Primary CNS Lymphoma
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Information Patients and the Public (8 links)


Information for Health Professionals / Researchers (8 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Wang XX, Huang HQ, Bai B, et al.
Clinical outcomes of patients with newly diagnosed primary central nervous system lymphoma are comparable on treatment with high-dose methotrexate plus temozolomide and with high-dose methotrexate plus cytarabine: a single-institution experience.
Leuk Lymphoma. 2014; [PubMed] Related Publications
The role of temozolomide in untreated PCNSL patients has not yet been clearly defined. The purpose of this study was to compare the efficacy and toxicity of MT and MC chemotherapy in this population. A total of 41 consecutive patients were enrolled from March 2001 to July 2011. The ORR and CRR for MT vs. MC were 70% vs. 61.9% and 45% vs. 38.1% on ITT basis, (p = NS); 73.7% vs. 68.4% and 47.4% vs. 42.1% on PP basis, respectively (p = NS). Grade 3-4 hematological toxicities were more common in MC than in MT group (85.7% vs. 15%, p = 0.0001). One treatment-related death was observed in each group. The 5-year PFS and OS of MT (36% and 62.2%) were comparable to MC (32.6% and 46.7%), (p = NS). In summary, our preliminary results suggest that MT combination may be a simplified and effective regimen comparable to MC for newly diagnosed PCNSL.


Wang Y, Wang X, Zhao Y, et al.
[Curative effect of pemetrexed on the treatment of relapsed primary central nervous system lymphoma].
Zhonghua Xue Ye Xue Za Zhi. 2014; 35(1):46-9 [PubMed] Related Publications
OBJECTIVE: To explore the efficacy and safety of pemetrexed in the treatment of relapsed primary central nervous system lymphoma (PCNSL).
METHODS: Seven cases with relapsed PCNSL admitted in our hospital between August 2012 and August 2013 were retrospectively reviewed.
RESULTS: Of the 7 relapsed cases, ectopic recurrence occurred in 3, in situ recurrence in 3 and leptomeningeal metastasis in 1. Patients with relapsed PCNSL were administered with high-dose pemetrexed (900 mg/m²) once for every 3 weeks and supplemented with folic acid and vitamin B₁₂. Complete remission was obtained in 2 patients, partial remission in 3 patients and progressive disease in 2. The overall response rate was 71.4% (5/7). The main adverse reactions were myelosuppression and gastrointestinal reaction.
CONCLUSION: Treatment of relapsed PCNSL is difficult, and its prognosis is very poor. Pemetrexed therapy is a meaningful trial.


Lossos C, Bayraktar S, Weinzierl E, et al.
LMO2 and BCL6 are associated with improved survival in primary central nervous system lymphoma.
Br J Haematol. 2014; [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is an aggressive sub-variant of non-Hodgkin lymphoma (NHL) with morphological similarities to diffuse large B-cell lymphoma (DLBCL). While methotrexate (MTX)-based therapies have improved patient survival, the disease remains incurable in most cases and its pathogenesis is poorly understood. We evaluated 69 cases of PCNSL for the expression of HGAL (also known as GCSAM), LMO2 and BCL6 - genes associated with DLBCL prognosis and pathobiology, and analysed their correlation to survival in 49 PCNSL patients receiving MTX-based therapy. We demonstrate that PCNSL expresses LMO2, HGAL(also known as GCSAM) and BCL6 proteins in 52%, 65% and 56% of tumours, respectively. BCL6 protein expression was associated with longer progression-free survival (P = 0·006) and overall survival (OS, P = 0·05), while expression of LMO2 protein was associated with longer OS (P = 0·027). Further research is needed to elucidate the function of BCL6 and LMO2 in PCNSL.


Myageri A, Vandana UG, Melkundi S, et al.
Squash cytology of primary central nervous system lymphoma in an immunocompetent patient.
Asian J Neurosurg. 2013; 8(4):195-8 [PubMed] Free Access to Full Article Related Publications
The diagnosis of primary central nervous system lymphoma (PCNSL) can be suggested by magnetic resonance imaging (MRI). Cerebral PCNSL in immunocompetent patients generally shows marked homogeneous contrast enhancement in MRI. However, confirmation of it requires demonstration of lymphoma cells in stereotactic biopsy of the lesion or in cerebrospinal fluid. In the present case, with radiologic diagnosis of glioma, complete resection of the lesion by micro-neurosurgical technique was performed. The tumor tissue was examined by squash smear, frozen section and paraffin sections. Cytologic appreciation of tumor cells as lymphoid origin in squash smears negated the other possibilities. The immunocytochemistry on squash smear and immunohistochemistry on paraffin sections confirmed the diagnosis of diffuse large B cell lymphoma of non-germinal center B cell phenotype.


