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.

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.

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 Primary Central Nervous System Lymphoma: Diffuse Large B-Cell Lymphoma

2019 ◽  
Vol 25 (4) ◽  
pp. 239-242
Author(s):  
Asuman Ali ◽  
Cemile Haki ◽  
Fatma Öz Atalay ◽  
Ramazan Yalçın
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Primary Central Nervous System Lymphoma: Diffuse Large B-Cell Lymphoma

2019 ◽  
Vol 25 (4) ◽  
pp. 239-242
Author(s):  
Asuman Ali ◽  
Cemile Haki ◽  
Fatma Öz Atalay ◽  
Ramazan Yalçın
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

A prospective study on the circulation and central nervous system after primary central nervous system B cell lymphoma treatment with rituximab.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e13518-e13518
Author(s):  
Tongyu Lin ◽  
Chen Peng ◽  
Shu Liu ◽  
Cheng-cheng Guo ◽  
Zhao Wang ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Control Group ◽  
Lymphoma Treatment ◽  
Time Required ◽  
A Prospective Study

e13518 Background: The use of rituximab(RTX)for the treatment of primary central nervous system lymphoma(PCNSL) is controversial, and whether the RTX permeability of the blood-brain barrier can be improved by craniotomy is unknown. Methods: ImmunocompetentPCNSL patients newly diagnosed via craniotomy or stereotactic biopsy were enrolled and received RTX (375 mg/m2, Q3w) treatment. Systemicnon-Hodgkin's B cell lymphoma (systemic-B-NHL)patients without CNS involvement served as the control group. The trough concentrations of RTX (CRTX) and CD19 levels in cerebrospinal fluid (CSF) and plasma were analyzed by ELISA and flow cytometry methods during each treatment cycle.The efficacy and adverse effects were recorded. Results: From December 2016 to February 2018, 21 PCNSL and 32 systemic-B-NHL patientswere enrolled. The CSF CRTXin the craniotomy-PCNSL group (0.2198±0.1866μg/ml) was significantly higher than those in the stereotactic-PCNSLgroup (0.0613±0.0408 μg/ml, P = 0.031) and the systemic-B-NHLgroup (0.0799±0.0614μg/ml, P = 0.046). The BBB penetrabilityof RTX in the craniotomy-PCNSL group (1.52±1.05%) was nearly four times that in the stereotactic-PCNSL group (0.41±0.19%, P = 0.048) and nearly three timesthat in the systemic-B-NHL group (0.54±0.61%, P = 0.012). No significant differences in the CRTXor BBB penetrability of RTX were observedbetween the stereotactic-PCNSL and systemic-B-NHL groups. CD19 levels in plasma fell below 0.1% in all patients before the second cycle of chemotherapy, and the time required for CSF CD19cell clearance in craniotomy-PCNSL patients tended to be reduced compared with that required by stereotactic-PCNSL patients. The CR and ORR rates of craniotomy-PCNSL patientswere 30% higher than those of stereotactic-PCNSL patients. Conclusions: The BBB penetrability of RTXand the CSF CRTXare significantly improved in PCNSL patients diagnosed via craniotomy.Rituximab could be recommended for routine use in craniotomy PCNSL patients. Clinical trial information: ChiCTR-TRC-11001687.

scholarly journals Diffuse large B-cell lymphoma in immunoprivileged sites: association of vitreoretinal, testicular and central nervous system lymphoma

2013 ◽  
Vol 92 (2) ◽  
pp. e159-e160 ◽  
Author(s):  
Anjo Riemens ◽  
Tom Missotten ◽  
Seerp Baarsma ◽  
Natasa Vidovic-Valentincic ◽  
Katrina Novac-Andrejcic ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

scholarly journals Marginal Zone B-cell Lymphoma of the Gasserian Ganglion: Case Report and Review of the Literature

2022 ◽  
Author(s):  
Ahmed Hegazy ◽  
Hieder Al-Shami ◽  
Biswas Arundhatai ◽  
Mohammed Fathy ◽  
Ahmed M. Salah ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Systemic Disease ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Gasserian Ganglion ◽  
B Cell Lymphomas

AbstractPrimary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin's lymphoma that accounts for 4% of newly diagnosed central nervous system (CNS) tumors. Most primary lymphomas of the central nervous system are of the subtype of diffuse large B-cell lymphomas, which have highly aggressive behavior and may involve the brain, leptomeninges, eyes or spinal cord without evidence of systemic disease. Primary CNS lymphomas are very rare in immunocompetent patients, but their rates are increasing. So far, only 11 primary Gasser ganglion lymphomas have been reported, with an incidence of 2.5 cases per 30,000,000 inhabitants. However, B cell lymphomas of the marginal zone of the Gasserian ganglion have been very rarely reported. We report here a clinical presentation characteristic of B cell lymphoma of the marginal zone of the Gasser ganglion in an immunocompetent patient who was treated with surgery and radiotherapy, evolving with improvement of symptoms and without recurrence in 3 months of follow-up.

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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