systematic review, we explored and critically appraised the evidence about the efficacy of the novel agent ibrutinib in treating R/R PCNSL.

scholarly journals A Rare Case of Spontaneous Remission and Relapse of a Primary Central Nervous System Lymphoma

2018 ◽  
Vol 31 (12) ◽  
pp. 777 ◽  
Author(s):  
Rui Ramos ◽  
João Soares Fernandes ◽  
Marta Almeida ◽  
Rui Almeida
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Case ◽  
Central Nervous System Lymphoma ◽  
Brain Biopsy ◽  
B Cell Lymphoma ◽  
Spontaneous Remission ◽  
Neurological Deficits ◽  
Pubmed Search ◽  
The Central Nervous System

Primary central nervous system lymphoma remission after steroid treatment is a well-known phenomenon, but remission without any type of treatment is extremely rare. We present a rare case of spontaneous remission of a diffuse large B-cell lymphoma of the central nervous system as well as its subsequent reappearance in another location. The atypical presentation misled the neurosurgeons and neurologists, delaying diagnosis and treatment. The patient underwent brain biopsy after the relapse and started radiotherapy and chemotherapy with cytarabine + methotrexate + rituximab. As of 32 months after the diagnosis, the patient remained asymptomatic, with no focal neurological deficits and the disease in complete remission. A PubMed search of the literature up to June 2017 regarding spontaneous remission central nervous system lymphoma was also carried out.

Nonglial Central Nervous System Tumors

2021 ◽  
pp. 931-948
Author(s):  
Akanksha Sharma ◽  
Alyx B. Porter
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Highly Active Antiretroviral Therapy ◽  
Central Nervous System Lymphoma ◽  
Non Hodgkin Lymphoma ◽  
Highly Active ◽  
Acquired Immunodeficiency ◽  
Immunodeficiency Syndrome ◽  
Adjusted Incidence ◽  
The Brain

Primary central nervous system lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin lymphoma that may involve the brain, leptomeninges, eyes, or spinal cord and accounts for up to 5% of all adult primary brain neoplasms. Age-adjusted incidence has increased in the past 3 decades. Infection with HIV and acquired immunodeficiency syndrome increase the risk of PCNSL by 3,600-fold. However, with highly active antiretroviral therapy, the frequency of immune system compromise sufficient for HIV-associated PCNSL (CD4 count ≤50) is dramatically reduced, along with the risk of this disorder.

scholarly journals Rare pathological variants and presentations of primary central nervous system lymphomas

2006 ◽  
Vol 21 (5) ◽  
pp. 1-6 ◽  
Author(s):  
Arnaldo Neves Da Silva ◽  
Maria Beatriz Lopes ◽  
David Schiff
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Brain Parenchyma ◽  
Low Grade ◽  
Rare Form ◽  
Large B Cell Lymphoma ◽  
The Brain

✓ Primary central nervous system lymphoma (PCNSL) is a rare form of primary brain neoplasm, accounting for less than 3% of all primary brain tumors. Ninety percent of cases involve a large B-cell lymphoma that presents as a homogeneously enhancing lesion or lesions, typically deep-seated in the brain parenchyma. The authors describe unusual pathological forms of PCNSLs, including low-grade, T-cell, and Burkitt types, and also rare presentations such as neurolymphomatosis and pituitary lymphomas.

Brain parenchyma involvement as isolated central nervous system relapse of systemic non-Hodgkin lymphoma: an International Primary CNS Lymphoma Collaborative Group report

Blood ◽  
2008 ◽  
Vol 111 (3) ◽  
pp. 1085-1093 ◽  
Author(s):  
Nancy D. Doolittle ◽  
Lauren E. Abrey ◽  
Tamara N. Shenkier ◽  
Siegal Tali ◽  
Jacoline E.C. Bromberg ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Confidence Interval ◽  
Hodgkin Lymphoma ◽  
Median Time ◽  
B Cell Lymphoma ◽  
Brain Parenchyma ◽  
Non Hodgkin Lymphoma ◽  
Cns Relapse ◽  
The Brain