Kinoshita M, Sasayama T, Narita Y, et al.
Different spatial distribution between germinal center B and non-germinal center B primary central nervous system lymphoma revealed by magnetic resonance group analysis.
Neuro Oncol. 2014; [PubMed] Related Publications
BackgroundMRI group analysis is a powerful tool for elucidating pathological conditions in the brain that are challenging to reveal from single subject analysis. This research aimed to elucidate special distribution characteristics of primary central nervous system lymphoma (PCNSL) within the brain with respect to molecular marker expression patterns.MethodsMR images from 100 treatment-naive PCNSL patients were collected and registered onto averaged standard anatomical MRI (MNI152). Gadolinium-enhanced lesions were extracted, and a lesion frequency map was created. Lymphoma subtypes were classified as germinal center B (GCB) or non-GCB by immunohistochemistry in 90 patients.ResultsA PCNSL frequency map showed that these tumors tended to occur around the lateral, third and fourth ventricles. Moreover, GCB (27 cases) and non-GCB (63 cases) PCNSL frequency maps showed GCB lymphomas located at the upper tegmentum and cerebellum around the fourth ventricle, while non-GCB lymphomas tended to occupy the anterior fornix. These differences were significant and confirmed by the existence of voxels with P values <.05 (random permutation analysis with voxel-wise Fisher' exact test). This is the very first report to address phenotypical and spatial distributional differences between GCB and non-GCB PCNSL using an MR group analytical method.


Cho J, Kim J, Ryu HJ, et al.
Pilot study of gamma-knife surgery-incorporated systemic chemotherapy omitting whole brain radiotherapy for the treatment of elderly primary central nervous system lymphoma patients with poor prognostic scores.
Med Oncol. 2014; 31(3):863 [PubMed] Related Publications
Systemic chemotherapy followed by whole brain radiotherapy (WBRT) is a widely used treatment strategy for patients with primary central nervous system lymphoma (PCNSL). However, the outcome of this treatment strategy in elderly patients, particularly with a poor prognostic score, was disappointing compared with younger patients, and the deterioration of cognitive function after WBRT is more problematic in these patients. To avoid this debilitating complication of WBRT and increase treatment efficacy in elderly patients, we designed systemic chemotherapy that incorporated interim gamma-knife surgery (GKS) treatment for elderly PCNSL patients (age≥65 years), omitting WBRT in this pilot trial. A total of four elderly patients with a poor prognostic score based on an International Extranodal Lymphoma Study Group were enrolled in this pilot clinical trial. All study patients acquired complete response and showed stable or improved neuropsychological function during the disease-free state. The median progression-free survival was 9.5 months (range 8.6-22.5 months), and the median overall survival was 15.8 months (range 13.3-25.1 months), which were likely to be similar to those of the chemotherapy followed by WBRT for those patients. This pilot study demonstrated that GKS-incorporated systemic chemotherapy can obtain complete response with high probability and considerably long survival, which suggests that this treatment strategy is efficient and neuropsychologically safe for elderly PCNSL patients with a poor prognostic score.


Pentsova E, Deangelis LM, Omuro A
Methotrexate re-challenge for recurrent primary central nervous system lymphoma.
J Neurooncol. 2014; 117(1):161-5 [PubMed] Related Publications
The prognosis of primary CNS lymphoma (PCNSL) recurring after methotrexate is poor (objective response rates [ORR] = 26-53 %; 1-year overall survival [OS] = 35-57 %). Salvage PCNSL chemotherapies have been based on the use of different agents to avoid cross-resistance; however, methotrexate is the most active agent in PCNSL, and methotrexate re-challenge may be an effective strategy for recurrent disease. We report our experience with methotrexate re-challenge in PCNSL. We reviewed 39 patients with histologically confirmed PCNSL who responded to methotrexate at initial diagnosis, experienced disease relapse and received methotrexate re-challenge. At the time of re-challenge, median age was 66 and median Karnofsky performance score (KPS) was 70. Median time from initial diagnosis was 26 m. Twenty-six patients were at first relapse and 13 at second or later relapse. At re-challenge, methotrexate was given in combination with other agents to 33 patients and as a single agent to six. The objective response rate was 85 %, with a complete response in 29 (75 %) patients, partial response in four (10 %) and disease progression in six (15 %). At median follow-up of 26 m, the median progression-free survival was 16 m; 1-year OS was 79 % (95 % CI 63-89) and median OS was 41 m. KPS was a prognostic factor for progression free survival (p = 0.04). In this population selected by previous methotrexate response, methotrexate re-challenge was a safe and effective strategy, indicating chemosensitivity was retained. Efficacy compared favorably to other salvage treatments suggesting methotrexate re-challenge should be considered in recurrent PCNSL patients who previously responded to methotrexate.


