scholarly journals Invasive and Multiple Lesions of Primary Central Nervous System Lymphoma: A Case Report

2021 ◽  
Author(s):  
Juanying Zhen ◽  
Minyan Zeng ◽  
Xiaodan Zheng ◽  
Hongyan Qiu ◽  
Jun Wu
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Magnetic Resonance ◽  
Histopathological Examination ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Cerebellar Hemisphere ◽  
Resonance Spectroscopy ◽  
Multiple Lesions

Abstract Background: Primary central nervous system lymphoma (PCNSL) is rare, malignant, non-Hodgkin lymphoma in the central nervous system. Solitary lesions occurred in the majority of cases. Invasive and multiple lesions were rarely reported. As clinical presentations and neuroimage are variable, early diagnosis is challenging. Case presentation: The patient was a 61-year-old male with normal immunity who firstly experienced drowsiness and dizziness. Cranial magnetic resonance imaging(MRI) revealed invasive and multiple lesions of cerebellar hemisphere, right pons, bilateral thalamus and right frontal lobe with slightly long T1 and T2 signal. Contrast-enhanced MRI demonstrated enhanced signal shadows in the cerebellar hemisphere rather than other areas. Magnetic resonance spectroscopy (MRS) showed lactate (Lac) peak increases and N-acetyl-aspartate (NAA) peak decreases moderately. Histopathological examination revealed infiltrative growth of abnormal lymphoid cells. The immunohistochemical analysis detected the result of CD10(-), Bcl-6(+), MUM-1(+), accessing the diagnosis of diffuse large B-cell lymphoma (Non-GCB).Conclusions: PCNSL with invasive lesions distributed in supratentorial and infratentorial sites were rarely reported, resulting in the delayed diagnosis in this case. This case not only expands the clinical spectrum but also extends the distribution of PCNSL.

scholarly journals A case of primary central nervous system lymphoma mimic neuromyelitis optica

2020 ◽  
Vol 11 (1) ◽  
pp. 28-33
Author(s):  
Xixi Sheng ◽  
Mingwei Xu ◽  
Xia Li
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Magnetic Resonance ◽  
Neuromyelitis Optica ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Clinical Findings ◽  
Resonance Spectroscopy ◽  
Steroid Pulse ◽  
Positron Emission

AbstractPrimary central nervous system lymphoma (PCNSL) is rare. And the symptoms of PCNSL are atypical, it is extremely easy to be misdiagnosed as other diseases. However, early treatment is crucial which is requesting early diagnosis. We report a case of a 47-year-old man who was initially diagnosed as neuromyelitis optica (NMO) on the basis of clinical findings, slightly high Aquaporin4 (AQP4) (1:10) and high signals of magnetic resonance imaging. Though his symptoms progressively improved after steroid pulse treatment, but worse when steroid was decreased to 40 mg per day. We considered the patient should be diagnosed as PCNSL. After the examination of magnetic resonance spectroscopy (MRS) and positron emission tomography (PET), the results indicated PCNSL was most possible. Therefore we gave him stereotactic biopsy of deep of supratentorial, which showed non-Hodgkin malignant B-cell lymphoma.

scholarly journals Histopathological variation in the demyelinating sentinel lesion of primary central nervous system lymphoma

2020 ◽  
Vol 11 ◽  
pp. 342
Author(s):  
Madan Bajagain ◽  
Tatsuki Oyoshi ◽  
Tomoko Hanada ◽  
Nayuta Higa ◽  
Tsubasa Hiraki ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Histological Finding ◽  
Central Nervous System Lymphoma ◽  
White Matter Lesions ◽  
Occipital Lobe ◽  
B Cell Lymphoma ◽  
Inflammatory Cells ◽  
Lymphoid Cells ◽  
Post Contrast

Background: Primary central nervous system lymphoma (PCNSL) is one of the least common malignant brain tumors. It is usually diagnosed initially as diffuse large B cell lymphoma (DLBCL). In rare cases, however, a demyelinating lesion referred to as a “sentinel lesion” precedes the actual diagnosis, which usually depicts two distinct patterns of inflammatory cells during histological analysis. This case report describes a unique histological finding and describes the recognized variations in sentinel lesion histopathology. Case Description: A 78-year-old female patient was found to have multiple white matter lesions of various degrees of enhancement on post-contrast T1-weighted magnetic resonance imaging. A stereotactic biopsy of a heterogeneous lesion in the left occipital lobe was performed, which revealed demyelination along with lymphocytic infiltration, reactive astrocytosis, abundant T cells, and foamy macrophages. There was no evidence of monoclonality, rapid regression of all lesions occurred, and the patient was thus treated for tumefactive demyelination. Three months later, all of the residual lesions had enlarged and were homogeneously enhancing. An endoscopic-guided biopsy of the right periventricular lesion showed diffuse atypical lymphoid cells. Conclusion: The sentinel lesion of PCNSL expresses a variable histological pattern of inflammatory cells. This case demonstrates a unique and rare picture of mixed perivascular and parenchymal infiltration of inflammatory cells, highlighting the importance of repeated biopsies and/or radiological examinations to obtain an accurate diagnosis.

