Primary CNS Lymphoma
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CNS Tumours

Information Patients and the Public (8 links)


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Latest Research Publications

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


Erratum: Hyperosmolar and methotrexate therapy avoiding surgery in the acute presentation of primary central nervous system lymphoma: Erratum.
Surg Neurol Int. 2014; 5:165 [PubMed] Free Access to Full Article Related Publications
[This corrects the article on p. S175 in vol. 5, PMID: 25184099.].


Gloghini A, Carbone A
Primary central nervous system lymphoma.
J Neurosci Rural Pract. 2015; 6(1):2-3 [PubMed] Free Access to Full Article Related Publications


Nakajima S, Okada T, Yamamoto A, et al.
Primary central nervous system lymphoma and glioblastoma: differentiation using dynamic susceptibility-contrast perfusion-weighted imaging, diffusion-weighted imaging, and (18)F-fluorodeoxyglucose positron emission tomography.
Clin Imaging. 2014; [PubMed] Related Publications
OBJECTIVE: The objective was to evaluate diagnostic performance of dynamic susceptibility-contrast perfusion-weighted imaging, diffusion-weighted imaging, and (18)F-fluorodeoxyglucose positron emission tomography in differentiating primary central nervous system lymphoma (PCNSL) and glioblastoma.
MATERIALS AND METHODS: Twenty-three glioblastomas and 11 PCNSLs were analyzed with uncorrected cerebral blood volume (CBV) ratio, fifth percentile value of cumulative apparent diffusion coefficient histogram (ADC5%), and maximum standardized uptake value (SUVmax) using regions of interest created semiautomatically on enhancing areas.
RESULTS: Uncorrected CBV ratio was highly capable of differentiating PCNSL from glioblastoma, as well as SUVmax and ADC5%.
CONCLUSIONS: Uncorrected CBV ratio demonstrates high diagnostic performance comparable to SUVmax.


Kawakami T, Sakai K, Mimura Y, et al.
Development of Primary Central Nervous System Lymphoma Associated with Human Immunodeficiency Virus and JC Virus Infection.
J Clin Exp Hematop. 2014; 54(3):211-7 [PubMed] Related Publications
We report here a case of a 37-year-old man with human immunodeficiency virus (HIV) infection followed by JC virus (JCV) infection and primary central nervous system lymphoma (PCNSL). The patient had been infected with HIV type 1 due to blood products for hemophilia A during infancy. He had progression of nervous symptoms and was diagnosed with progressive multifocal leukoencephalopathy (PML) clinically at the age of 36, when his CD4-positive lymphocyte counts ranged between 350 and 450/μl. Oral mefloquine, intravenous methylprednisolone pulse therapy, and intravenous immunoglobulin were not effective for the PML, and the patient entered a vegetative state. Brain biopsy revealed JCV infection without pathological findings of PML. Eight months after the clinical diagnosis of PML, he developed respiratory failure and brain magnetic resonance imaging revealed a mass lesion in the brain stem. The patient died 19 months after the diagnosis of PML. Autopsy findings were compatible with PCNSL. EBV-encoded small RNA-1-positive cells were not detected. We present a case of JCV-positive PCNSL with HIV infection complicated with clinical PML.


Maekawa K, Moriguchi-Goto S, Kamiunten A, et al.
Primary Central Nervous System Lymphoma in Miyazaki, Southwestern Japan, a Human T-Lymphotropic Virus Type-1 (HTLV-1)-Endemic Area: Clinicopathological Review of 31 Cases.
J Clin Exp Hematop. 2014; 54(3):179-85 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare and aggressive brain tumor. The aim of this study was to clarify the prevalence of T-cell-type PCNSL (T-PCNSL) in a human T-lymphotropic virus type-1 (HTLV-1)-endemic area of Southwestern Japan. We retrospectively investigated 31 PCNSL cases diagnosed between 1996 and 2013 at the University of Miyazaki Hospital. These cases accounted for 4.4% of all nodal or extranodal malignant lymphomas. Histologically, most of these cases were diagnosed as diffuse large B-cell lymphoma, while only two cases were considered to be low-grade and high-grade B-cell lymphoma (not otherwise specified). No T-PCNSL was found in this series. In addition, Epstein-Barr virus-encoded RNAs were not detected by in situ hybridization in any of the cases. Overall, no T-PCNSL cases were found in 18 years in a region with a high frequency of HTLV-1 seropositivity, namely, Southwestern Japan. This suggests that PCNSL and lymphomas of other anatomical sites are biologically distinct.