Waliszewska-Prosół M, Bladowska J, Hałoń A, et al.
An older woman after a first generalized seizure. Primary central nervous system lymphoma of the B cell.
Eur Neurol. 2013; 70(5-6):366-8 [PubMed] Related Publications


Yamamoto S, Nagano S, Shibata S, et al.
[Primary central nervous system lymphoma mimicking ventriculitis].
Rinsho Shinkeigaku. 2013; 53(10):831-4 [PubMed] Related Publications
A 66-year-old man presented with deteriorated bradykinesia, gait disturbance, disorientation, and urinary incontinence for three weeks. Magnetic resonance imaging (MRI) showed dilatation of the ventricles. Cerebrospinal fluid (CSF) examination demonstrated lymphocytic pleocytosis, elevation of protein levels, and decreased of glucose levels. A gadolinium-enhanced MRI revealed lesions in the ventricular wall and choroid plexus, mimicking ventriculitis. No evidence of bacterial, fungal, mycobacterial, or viral infections were observed in the CSF. Flow cytometry of CSF showed predominance of CD20+, λ+ cells. PCR examination of CSF revealed positive IgH gene rearrangement, suggesting B cell lymphoma. Endoscopic brain biopsy showed diffuse large B cell lymphoma. As the patient had no evidence of lymphoma in the other organs, we made a diagnosed of primary central nervous system lymphoma (PCNSL). A limited intraventricular spread of PCNSL is rare but important as one of differential diagnosis of ventriculitis.


Kuittinen O, Siniluoto T, Isokangas M, et al.
[Chemotherapy in conjunction with blood brain barrier disruption in the treatment of primary central nervous system lymphoma].
Duodecim. 2013; 129(15):1563-70 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is sensitive to both chemotherapy and radiation, but the blood-brain barrier limits the usefulness of the most effective chemotherapeutic agents. On the other hand radiation therapy carries along serious long term adverse events. In BBBD-therapy the blood-brain barrier is opened with intra-arterial mannitol infusion thus permitting both the chemotherapeutics and antibodies to enter through blood-brain barrier. So far 17 patients have started the therapy in our clinics. Ten patients have reached a complete response and 8 of these responses are ongoing with follow-up times of 6-62 months.


Aoki H, Ogura R, Tsukamoto Y, et al.
Advantages of dose-dense methotrexate protocol for primary central nervous system lymphoma: comparison of two different protocols at a single institution.
Neurol Med Chir (Tokyo). 2013; 53(11):797-804 [PubMed] Related Publications
The efficacy and toxicity of high-dose methotrexate (HD-MTX)-based chemotherapy were retrospectively reviewed in patients with primary central nervous system lymphoma (PCNSL). All immunocompetent patients with histologically or radiographically diagnosed PCNSL treated between 2006 and 2012 at Niigata University Hospital were enrolled. Thirty-eight patients with a diagnosis of PCNSL were treated with one of two regimens during different time periods. During the first period, from 2006 to 2009, three 3-week cycles of MPV (MTX + procarbazine + vincristine) were administered (MPV3 group). In the second period, from 2010 to 2012, five 2-week cycles of MTX were administered (MTX5 group). High-dose cytarabine was used in both groups following HD-MTX-based chemotherapy. Whole-brain radiotherapy was used for patients who did not attain a complete response (CR) based on magnetic resonance images. In the MPV3 group, 20 out of 23 patients (87%) completed the planned treatment. The CR rate after chemotherapy was 30%, and 57% after radiation therapy. Thirteen out of 15 patients (87%) in the MTX5 group completed the planned treatment. The CR rates after chemotherapy and radiation therapy were 53% and 93%, respectively. Renal dysfunction was assessed by measuring creatinine clearance rates, which were very similar in both groups. In terms of hematologic toxicity and other adverse reactions, there was no significant difference between the two groups. In conclusion, dose-dense MTX chemotherapy improved outcome with acceptable toxicity compared with the treatment schedule for three cycles of MPV treatment.


