scholarly journals Primary Central Nervous System Lymphoma Presenting as Parkinsonism with Atypical MRI Findings and Elevated 14-3-3 Protein

2020 ◽  
Vol 11 (03) ◽  
pp. 492-494
Author(s):  
Elanagan Nagarajan ◽  
Sushma Y. Yerram ◽  
Lakshmi P. Digala ◽  
Pradeep C. Bollu
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Movement Disorders ◽  
Central Nervous System Lymphoma ◽  
Mass Effect ◽  
High Dose ◽  
Rapid Progression ◽  
Mri Findings ◽  
Atypical Presentations ◽  
The Right

AbstractPrimary central nervous system lymphoma (PCNSL) is a rare neoplasm with an incidence of 2 to 3% of all CNS malignancies. The diagnosis can be challenging, especially with atypical presentations. Movement disorders can be one of the rare presentations of PCNSL. Here, we present an unusual case of gradually progressing Parkinsonism with an elevation of cerebrospinal fluid (CSF) 14–3-3 protein and atypical imaging findings found to have PCNSL. A 76-year-old female patient presented with gait and intermittent speech difficulty. Initial workup revealed a contrast-enhancing lesion in the bilateral putamen and head of caudate without any mass effect. Her symptoms were rapidly progressed over 6 months and presented with mild dysarthria, bradykinesia, mild rigidity, and reduced left arm swinging. These features were consistent with Parkinsonism. The repeat imaging showed the progression of hyperintensities in the bilateral putamen. The patient underwent a stereotypic biopsy of the right caudate nucleus, which revealed PCNSL. She was treated with high-dose methotrexate and is currently in remission. Diagnosis of movement disorders remains clinical and rapid progression of symptoms, and atypical presentation must warrant further imaging and workup.

scholarly journals RARE-29. PRIMARY CENTRAL NERVOUS SYSTEM NON-HODGKIN LYMPHOMA IN AN 11-YEAR-OLD BOY: A CASE REPORT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii448-iii448
Author(s):  
Jorge Luis Ramírez-Melo ◽  
Regina M Navarro-Martin del Campo ◽  
Manuel D Martinez-Albarran ◽  
Fernando Sánchez-Zubieta ◽  
Ana L Orozco-Alvarado ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hodgkin Lymphoma ◽  
Central Nervous System Lymphoma ◽  
Complete Response ◽  
High Dose ◽  
Illustrative Case ◽  
Tumor Biopsy ◽  
Non Hodgkin Lymphoma ◽  
The Right

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed. CONCLUSION This report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.

scholarly journals A Case of Primary T-Cell Central Nervous System Lymphoma: MR Imaging and MR Spectroscopy Assessment

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
G. Manenti ◽  
F. Di Giuliano ◽  
A. Bindi ◽  
V. Liberto ◽  
V. Funel ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Mr Imaging ◽  
Mr Spectroscopy ◽  
Neuropsychiatric Symptoms ◽  
Central Nervous System Lymphoma ◽  
Mri Findings ◽  
Characteristic Appearance ◽  
The Right ◽  
Cns Lymphomas

Primary central nervous system lymphomas (PCNSLs) are mainly B-cells lymphomas. A risk factor for the development of PCNSL is immunodeficiency, which includes congenital disorders, iatrogenic immunosuppression, and HIV. The clinical course is rapidly fatal; these patients usually present signs of increased intracranial pressure, nausea, papilledema, vomiting, and neurological and neuropsychiatric symptoms. PCNSL may have a characteristic appearance on CT and MR imaging. DWI sequences and MR spectroscopy may help to differentiate CNS lymphomas from other brain lesions. In this paper, we report a case of a 23-year-old man with T-primary central nervous system lymphoma presenting with a mass in the right frontotemporal lobe. We describe clinical, CT, and MRI findings. Diagnosis was confirmed by stereotactic biopsy of the lesion.

A 75-Year-Old Man With 5 Days of Progressive Gait Difficulties and Confusion

2021 ◽  
pp. 195-198
Author(s):  
Julie E. Hammack
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Blood Cells ◽  
Central Nervous System Lymphoma ◽  
High Dose ◽  
Normal Glucose ◽  
History Of ◽  
The Right ◽  
Large B Cell

A 75-year-old man with a history of chronic obstructive pulmonary disease and ischemic cardiomyopathy was brought to the emergency department after a fall at home. He had a 5-day history of progressive gait disturbance, right-sided weakness, and confusion. He had reported floaters in the right eye for the past month. He was previously well with no history of trauma, fever, anorexia, or change in body weight. Cerebrospinal fluid evaluation showed an increased protein concentration, normal glucose level, no red blood cells, and 4 white blood cells/µL. Cytologic and flow cytometry evaluations were negative. Computed tomograph of the chest, abdomen, and pelvis indicated no adenopathy or visceral lesions. Slitlamp examination of the right eye showed clumps of cells in the vitreous. Vitrectomy was performed, and analysis showed atypical monoclonal B cells consistent with large B-cell lymphoma. The patient was diagnosed with primary central nervous system lymphoma. The patient had an excellent initial clinical response to intravenous corticosteroids (dexamethasone) administered after vitrectomy. Subsequent staging showed no systemic lymphoma. He had hematology-oncology evaluations and was treated with chemotherapy (high-dose intravenous methotrexate, temozolomide, and rituximab) together with intravitreal rituximab for 1 year. He had an excellent clinical and radiographic response to treatment. He remained in complete remission until his death 6 years later (age 81 years) of pneumonia. Primary central nervous system lymphoma accounts for approximately 4% of primary brain tumors and occurs more commonly in persons older than 60 years and those with compromised immune systems. The tumor represents an extranodal form of non-Hodgkin lymphoma and is typically of the diffuse large B-cell type.