Abstract Isolated central nervous system (CNS) relapse involving the brain parenchyma is a rare complication of systemic non-Hodgkin lymphoma. We retrospectively analyzed patient characteristics, management, and outcomes of this complication. After complete response to initial non-Hodgkin lymphoma treatment, patients with isolated CNS relapse with the brain parenchyma as initial relapse site were eligible. Patients with isolated CNS relapse involving only the cerebrospinal fluid were not eligible. Information on 113 patients was assembled from 13 investigators; 94 (83%) had diffuse large B-cell lymphoma. Median time to brain relapse was 1.8 years (range, 0.25-15.9 years). Brain relapse was identified by neuroimaging in all patients; in 54 (48%), diagnostic brain tumor specimen was obtained. Median overall survival from date of brain relapse was 1.6 years (95% confidence interval, 0.9-2.6 years); 26 (23%) have survived 3 years or more. Median time to progression was 1.0 year (95% confidence interval, 0.7-1.7 years). Age less than 60 years (P = .006) at relapse and methotrexate use (P = .008) as front-line treatment for brain relapse were significantly associated with longer survival in a multivariate model. Our results suggest systemic methotrexate is the optimal treatment for isolated CNS relapse involving the brain parenchyma. Long-term survival is possible in some patients.

scholarly journals Tumor and Cerebrospinal Fluid microRNAs in Primary Central Nervous System Lymphomas

Cancers ◽  
2019 ◽  
Vol 11 (11) ◽  
pp. 1647
Author(s):  
Michalina Zajdel ◽  
Grzegorz Rymkiewicz ◽  
Maria Sromek ◽  
Maria Cieslikowska ◽  
Pawel Swoboda ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Expression Profiles ◽  
Central Nervous System Lymphoma ◽  
Brain Biopsy ◽  
B Cell Lymphoma ◽  
Brain Lesions ◽  
Non Hodgkin Lymphoma ◽  
Precise Diagnosis

Primary central nervous system lymphoma (PCNSL) is a rare, highly aggressive, extranodal form of non-Hodgkin lymphoma, predominantly diagnosed as primary diffuse large B-cell lymphoma of the central nervous system (CNS DLBCL). Fast and precise diagnosis of PCNSL is critical yet challenging. microRNAs, important regulators in physiology and pathology are potential biomarkers. In 131 patients with CNS DLBCL and with non-malignant brain lesions (n-ML), miR-21, miR-19b and miR-92a, miR-155, miR-196b, miR-let-7b, miR-125b, and miR-9 were examined by RT-qPCR in brain biopsy samples (formalin-fixed paraffin-embedded tissues, FFPET; CNS DLBCL, n = 52; n-ML, n = 42) and cerebrospinal fluid samples (CSF; CNS DLBCL, n = 30; n-ML, n = 23) taken for routine diagnosis. FFPET samples were split into study and validation sets. Significantly higher CSF levels of miR-21, miR-19b, and miR-92a were identified in PCNSL but not in n-ML, and differentiated PCNSL from n-ML with 63.33% sensitivity and 80.77% specificity. In FFPETs, miR-155 and miR-196b were significantly overexpressed and miR-let-7b, miR-125b, and miR-9 were downregulated in PCNSL as compared to n-ML. Combined miR-155 and miR-let-7b expression levels in FFPETs discriminated PCNSL and n-ML with a 97% accuracy. In conclusion, tissue miR-155, miR-196b, miR-9, miR-125b, and miR-let-7b expression profiles differentiate PCNSL from n-ML. PCNSL CSFs and the relevant biopsy samples are characterized by specific, different microRNA profiles. A logistic regression model is proposed to discriminate between PCNSL and non-malignant brain lesions. None of the examined microRNAs influenced overall survival of PCNSL patients. Further ongoing developments involve next generation sequencing-based profiling of biopsy and CSF samples.

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