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.

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.

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.

Primary Central Nervous System Lymphoma Mimicking Longitudinally Extensive Transverse Myelitis

2020 ◽  
pp. 194187442096756
Author(s):  
Prashant Anegondi Natteru ◽  
Shashank Shekhar ◽  
Lakshmi Ramachandran Nair ◽  
Hartmut Uschmann
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Oligoclonal Bands ◽  
Relative Reduction ◽  
Thalamic Lesion ◽  
The Right

Primary central nervous system lymphoma (PCNSL) is an uncommon variant of extra-nodal non-Hodgkin’s lymphoma. Three regions can be involved in PCNSL: the brain, the spine, or the vitreus and retina. Spinal PCNSL is rare. It can mimic neoplasm, infection, and inflammation. Diagnostic confirmation is by tissue biopsy, and even then, tissue corroboration may be altered by an inflammatory overlay. We report a 59-year-old woman who we saw after she had 4 weeks of ascending tetraparesis plus bowel and bladder incontinence. Upon presentation, the patient was ventilator-dependent and locked-in. She reported normal sensation through eye-blinking. Magnetic resonance imaging (MRI) brain revealed signal intensity in the bilateral corona radiata and restricted diffusion in the right thalamus, whereas, MRI cervical, and thoracic spine showed T2 prolongation in the anterior medulla and upper cervical cord, with enhancement to C2-C3, and long segment hyperintensity from T1-T9 levels, respectively, suggestive of neuromyelitis optica spectrum disorder. Cerebrospinal fluid cytomorphology and flow cytometry were inconclusive for lymphoma/leukemia, but oligoclonal bands were present. Serum aquaporin-4 (AQP-4) antibodies were negative. MR spectroscopy demonstrated NAA reduction, mild lipid lactate peak, and relative reduction of choline on the side of the lesion, favoring demyelination. She received 5-days of intravenous methylprednisolone, followed by 7 sessions of plasma exchange without clinical improvement. Stereotactic biopsy of the right thalamic lesion revealed diffuse large B-cell lymphoma. PCNSL can mimic a demyelinating process early on, as steroid treatment could disrupt B-cell lymphoma cells, thus masking the correct diagnosis.

scholarly journals T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Presenting as a Primary Central Nervous System Lymphoma

Rare Tumors ◽  
2015 ◽  
Vol 7 (4) ◽  
pp. 160-162 ◽  
Author(s):  
Pooja Advani ◽  
Jason Starr ◽  
Abhisek Swaika ◽  
Liuyan Jiang ◽  
Yushi Qiu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
T Cell ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

scholarly journals Pathway analysis of primary central nervous system lymphoma

Blood ◽  
2008 ◽  
Vol 111 (6) ◽  
pp. 3200-3210 ◽  
Author(s):  
Han W. Tun ◽  
David Personett ◽  
Karen A. Baskerville ◽  
David M. Menke ◽  
Kurt A. Jaeckle ◽  
...  
Keyword(s):  
Gene Expression ◽  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Pathway Analysis ◽  
Metastatic Cancer ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Significant Differential Expression ◽  
A Genome

Abstract Primary central nervous system (CNS) lymphoma (PCNSL) is a diffuse large B-cell lymphoma (DLBCL) confined to the CNS. A genome-wide gene expression comparison between PCNSL and non-CNS DLBCL was performed, the latter consisting of both nodal and extranodal DLBCL (nDLBCL and enDLBCL), to identify a “CNS signature.” Pathway analysis with the program SigPathway revealed that PCNSL is characterized notably by significant differential expression of multiple extracellular matrix (ECM) and adhesion-related pathways. The most significantly up-regulated gene is the ECM-related osteopontin (SPP1). Expression at the protein level of ECM-related SPP1 and CHI3L1 in PCNSL cells was demonstrated by immunohistochemistry. The alterations in gene expression can be interpreted within several biologic contexts with implications for PCNSL, including CNS tropism (ECM and adhesion-related pathways, SPP1, DDR1), B-cell migration (CXCL13, SPP1), activated B-cell subtype (MUM1), lymphoproliferation (SPP1, TCL1A, CHI3L1), aggressive clinical behavior (SPP1, CHI3L1, MUM1), and aggressive metastatic cancer phenotype (SPP1, CHI3L1). The gene expression signature discovered in our study may represent a true “CNS signature” because we contrasted PCNSL with wide-spectrum non-CNS DLBCL on a genomic scale and performed an in-depth bioinformatic analysis.

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