Lapa C, Löhr M, Bluemel C, et al.
Primary central nervous system lymphoma and meningioma in DOTATATE PET/CT.
Clin Nucl Med. 2013; 38(12):994-5 [PubMed] Related Publications
Although meningiomas are among the most frequent intracranial tumors, primary central nervous system lymphoma represents a rare variant of extranodal non-Hodgkin-type lymphoma. Here, we report on a 73-year-old man with 2 suspicious intracerebral lesions. Combined DOTATATE PET/CT identified 1 lesion as meningioma, whereas the second lesion could not be further specified although a different meningioma was felt very unlikely. Open biopsy of this lesion confirmed the diagnosis of primary central nervous system lymphoma.


Grossman R, Nossek E, Shimony N, et al.
Intraoperative 5-aminolevulinic acid-induced fluorescence in primary central nervous system lymphoma.
J Neurosurg. 2014; 120(1):67-9 [PubMed] Related Publications
The authors report a case of primary CNS lymphoma located in the floor of the fourth ventricle that showed intense fluorescence after preoperative administration of 5-aminolevulinic acid. The authors believe that this is the first demonstration of a 5-aminolevulinic acid-induced fluorescence pattern in primary CNS lymphoma.


Taylor JW, Flanagan EP, O'Neill BP, et al.
Primary leptomeningeal lymphoma: International Primary CNS Lymphoma Collaborative Group report.
Neurology. 2013; 81(19):1690-6 [PubMed] Article available free on PMC after 05/11/2014 Related Publications
OBJECTIVE: To evaluate clinical presentation, optimal diagnostic evaluation and treatment, and outcome in primary leptomeningeal lymphoma, a rare form of primary CNS lymphoma without parenchymal or systemic involvement.
METHODS: The International Primary CNS Lymphoma Collaborative Group, a multidisciplinary group of physicians with a particular interest in primary CNS lymphoma, retrospectively identified cases of lymphoma isolated to the leptomeninges as diagnosed by CSF cytology, flow cytometry, or biopsy, without systemic or parenchymal brain/spinal cord lymphoma or immunodeficiency.
RESULTS: Forty-eight patients were identified, with median age at diagnosis of 51 years and median Eastern Cooperative Oncology Group performance status of 2. Presenting symptoms were multifocal in 68%. Leptomeningeal enhancement was seen in 74% and CSF profile was abnormal in all cases. CSF cytology detected malignant lymphocytes in 67%. Flow cytometry identified monoclonal population in 80%, as did receptor gene rearrangement studies in 71%. Sixty-two percent had B-cell lymphoma, 19% T-cell, and 19% unclassified. Treatment varied and included fractionated radiotherapy (36%), systemic chemotherapy (78%), and intra-CSF chemotherapy (66%), with 66% receiving ≥ 2 modalities. Seventy-one percent had a favorable clinical response; ultimately, 44% received salvage treatment. Median overall survival was 24 months, with 11 patients still alive at 50 months follow-up.
CONCLUSION: Primary leptomeningeal lymphoma is a rare form of primary CNS lymphoma. Patients usually present with multifocal symptoms, with evidence of leptomeningeal enhancement and diagnostic CSF analysis. Although treatment is highly variable, patients have a better prognosis than previously reported and a subset may be cured.