scholarly journals ML-14 LOW INVASIVE APPROACH FOR PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA TREATMENT INVOLVING RITUXIMAB–METHOTREXATE + PROCARBAZINE + VINCRISTINE AND TRIPLE INTRATHECAL INJECTION

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii35-ii35
Author(s):  
Takahiro Ogawa ◽  
Kei Oowada ◽  
Kazuna Tanba ◽  
Kouichi Hirakawa
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Lateral Ventricle ◽  
Needle Biopsy ◽  
Central Nervous System Lymphoma ◽  
High Dose ◽  
High Dose Methotrexate ◽  
Invasive Approach ◽  
Dose Methotrexate ◽  
The Right

Abstract INTRODUCTION Although the treatment outcomes of primary central nervous system lymphoma (PCNSL) with multiple treatment regimens involving high–dose methotrexate (MTX)–based multiagent chemotherapy have improved compared with the outcomes with high–dose methotrexate therapy in recent years, its regimen has not been established. Additionally, it is controversial whether intrathecal (IT) MTX injection can be included in a regimen. Our facilities treat PCNSL with R–MPV (rituximab–methotrexate + procarbazine + vincristine) and triple IT injection (methotrexate + Ara C + prednisolone). Here we report a low invasive approach for PCNSL treatment involving R–MPV therapy and triple IT injection. CASE DESCRIPTION A 58–year–old woman complained of prolonged headache and had undergone a medical checkup at our department. Head computed tomography revealed a mass lesion with edema near the right anterior horn of the lateral ventricle. After being hospitalized, biopsy was performed via a small craniotomy for the mass lesion near the right anterior horn of the lateral ventricle, which was suspected to be PCNSL. Multipurpose Head Frame 2 (Mizuho Co., Ltd.) was used for four points of head fixation. Vertek Biopsy Kit (Medtronic Co., Ltd.) was attached to it, and needle biopsy guided by neuronavigation was performed. Finally, biopsy samples were obtained from the lesion. Changes in the entry and target points could be easily made through the operative procedure, which was performed uneventfully. The diagnosis was B–cell–related lymphoma. Subsequently, an Ommaya reservoir was placed via lumbar puncture on postoperative day 7. Finally, the patient was successfully treated with R–MPV therapy and triple IT injection, thereby avoiding repeated lumbar puncture and reduced pain associated with IT injection. CONCLUSION We have reported the diagnosis of PCNSL with needle biopsy guided by neuronavigation and its treatment with R–MPV therapy and triple IT injection after lumbar Ommaya reservoir placement. This approach was associated with reduced invasion and pain in the patient.

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 Elderly Patients: Management and Perspectives

Cancers ◽  
2021 ◽  
Vol 13 (14) ◽  
pp. 3479
Author(s):  
Andrea Morales-Martinez ◽  
Fernando Lozano-Sanchez ◽  
Alberto Duran-Peña ◽  
Khe Hoang-Xuan ◽  
Caroline Houillier
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Elderly Patients ◽  
Treatment Strategies ◽  
Central Nervous System Lymphoma ◽  
High Dose Chemotherapy ◽  
Current Treatment ◽  
Cns Lymphoma ◽  
High Dose ◽  
Consolidation Phase

The management of elderly patients suffering from primary central nervous system (CNS) lymphoma, who represent a rapidly growing population, is challenging. Despite the advances made in PCNSL treatment, the prognosis in older patients remains unsatisfactory. The high risk of systemic and CNS toxicity induced by a high-dose chemotherapy regimen and radiation therapy, respectively, limits the use of consolidation phase treatments in elderly patients and contributes to the poor outcome of these patients. Here, we review the current treatment strategies and ongoing trials proposed for elderly PCNSL patients.

scholarly journals Patients with Primary Central Nervous System Lymphoma Not Eligible for Clinical Trials: Prognostic Factors, Treatment and Outcome

Cancers ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 2934
Author(s):  
Sabine Seidel ◽  
Michelle Margold ◽  
Thomas Kowalski ◽  
Alexander Baraniskin ◽  
Roland Schroers ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Clinical Trials ◽  
Nervous System ◽  
Prognostic Factors ◽  
Central Nervous System Lymphoma ◽  
Initial Response ◽  
Early Response ◽  
Multicenter Trial ◽  
High Dose ◽  
Therapeutic Trials

Patients with primary central nervous system lymphoma (PCNSL) not fulfilling inclusion criteria for clinical trials represent an underreported population. Thirty-four consecutive PCNSL patients seen at our center between 2005 and 2019 with exclusion criteria for therapeutic trials were analyzed (non-study patients) and compared with patients from the G-PCNSL-SG-1 (German PCNSL Study Group 1) study (study patients), the largest prospective multicenter trial on PCNSL, comprising 551 patients. Median follow up was 68 months (range 1–141) in non-study patients and 51 months (1–105) in study patients. Twenty-seven/34 (79.4%) non-study patients received high dose methotrexate (HDMTX), while seven/34 (20.6%) with a glomerular filtration rate (GFR) < 50 mL/min did not. Median overall survival (OS) was six months (95% confidence interval [CI] 0–21 months) in those 34 non-study patients. The 27 non-study patients treated with HDMTX were compared with 526/551 G-PCNSL-SG-1 study patients who had received HDMTX as well. Median OS was 20 months (95% CI 0–45)/21 months (95% CI 18–25) in 27 non-study/526 study patients (p = 0.766). Favorable prognostic factors in non-study patients were young age, application of HDMTX and early response on magnet resonance imaging (MRI). If HDMTX-based chemotherapy can be applied, long-term disease control is possible even in patients not qualifying for clinical trials. Initial response on early MRI might be useful for decision on treatment continuation.

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.

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