Yamaguchi S, Hirata K, Kaneko S, et al.
Combined use of 18 F-FDG PET and corticosteroid for diagnosis of deep-seated primary central nervous system lymphoma without histopathological confirmation.
Acta Neurochir (Wien). 2014; [PubMed] Related Publications
BACKGROUND: Although histological diagnosis is indispensable in treating primary central nervous system lymphoma (PCNSL), we sometimes face an intractable situation in which tissue can be obtained only from a deep-seated brain lesion. In place of a histological diagnosis, the diagnostic adequacy of the combined use of 18 F-FDG PET and corticosteroid administration for PCNSL located in a deep-seated brain structure is reported.
METHODS: Patients with a deep-seated tumor were treated as having PCNSL without histological confirmation, based on the following criteria: (1) there was no evidence of systemic malignancy; (2) the tumor showed an extremely high FDG uptake relative to normal gray matter on pretreatment 18 F-FDG PET; (3) the tumor decreased in size 1 week after diagnostic therapy by corticosteroid administration on contrast-enhanced T1-weighted magnetic resonance imaging (MRI). FDG uptake of the lesion was evaluated by the maximum of standardized uptake values (SUVmax) and tumor-to-normal ratio of the SUV (T/N ratio). The extent of the tumor reduction was calculated by volumetric analysis for the treatment response to corticosteroid administration.
RESULTS: Ten patients (4 males and 6 females) matched these criteria. On pretreatment 18 F-FDG PET, mean SUVmax in the tumor was 24.8 (8.75-60.75), and mean T/N ratio was 3.24 (2.17-5.12). The extent of tumor volume reduction was shown to be 21 to 68 % 1 week after diagnostic therapy by corticosteroids. Mean total dose and duration of corticosteroids were 719 mg as prednisolone and 6.5 days, respectively. Nine patients achieved complete response and one patient achieved partial response on MRI after standard treatment for PCNSL with high-dose methotrexate and/or whole-brain irradiation.
CONCLUSION: Although the value of biopsy is universal, combining 18 F-FDG PET and corticosteroid administration is an important alternative method that may lead to the diagnosis of deep-seated PCNSLs in cases with intractable histopathological confirmations.


Tse TP, Chan AN, Chan TK, Po YC
Post-transplantation primary central nervous system lymphoma in a patient with systemic lupus erythematosus and prolonged use of immunosuppressant.
Hong Kong Med J. 2014; 20(6):541-4 [PubMed] Related Publications
Post-transplantation primary central nervous system lymphoma is an uncommon and fatal post-transplant lymphoproliferative disorder. Such lymphomas have been described in only a few case series in the literature. The incidence of this condition is rising with improved survival after organ transplantation. A case of post-transplantation primary central nervous system lymphoma in a young Chinese woman with systemic lupus erythematosus is described here. She presented with right-sided weakness and memory loss after tooth extraction 2 weeks before admission. Contrast computed tomography of the brain demonstrated a contrast rim-enhancing lesion over the left frontal lobe. With a history of recent dental procedure, long-term immunosuppressive therapy and computed tomography findings, cerebral abscess was highly suspected. Emergency operation was performed. Histopathology showed post-transplantation primary central nervous system lymphoma, with cells positive for B-cell marker CD20. Immunosuppressant was stopped and she was treated with radiotherapy and rituximab (anti-CD20 monoclonal antibody). She remained disease-free at 16 months. Post-transplantation primary central nervous system lymphoma is rare with variable presentation and radiological features. We believe rituximab may have a role in the treatment of such lymphomas.