Uldrick TS, Pipkin S, Scheer S, Hessol NA
Factors associated with survival among patients with AIDS-related primary central nervous system lymphoma.
AIDS. 2013; [PubMed] Related Publications
OBJECTIVE:: AIDS-related primary central nervous system lymphoma (AR-PCNSL) has a poor prognosis. Improved understanding of specific patient, infectious, diagnostic, and treatment-related factors that affect overall survival (OS) are required to improve outcomes.
DESIGN:: Population-based registry linkage study.
METHODS:: Adult cases from the San Francisco AIDS registry (1990-2000) were matched with the California Cancer Registry (1985-2002) to ascertain AR-PCNSL data. Survival time was assessed through 31 December 2007. Risk factors and temporal trends for death were measured using two-sided Kaplan-Meier and Cox analyses.
RESULTS:: Two hundred and seven AR-PCNSL patients were identified: 68% were white, 20% Hispanic, 10% African-American, and 2% Asian. Nineteen percent of patients had central nervous system (CNS) opportunistic infections diagnosed prior to AR-PCNSL. Fifty seven percent of patients received radiation and/or chemotherapy and 12% used HAART prior to or within 30 days of AR-PCSNL diagnosis. One hundred and ninety-nine patients died (34 deaths/100 person-years). In adjusted analysis, prior CNS opportunistic infections diagnosis increased risk of death (hazard ratio 1.9, P = 0.0006) whereas radiation and/or chemotherapy decreased risk (hazard ratio 0.6, P < 0.0001). AR-PCNSL diagnosis 1999-2002 had a lower mortality risk (hazard ratio = 0.4, P = 0.02) compared to 1990-1995. African-Americans had an increased risk of death compared to whites or Asians (hazard ratio = 2.0, P = 0.007).
CONCLUSION:: OS among AR-PCSNL patients improved over time but remains poor, especially among African-Americans. Prospective evaluation of curative therapy in AR-PCNSL is urgently needed. Accurate diagnosis of CNS mass lesions in patients with AIDS is required and for those with AR-PCNSL, antiretroviral therapy with concomitant AR-PCNSL therapy, and antimicrobial supportive care may improve OS.


Miki Y, Tomiyama M, Kurotaki H, et al.
Primary central nervous system lymphoma mimicking Bickerstaff's encephalitis.
Neurol Sci. 2014; 35(1):139-41 [PubMed] Related Publications


Salamoon M, Hussein T, Kenj M, Bachour M
High-dose methotrexate, high-dose cytarabine and temozolomide for the treatment of primary central nervous system lymphoma (PCNSL).
Med Oncol. 2013; 30(4):690 [PubMed] Related Publications
Treatment of primary central nervous system lymphoma (PCNSL) associates with low response rates and poor survival using conventional radio and chemotherapy. Due to its favorable toxicity profile, temozolomide has emerged as a new option for treatment of PCNSL in young patients. In this study, we report a series of PCNSL patients treated with an innovative regimen combining high dose of both cytarabine and methotrexate with temozolomide without radiotherapy or intrathecal chemotherapy. To evaluate a new intensive chemotherapy with temozolomide, trying to assess response and progression-free survival rates and if the results are promising, we are aiming at evaluating the overall survival (OS) taking into consideration the toxicity profile. The study was performed at Al Mowassa Charity Hospital in Damascus (Syria). Forty patients with histologically confirmed PCNSL median age 52 years (range 20-65) years were included. Biopsies were cultured, and a karyotyping was made in 32 patients. An induction chemotherapy was started, and methotrexate 3 gr/m² over 12 h on day 1, cytarabine 3 gr/m² every 12 h on day 1 and temozolomide 150 mg/m² from day 2 through day 6 with a total of 6 cycles were given on a monthly basis. Among the 40 patients included in the study, a complete response was observed in 34 patients (85%) and a partial response in the remaining 6 patients (15%). Disease progressed in 8 out of 40 patients (20%) while 32 patients are still living at 5 years making the OS reaching 77%. Grade II nephrotoxicity was observed in 2 patients while grade III and IV hematotoxicity was observed in 5 patients. High dose of both Ara-C and MTX combined with temozolomide appears to be a good choice in the treatment of PCNSL, in the light of good response and OS rates, taking into consideration the acceptable toxicity profile. However, a larger trial is needed to make it an acceptable new combination as a first line for PCNSL patients.


Nayak L, Batchelor TT
Recent advances in treatment of primary central nervous system lymphoma.
Curr Treat Options Oncol. 2013; 14(4):539-52 [PubMed] Article available free on PMC after 05/11/2014 Related Publications
OPINION STATEMENT: Therapeutic options are limited in primary central nervous system lymphoma (PCNSL) with no uniform consensus on optimal management and few published, randomized trials. High-dose methotrexate in combination with other chemotherapeutic agents forms the mainstay of treatment. There hasn't been much progress beyond high-dose methotrexate in this disease, and although results from trials using high-dose chemotherapy and autologous stem-cell transplant seem promising, these need to be further validated. Moreover, the role of whole brain radiation in the upfront setting remains to be determined. However, international efforts in this direction are underway, with ongoing randomized trials in newly diagnosed PCNSL, more research on the molecular pathogenesis and biomarkers, and the use of novel agents in salvage therapy. There also is emphasis on quality of life parameters and neurocognitive status. Future treatment options should optimize high-efficacy rates while minimizing the risk of neurotoxicity.


