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 ◽  
...  

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.

2020 ◽  
Vol 11 (03) ◽  
pp. 492-494
Author(s):  
Elanagan Nagarajan ◽  
Sushma Y. Yerram ◽  
Lakshmi P. Digala ◽  
Pradeep C. Bollu

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.


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 ◽  
...  

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.


2020 ◽  
pp. 194187442096756
Author(s):  
Prashant Anegondi Natteru ◽  
Shashank Shekhar ◽  
Lakshmi Ramachandran Nair ◽  
Hartmut Uschmann

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.


Radiology ◽  
2014 ◽  
Vol 272 (3) ◽  
pp. 843-850 ◽  
Author(s):  
Philipp Kickingereder ◽  
Benedikt Wiestler ◽  
Felix Sahm ◽  
Sabine Heiland ◽  
Matthias Roethke ◽  
...  

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2161-2161
Author(s):  
Xiuli Wang ◽  
Ryan Urak ◽  
Walter Miriam ◽  
Laura Lim ◽  
Brenda Aguilar ◽  
...  

Abstract Central nervous system lymphoma (CNSL) is a lymphoid malignancy in which tumors from lymph tissue start in the brain, spinal cord, eye, and/or meninges (primary CNSL) or present as a result of metastasis from initial systemic sites to the CNS (secondary CNSL). The most common CNS lymphomas (about 90%) are B-cell lymphomas. The incidence of primary CNS lymphoma has been increasing over the past 20 years. Multifocal lesions are common. CNS lymphomas carry a worse prognosis than systemic lymphoma. Only a few chemotherapeutic drugs can cross and achieve a therapeutic concentration in the CNS. Therefore, effective treatment is limited and the outcome of disease in relapsed or refractory setting is poor. Recent studies show that intraventricular delivery of rituximab in CNS lymphomas is well tolerated. T cell products that are genetically engineered with chimeric antigen receptors (CARs) targeting CD19 have broad application for adoptive therapy of B cell lineage malignancies and have shown tremendous potential in treatment of systemic lymphoma. In all CD19CAR T cell trials, T cell products are administrated intravenously. CD19CAR T cell trafficking in cerebrospinal fluid (CSF) is frequently reported but most if not all protocols exclude patients with active CNS involvement. In this study, we set out to investigate the feasibility and efficacy of the use of CD19CAR T cells to treat CNSL. Methods and Results: Isolated naïve and central memory T cells (Tn/Tmem) were genetically modified with CD19CAR lentivirus and expanded in vitro for 14 days. 0.1x10^6 human B cell lymphoma Daudi cells were injected intracranially into NSG mice. Tumor was allowed to engraft for 5 days. We administered CD19CAR T cells via three different delivery routes: intracranial local infusion with 1x10^6 CD19CAR T cells (i.c), intracerebroventricular (i.c.v) administration with 1x10^6 cells to bypass the blood-brain barrier and target tumor throughout the entire CNS, and intravenous injection (i.v) with 3x10^6 cells. We repeatedly observed in 2 separate experiments (N=5 mice in each experiment) that both a single i.c infusion and a single i.c.v delivery of CD19CAR T cells were able to completely eradicated CNS lymphoma in all mice by day 14 post CAR T cell infusion; and that a single dose of i.v infusion induced significant anti-CNSL activity with a slightly delayed response as compared to i.c and i.c.v treatment and all mice achieved complete remission 21 days post T cell infusion. CAR T cells were detected in peripheral blood obtained from retro-orbital bleeding, not only in the i.v treated mice, but also in i.c.v treated mice 28 days after CAR T cell infusion, suggesting that i.c.v not only controls CNSL but may also play a role in immune surveillance for systemic tumors. To confirm this, we established an NSG CNS B cell lymphoma model by also inoculating subcutaneous tumors on the animal's flank, 3 weeks prior to i.c tumor injection into the same mouse. CD19CAR T cells were delivered via i.c.v 5 days after i.c. tumor injection. CAR T cell injection resulted in complete remission of both the brain tumor and the flank tumor 14 days after CAR T cell administration. In conclusion,intracerebroventricular delivery of CD19CAR T cells is a promising and feasible therapeutic approach for both primary central nervous system lymphoma and systemic lymphoma with concurrent CNS involvement. Disclosures No relevant conflicts of interest to declare.


2017 ◽  
Vol 41 (S1) ◽  
pp. S489-S490
Author(s):  
U. Cikrikcili ◽  
B. Saydam ◽  
M. Aktan

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.


Radiology ◽  
1995 ◽  
Vol 197 (3) ◽  
pp. 649-654 ◽  
Author(s):  
R J Chinn ◽  
I D Wilkinson ◽  
M A Hall-Craggs ◽  
M N Paley ◽  
R F Miller ◽  
...  

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