Pulczynski EJ, Kuittinen O, Erlanson M, et al.
Successful change of treatment strategy in elderly patients with primary central nervous system lymphoma by de-escalating induction and introducing temozolomide maintenance: results from a phase 2 study by The Nordic Lymphoma Group.
Haematologica. 2014; [PubMed] Related Publications
Background: Nordic Lymphoma Group has conducted a phase ll trial in primary central nervous system lymphoma patients applying age-adjusted multi-agent immunochemotherapy regimen, which in elderly patients included temozolomide maintenance treatment. Design and Methods: Patients with newly diagnosed PCNSL aged 18-75 years were eligible. Sixty-six patients (median age 64 years) were enrolled. Two age groups were predefined as those of 18-65 and 66-75 years of age. Results: The overall response rate was 90.8 %. With a median follow-up of 22 months, the 2-year overall survival probability was 60.7 % in patients < 65 years and 55.6% in patients > 65 years (p= 0.40). The estimated progression-free survival at 2 years was 33.1% (CI: 19.1%-47.9%) in patients < 65 years and 44.4% (CI: 25.6%-61.8%) in the elderly subgroup (p=0.74). Median duration of response was 10 months in the younger, not reached in the elderly patients (p=0.33). Four patients aged 64-75 years (6 %) died from treatment related complications. Conclusion: Survival in the two age groups was similar despite a de-escalation of induction treatment in patients > 65 years. Duration of response in elderly patients receiving maintenance temozolomide was longer than in the younger age subgroup. While toxicity during induction is still of concern especially in the elderly patients we conclude from these data that de-escalation of induction therapy in elderly PCNSL patients followed by maintenance treatment seems to be a promising treatment strategy. Trial registration: ClinicalTrials.gov number NCT01458730.


Ichikawa T, Kurozumi K, Michiue H, et al.
Reduced neurotoxicity with combined treatment of high-dose methotrexate, cyclophosphamide, doxorubicin, vincristine and prednisolone (M-CHOP) and deferred radiotherapy for primary central nervous system lymphoma.
Clin Neurol Neurosurg. 2014; 127:106-11 [PubMed] Related Publications
OBJECTIVE: Although high-dose methotrexate and whole-brain radiation therapy (WBRT) is the current standard for primary central nervous system lymphoma (PCNSL), it has a limited response rate and produces radiation-induced neurotoxicity. We report the effect of a combined treatment of high-dose methotrexate, cyclophosphamide, doxorubicin, vincristine and prednisolone (M-CHOP) for immunocompetent patients with PCNSL.
METHODS: We analyzed 24 patients who had received M-CHOP administered in 28-day cycles with or without WBRT. The response rate to M-CHOP, overall survival (OS), and recurrence-free survival (RFS) were analyzed.
RESULTS: Nine patients were treated with M-CHOP plus WBRT and 15 patients were treated with M-CHOP alone. Twenty-one patients achieved a complete response and three patients achieved a partial response to M-CHOP, for a 100% response rate. With a median follow-up of 70 months, the median OS and RFS were 33 and 13 months, respectively. The median OS for patients treated with M-CHOP plus WBRT and M-CHOP alone was 33 and 32 months, respectively. Of the 13 patients whose age was above 65 years, the median OS for the M-CHOP plus WBRT group (two patients) and the M-CHOP alone group (11 patients) was 14 and 32 months, respectively. Toxicities related to M-CHOP were mostly hematologic and generally mild to moderate. Two patients whose age was above 65 years in the M-CHOP plus WBRT group developed neurotoxicity.
CONCLUSION: Combined treatment with M-CHOP was well tolerated and produced a high response rate. Deferring WBRT was associated with reduced neurotoxicity without worsening the prognosis, especially in elderly patients.


Aziz HA, Peereboom DM, Singh AD
Primary central nervous system lymphoma.
Int Ophthalmol Clin. 2015; 55(1):111-21 [PubMed] Related Publications


Mercadal S, Cortés-Romera M, Vélez P, et al.
[Positron emission tomography combined with computed tomography in the initial evaluation and response assessment in primary central nervous system lymphoma.]
Med Clin (Barc). 2014; [PubMed] Related Publications
OBJECTIVE: To evaluate the role of positron emission tomography combined with computed tomography (PET-CT) in the initial evaluation and response assessment in primary central nervous system lymphoma (PCNSL).
MATERIAL AND METHOD: Fourteen patients (8 males) with a median age 59.5 years diagnosed of PCNSL. A brain PET-CT and magnetic resonance imaging (MRI) were performed in the initial evaluation. In 7 patients a PET-CT after treatment was performed.
RESULTS: PET-CT showed at diagnosis 31 hypermetabolic focuses and MRI showed 47 lesions, with a good grade of concordance between both (k=0.61; P=.005). In the response assessment, correlation between both techniques was good, and PET-CT was helpful in the appreciation of residual MRI lesions. Overall survival at 2 years of negative vs. positive PET-CT at the end of treatment was 100 vs. 37.5%, respectively (P=.045).
CONCLUSIONS: PET-CT can be useful in the initial evaluation of PCNSL, and especially in the assessment of response. Despite the fact that PET-CT detects less small lesions than MRI, a good correlation between MRI and PET-CT was observed. It is effective in the evaluation of residual lesions. Prospective studies are needed to confirm their possible prognostic value.