Jankowski PP, Levy ML, Crawford JR
Post-traumatic basal ganglia haemorrhage in a child with primary central nervous system lymphoma.
BMJ Case Rep. 2013; 2013 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare tumour of childhood with 15-20 cases reported yearly in North America. We present a case of a 13-year-old boy diagnosed with PCNSL who presented more than one-and-a-half years post-treatment with high dose cytosine arabinoside and methotrexate with a right-sided basal ganglia haemorrhage on MRI following a concussion while playing organised football against medical advice. There was no evidence of an underlying vascular malformation or recurrent disease by MRI, cerebrospinal fluid analysis or positron emission tomography computed tomography (PET-CT). However, 6 months post-injury he presented with asymptomatic disease recurrence of the frontal lobe. Our case reports an unusual MRI pattern of post-traumatic injury in a child previously treated for PCNSL that would support a recommendation for the avoidance of contact sports in this population.


Xie H, Dahiya S, Murphy ES, et al.
Primary central nervous system lymphoma in the elderly: the Cleveland clinic experience.
Anticancer Res. 2013; 33(8):3251-8 [PubMed] Related Publications
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a type of extranodal non-Hodgkin lymphoma that involves only the central nervous system. Untreated PCNSL in the elderly has a rapidly fatal course.
PATIENTS AND METHODS: In this retrospective study, we evaluated the demographics, management, and outcomes of patients over 60 years of age with PCNSL at our institution.
RESULTS: A total of 54 patients with a median age of 67 years were included in the analysis. The initial treatment regimens included whole-brain radiation therapy (WBRT), chemotherapy with or without consolidation WBRT. The median progression-free survival (PFS) was 8.0 months (95% confidence interval CI=2.7-22 months) and the median overall survival (OS) was 38 months (95% CI=18-65 months). On multivariable analysis, age younger than 70 years and Karnofsky Performance Status (KPS) no less than 70 were favorable prognostic factors for both OS and PFS.
CONCLUSION: Aggressive treatment strategies for elderly patients with PCNSL with good performance status can lead to improved outcomes in this patient population.


O'Neill BP, Decker PA, Tieu C, Cerhan JR
The changing incidence of primary central nervous system lymphoma is driven primarily by the changing incidence in young and middle-aged men and differs from time trends in systemic diffuse large B-cell non-Hodgkin's lymphoma.
Am J Hematol. 2013; 88(12):997-1000 [PubMed] Related Publications
There has been an overall decline in the United States incidence of Primary CNS Lymphoma (PCNSL) from 1998 to 2008. This study's intent was to characterize the cohorts contributing to it. First, calculated the PCNSL incidence rates from nine Surveillance, Epidemiology, and End Results (SEER) registries for time period 1973 to 2008. Second, examined the time trends overall and by age and gender. Third, used 1992-2008 SEER data from the same registries to obtain overall trends for diffuse large B-cell lymphoma (DLBCL). Last, rates were age-adjusted to the 2000 US standard population and reported per 100,000 person-years. Rates continued to increase in women at all ages and men aged 65 and older. In men aged 20-39 and 40-64 years incidence rates peaked in 1995 and then declined dramatically, stabilizing after 1998. The trends in the incidence of PCNSL over this time frame were significantly different from DLBCL for ages 20-39 (P < 0.001) and 40-64 (P < 0.001) years but were not different for the 65 years and older age group (P = 0.99). The overall PCNSL incidence rate declined since 1995 and was driven primarily by the changing incidence in young and middle-aged men. The rate has continued to increase in men aged 65 years and older and in women. The trends in incidence in the younger age groups over this time period did not parallel those observed for DLBCL.

Related: AIDS-Related Lymphoma Non Hodgkin's Lymphoma Kaposi Sarcoma Skin Cancer USA


Park S, Kang JI, Bang H, et al.
A case of the cauda equina syndrome associated with the intrathecal chemotherapy in a patient with primary central nervous system lymphoma.
Ann Rehabil Med. 2013; 37(3):420-5 [PubMed] Article available free on PMC after 05/11/2014 Related Publications
The intrathecal chemotherapy with methotrexate and cytarabine arabinoside is used for the treatment and prophylaxis of the primary central nervous system lymphoma. The therapy may induce neurotoxicity including the cauda equina syndrome. We report a case of a 58-year-old man with the diffuse large B-cell lymphoma, who developed the cauda equina syndrome after the administration of intrathecal methotrexate and cytarabine arabinoside, as diagnosed by the electrodiagnostic, urodynamic, and radiologic approaches.


