Primary Central Nervous System Lymphoma

2008 ◽  
Vol 132 (11) ◽  
pp. 1830-1834 ◽  
Author(s):  
Sharathkumar Bhagavathi ◽  
Jon D. Wilson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Molecular Mechanisms ◽  
Neuropsychiatric Symptoms ◽  
Central Nervous System Lymphoma ◽  
Low Grade ◽  
Non Hodgkin Lymphoma ◽  
Demyelinating Disorders ◽  
Immunocompetent Patients

Abstract Primary central nervous system lymphoma (PCNSL) is an uncommon extranodal non-Hodgkin lymphoma. Its incidence has increased during the last 3 decades and has been reported in both immunocompromised and immunocompetent patients. Immunocompromised patients are affected at a younger age compared with immunocompetent patients. It presents with raised intracranial pressure and focal neurologic and neuropsychiatric symptoms. The lesions are typically solitary. The majority of the lesions are located in the periventricular area, whereas in a few cases they are located in the supratentorial area. Diffuse large B-cell lymphomas constitute most PCNSLs, whereas T-cell, low-grade, anaplastic, and Hodgkin lymphomas are rarely encountered. The morphology of PCNSL shows a characteristic angiocentric pattern and is positive for B-cell markers by immunohistochemistry. The differential diagnosis of PCNSL includes central nervous system gliomas, metastatic tumors, demyelinating disorders, subacute infarcts, and space-occupying lesions due to an infectious etiology. The understanding of the molecular mechanisms involved in the pathogenesis of PCNSL and the identification of molecular biomarkers have lagged behind that of systemic nodal lymphomas. Primary central nervous system lymphomas are treated with combined radiotherapies and chemotherapies. The prognosis for PCNSL is worse than for other extranodal lymphomas.

Low-grade primary central nervous system lymphoma in immunocompetent patients

2005 ◽  
Vol 128 (5) ◽  
pp. 616-624 ◽  
Author(s):  
Kristoph Jahnke ◽  
Eckhard Thiel ◽  
Andreas Schilling ◽  
Ulrich Herrlinger ◽  
Michael Weller ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Lymphoma ◽  
Low Grade ◽  
Immunocompetent Patients

A rare type primary central nervous system lymphoma with primarily psychiatric diagnosis- a case report

2017 ◽  
Vol 41 (S1) ◽  
pp. S489-S490
Author(s):  
U. Cikrikcili ◽  
B. Saydam ◽  
M. Aktan
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Neuropsychiatric Symptoms ◽  
Clinical Manifestations ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Histopathological Diagnosis ◽  
High Grade

Primary central nervous system lymphoma (PCNSL) is a high-grade malignant B-cell non-Hodgkin neoplasm that is an infrequent variant of all intracranial neoplasms (1%) and all lymphomas (< 1%)PCNSL is documented mainly in immunocompromised patient groups, although it may also be diagnosed in immunocompetent patients. It affects mainly the eyes, supratentorial areas, or the spinal cord. The lesions are typically localized in frontal lobes, corpus callosum and basal ganglia. Additionally, lesions might rarely be detected at infratentorial areas and in medulla spinalis. Even though a wide spectrum of treatment options are available, such as chemotherapy, radiotherapy, or surgery; response rates are low and prognosis is poor in spite of appropriate treatment.The case we reported here is 57-year-old male presented with symptoms of aggresivity, impulsivity, depressive mood and personality changes. Histopathological diagnosis was CD5 positive diffuse large B cell lymphoma, which is very rare in high-grade lymphomas. There were no neurological signs related to CNS tumor and the clinical manifestations responded very well to chemotherapy consisting of high dose methotrexate, vincristine and procarbazine. The significance of such neuropsychiatric symptoms in the course of treatment for PCNSL has been previously documented as well. These behavioral and emotional symptoms might manifest themselves based on where the neoplasm is localized. Therefore, psychiatrists should be more aware of the uncommon manifestation of the disorder as reported in this case. Consultation for differential diagnosis might also be necessary in such cases.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals A genome-wide association study identifies susceptibility loci for primary central nervous system lymphoma at 6p25.3 and 3p22.1: a LOC Network study

2019 ◽  
Vol 21 (8) ◽  
pp. 1039-1048 ◽  
Author(s):  
Karim Labreche ◽  
Mailys Daniau ◽  
Amit Sud ◽  
Philip J Law ◽  
Louis Royer-Perron ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Association Study ◽  
Genome Wide Association Study ◽  
Central Nervous System Lymphoma ◽  
Genome Wide Association ◽  
Non Hodgkin Lymphoma ◽  
Genome Wide ◽  
A Genome