Ma SK, Lee KH, Kim SW
Hyponatremia associated with primary central nervous system lymphoma.
Clin Exp Nephrol. 2014; 18(1):170-1 [PubMed] Related Publications


Wang Y, Liu B, Xu D, et al.
Phase II trial of temozolomide plus concurrent whole-brain radiation followed by TNV regimen as adjuvant therapy for patients with newly diagnosed primary CNS lymphoma.
Neurol India. 2013 May-Jun; 61(3):260-4 [PubMed] Related Publications
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is an aggressive extranodal non-Hodgkin's lymphoma limited to the CNS. Treatment of PCNSL with high-dose methotrexate (HD-MTX)-based chemotherapy and whole-brain radiotherapy (WBRT) is associated with high rates of relapse and severe treatment-related neurotoxicity.
AIM: To report our experience of treating newly diagnosed PCNSL with temozolomide, nedaplatin, and vincristine (TNV), as the replacement of HD-MTX, in combination with concurrent chemoradiotherapy.
MATERIALS AND METHODS: Newly diagnosed PCNSL patients were given concurrent temozolomide (75 mg/m 2 , orally) daily during WBRT. Then, the TNV regimen was given after four weeks. The TNV regimen consisted of temozolomide (200 mg/m 2 orally: Days 1-5), nedaplatin (80 mg/m 2 intravenous: Day 1), and vincristine (1.4 mg/m 2 intravenous: Day 1). Each cycle was of a duration of four weeks and a maximum of six cycles were applied. The primary end point was response to treatment obtained by magnetic resonance imaging (MRI). Secondary end points were progression-free survival (PFS) and fewer toxic effects.
RESULTS: The study subjects included 14 patients (median age: 53.5, median Karnofsky Performance Scale (KPS): 75). The median number of TNV cycles given was five. RESPONSE TO TREATMENT: Complete response in 12 (85.7%) patients, partial response in 2 (14.3%) patients, and none with progressive disease. The objective response rate was 100%, and median PFS was 21.4 months. Toxicity was relatively mild, which mainly included nausea in six and fatigue in five, grade 3-4 hematotoxicity in one, and abnormal liver functions in five patients. No neurotoxicity has been observed till date.
CONCLUSION: The efficacy outcomes in this study are comparable to other reported HD-MTX-based regimens plus WBRT, with an added favorable toxicity profile. Prospective, randomized controlled trials are warranted to confirm such results.

Related: Dacarbazine Non Hodgkin's Lymphoma Vincristine Temozolomide Nedaplatin


Cachia D, Izzy S, Smith T, Ionete C
A rare presentation of hypertrophic olivary degeneration secondary to primary central nervous system lymphoma.
JAMA Neurol. 2013; 70(9):1192-3 [PubMed] Related Publications


Kawai N, Miyake K, Yamamoto Y, et al.
18F-FDG PET in the diagnosis and treatment of primary central nervous system lymphoma.
Biomed Res Int. 2013; 2013:247152 [PubMed] Article available free on PMC after 05/11/2014 Related Publications
This paper summarizes the usefulness and limitation of positron emission tomography (PET) with (18)F-fluorodeoxyglucose ((18)F-FDG) in the diagnosis and treatment of primary central nervous system lymphoma (PCNSL). The (18)F-FDG uptake in typical PCNSL is about 2.5 times higher than that in the normal gray matter, and the tumor can usually be identified visually. The (18)F-FDG uptake pattern and value provide useful information for differentiating PCNSL from other enhancing malignant brain tumors especially glioblastoma (GB). The (18)F-FDG uptake in typical PCNSL is usually homogenous, and the uptake value is significantly higher than that in GB. However, (18)F-FDG PET often fails to show the presence of tumor in the brain as (18)F-FDG uptake is faint in atypical PCNSL such as disseminated or nonenhancing lesions. (18)F-FDG PET is also useful for evaluating the treatment response at a very early stage after the initial treatment. Pretreatment and posttreatment (18)F-FDG uptake values may have a prognostic value in patients with PCNSL. In conclusion, (18)F-FDG PET is very useful in the diagnosis of typical PCNSL and can differentiate PCNSL from other malignant brain tumors. However, the usefulness of (18)F-FDG PET is limited in the diagnosis of atypical PCNSL.