AbstractBackgroundPrimary central nervous system lymphoma (PCNSL) is a rare form of extra-nodal non-Hodgkin lymphoma. PCNSL is a distinct subtype of non-Hodgkin lymphoma, with over 95% of tumors belonging to the diffuse large B-cell lymphoma (DLBCL) group. We have conducted a genome-wide association study (GWAS) on immunocompetent patients to address the possibility that common genetic variants influence the risk of developing PCNSL.MethodsWe performed a meta-analysis of 2 new GWASs of PCNSL totaling 475 cases and 1134 controls of European ancestry. To increase genomic resolution, we imputed >10 million single nucleotide polymorphisms using the 1000 Genomes Project combined with UK10K as reference. In addition we performed a transcription factor binding disruption analysis and investigated the patterns of local chromatin by Capture Hi-C data.ResultsWe identified independent risk loci at 3p22.1 (rs41289586, ANO10, P = 2.17 × 10−8) and 6p25.3 near EXOC2 (rs116446171, P = 1.95 x 10−13). In contrast, the lack of an association between rs41289586 and DLBCL suggests distinct germline predisposition to PCNSL and DLBCL. We found looping chromatin interactions between noncoding regions at 6p25.3 (rs11646171) with the IRF4 promoter and at 8q24.21 (rs13254990) with the MYC promoter, both genes with strong relevance to B-cell tumorigenesis.ConclusionTo our knowledge this is the first study providing insight into the genetic predisposition to PCNSL. Our findings represent an important step in defining the contribution of common genetic variation to the risk of developing PCNSL.

INNV-25. RITUXIMAB MONOTHERAPY FOR TREATMENT OF RARE LOW GRADE PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA; A CASE REPORT AND LITERATURE REVIEW

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi110-vi110
Author(s):  
Grace Tobin ◽  
Elizabeth Neil
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Low Grade ◽  
Thalamic Lesion ◽  
Cns Involvement

Abstract Primary central nervous system lymphoma (PCNSL) is a specific variant of non-Hodgkin's lymphoma confined to the brain, leptomeninges, spinal cord, and/or eyes. The majority of PCNSL is diffuse large B-cell lymphoma (DLBCL), however, a small percentage are categorized as low-grade lymphomas (LGL). Compared to high-grade and aggressive DLBCL, LGL are indolent; allowing for targeted and less neurotoxic first-line treatments. There is currently no consensus for LGL treatment. A patient at our institution was diagnosed with LGL without extra-CNS involvement 11/2018 and then, successfully treated with rituximab monotherapy. This 65 year-old, immunocompetent woman presented with one month of right leg weakness and numbness. Brain MRI demonstrated a subtly enhancing infiltrative left thalamic lesion extending into the left frontal lobe with surrounding edema. Flow cytometry on spinal fluid showed rare monotypic B-cells. Brain biopsy results showed an atypical predominantly perivascular lymphoid infiltrate. These atypical small lymphoid cells had mature-appearing nuclear chromatin, absent nucleoli, and uniformly expressed CD79a and CD20 with variable PAX-5 expression, lacked CD56 and CD117 expression, and had plasmacytic differentiation; thus consistent with marginal zone lymphoma, a type of indolent B-cell lymphoma. There was no evidence of extra-CNS involvement on PET scan, bone marrow biopsy, or ocular exam. She had normal cognitive functioning on neuropsychological testing. Interestingly, SPEP showed a monoclonal IgM immunoglobulin of kappa-light chain-type that became a monoclonal protein in the gamma fraction. Additionally, CT abdomen initially showed splenomegaly that resolved on repeat imaging a year later. Radiation therapy was deferred due to high risk. Weekly rituximab 500mg/m2 was initiated for 4 doses, then monthly for 4 doses. Therapy was well tolerated and she noted clinical improvement plus there was positive response on brain imaging. Repeat ophthalmology exam and CT body without any evidence of cancer. She is now 29 months progression-free since completing rituximab.

Radiological morphology of low-grade primary central nervous system lymphoma in immunocompetent patients

2005 ◽  
Vol 23 (16_suppl) ◽  
pp. 1536-1536
Author(s):  
E. Thiel ◽  
K. Jahnke ◽  
A. Schilling ◽  
L. Fischer ◽  
J. Heidenreich ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Lymphoma ◽  
Low Grade ◽  
Immunocompetent Patients

Targeting the PI3K/AKT/mTOR Signaling Pathway in Primary Central Nervous System Lymphoma: Current Status and Future Prospects

2020 ◽  
Vol 19 (3) ◽  
pp. 165-173
Author(s):  
Xiaowei Zhang ◽  
Yuanbo Liu
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Signaling Pathway ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Mtor Signaling ◽  
Mtor Signaling Pathway ◽  
Antitumor Effects ◽  
Term Survival

Primary Central Nervous System Lymphoma (PCNSL) is a rare invasive extranodal non- Hodgkin lymphoma, a vast majority of which is Diffuse Large B-Cell Lymphoma (DLBCL). Although high-dose methotrexate-based immunochemotherapy achieves a high remission rate, the risk of relapse and related death remains a crucial obstruction to long-term survival. Novel agents for the treatment of lymphatic malignancies have significantly broadened the horizons of therapeutic options for PCNSL. The PI3K/AKT/mTOR signaling pathway is one of the most important pathways for Bcell malignancy growth and survival. Novel therapies that target key components of this pathway have shown antitumor effects in many B-cell malignancies, including DLBCL. This review will discuss the aberrant status of the PI3K/AKT/mTOR signaling pathways in PCNSL and the application prospects of inhibitors in hopes of providing alternative clinical therapeutic strategies and improving prognosis.

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