Adachi K, Yamaguchi F, Node Y, et al.
Neuroimaging of primary central nervous system lymphoma in immunocompetent patients: comparison of recent and previous findings.
J Nippon Med Sch. 2013; 80(3):174-83 [PubMed] Related Publications
The typical neuroimaging features of primary central nervous system lymphoma (PCNSL) have been described as single or multiple intra-axial, homogenous, contrast-enhancing lesions with marked perilesional edema and restricted diffusion, usually contacting the cerebrospinal fluid surface. Necrosis, peripheral enhancement, hemorrhages, and calcifications are unusual. Recently, some of our patients with PCNSL have had atypical neuroimaging features even before treatment. In this article, we review the neuroimaging characteristics of PCNSL in immunocompetent patients and analyzed how imaging findings over the last 10 years differ from those from more than 10 years ago. Neuroimaging findings suggest that PCNSL is a disease that affects the entire brain. Although some imaging findings are characteristic of PCNSL, the frequency of atypical findings on conventional neuroimaging is increasing. Atypical neuroimaging findings do not rule out PCNSL, even in immunocompetent patients.


Mao X, Sun Y, Tang J
Serum miR-21 is a diagnostic and prognostic marker of primary central nervous system lymphoma.
Neurol Sci. 2014; 35(2):233-8 [PubMed] Related Publications
The standard of care for primary central nervous system lymphoma (PCNSL) is systemic chemotherapy with or without whole brain radiotherapy or intrathecal chemotherapy. In contrast to treatment for other brain tumors, efforts at resection are discouraged. However, it is difficult to distinguish PCNSL from other central nervous system tumors which need aggressive surgery in both CT and MRI images. In this study, we assessed whether measurement of miR-21 in the serum could improve diagnostic accuracy for PCNSL. We found that serum miR-21 significantly increased in PCNSL when compared with other brain tumors and normal controls in both test and validation cohort. Further, serum miR-21 could discriminate PCNSL from all controls with an area under the curve of 0.930 for the test cohort and 0.916 for the validation cohort in ROC analysis. Similar results were also obtained in the validation cohort. Besides, raised concentrations of miR-21 in serum could differentiate PCNSL from glioblastoma under the curve of 0.883 for the test cohort and 0.851 for the validation cohort in ROC analysis. Furthermore, Kaplan-Meier curve analysis (p = 0.03 for test cohort and 0.02 for validation cohort) and Multivariable Cox regression (p = 0.03 for test cohort and 0.01 for validation cohort) revealed serum miR-21 as an independent and powerful predictor of overall survival. Taken together, our results demonstrate that serum miR-21 may represent a diagnostic and prognostic marker for PCNSL.


He J, Wu J, Jiao Y, et al.
Limited detection of IgH gene rearrangements in plasma of patients with primary central nervous system lymphoma.
J Neurooncol. 2013; 114(3):275-9 [PubMed] Related Publications
Chemotherapy-based treatment of patients with primary central nervous system lymphoma can lead to durable remissions and potentially cure in a fraction of patients. Accurate assessment of residual disease is necessary to determine the duration and success of treatment that cannot be achieved by contrast-enhanced imaging due to limited sensitivity and specificity. A tumor-derived blood-based biomarker, if detectable and quantifiable, could serve as a more specific and reliable marker for these patients. The goal of this study was to assess whether lymphoma-specific IgH rearrangements can be detected in plasma of patients with PCNSL. PCNSL tissue was analyzed by capturing and sequencing the IgH genomic regions (IgCap) using next generation sequencing with the Illumina platform. Plasma of patients with detected IgH rearrangement was then analyzed for presence of the respective rearrangement using polymerase chain reaction. Tumor tissue and matched plasma of five treatment-naïve patients with biopsy-proven PCNSL (mean age of 65.6 years; range 62-68 years) were analyzed. All patients had measurable contrast-enhancing disease on MRI at time of plasma collection. IgH rearrangements were identified in 4 of 5 analyzed PCNSL tissue samples. The respective rearrangement could be detected in the plasma of 1 patient (25 %) but not in the others. IgH rearrangements can be detected in tumor tissue of patients with PCNSL using IgCap, however, they are absent or only present in minimal quantities in plasma, even in treatment-natïve patients with bulky disease. Alternative strategies to develop circulating biomarkers for PCNSL patients need to be explored.

Related: Non Hodgkin's Lymphoma


Madkhali I, Bakshi N, Akhtar S, Maghfoor I
Local recurrence of primary central nervous system lymphoma due to tumor seeding.
Ann Saudi Med. 2013 May-Jun; 33(3):310-1 [PubMed] Related Publications


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