Zhao HT, Chen J, Shi SB, et al.
Pemetrexed plus rituximab as second-line treatment for primary central nervous system lymphoma.
Med Oncol. 2015; 32(1):351 [PubMed] Related Publications
The aim of the study was to determine the efficacy and toxicity of pemetrexed plus rituximab in patients with primary central nervous system lymphoma, who had undergone treatment with high-dose (HD) methotrexate-based regimens. Patients who had failed HD methotrexate-based regimens treatment had ECOG performance status ranging from 0 to 2. Twenty-seven patients received pemetrexed plus rituximab as second-line treatment. Rituximab 375 mg/m(2) was administered on day 0 and pemetrexed 500 mg/m(2) was administered on day 1 every 3 weeks. Six patients (22.2 %) experienced CR, 11 patients (40.7 %) had PR, eight patients (29.6 %) had SD, and two patients had PD. The response rate was 62.9 %. The median time to progression (PFS) was 6.9 months (95 % CI, 5.6-8.3), and the median overall survival was 11.2 months (95 % CI, 9.1-13.4). In the subgroup analysis, PFS had a significant difference among the low level of serum miR-21 and high level of serum miR-21. PFS was 9.0 (95 % CI, 6.3-11.6) and 5.7 months (95 % CI, 4.6-6.9, log rank = 0.015), respectively. None of the patient experienced grade 4 toxicity. A regimen of pemetrexed combined with rituximab is marginally effective and well tolerated in patients with PCNSL who had failed HD methotrexate-based regimens first-line treatment.


Ding Y, Xing Z, Liu B, et al.
Differentiation of primary central nervous system lymphoma from high-grade glioma and brain metastases using susceptibility-weighted imaging.
Brain Behav. 2014; [PubMed] Free Access to Full Article Related Publications
BACKGROUND AND PURPOSE: Conventional MRI is often difficult to distinguish between primary central nervous system lymphomas (PCNSLs), high-grade gliomas and brain metastases due to the similarity of their appearance. The aim of this study was to investigate whether the susceptibility-weighted imaging (SWI) has higher sensitivity than conventional MRI in detecting hemorrhage between PCNSLs, high-grade gliomas and brain metastases, and can be used to differentiate the diagnosis between these tumors.
METHODS: The number of lesions with hemorrhage was quantified by both the conventional MR imaging and SWI. The number of micro-hemorrhage and vessels within lesions were counted on SWI.
RESULTS: The detection rate of hemorrhage on SWI was significantly higher than that on the conventional MR imaging. The intralesional hemorrhagic burden and the number of the vessels within lesions detected by SWI were significantly higher in high-grade gliomas and brain metastases than those in PCNSLs. There was no significant difference in these two parameters between high-grade gliomas and brain metastases. The best predictor to differentiate PCNSLs from high-grade gliomas and brain metastases was intralesional vessel number that yielded the best ROC characteristics and highest classification accuracy.
CONCLUSIONS: SWI is useful in differentiating of PCNSLs from high-grade gliomas and brain metastases.


Roth P, Hoang-Xuan K
Challenges in the treatment of elderly patients with primary central nervous system lymphoma.
Curr Opin Neurol. 2014; 27(6):697-701 [PubMed] Related Publications
PURPOSE OF REVIEW: Approximately 50% of all patients with primary central nervous system lymphoma (PCNSL) are 60 years or older and may therefore be considered as elderly. Although the diagnostic work-up is basically the same in young and in elderly patients, therapeutic strategies vary considerably. Here, we review the characteristics of elderly PCNSL patients with a particular focus on advances in the optimization of treatment regimens.
RECENT FINDINGS: Age has been repeatedly confirmed as a major therapy-independent negative prognostic factor. Benefit from treatment and the tolerability of tumor-specific therapy, particularly whole-brain radiotherapy, are significantly lower in the elderly patients. Still, for patients with newly diagnosed PCNSL, several studies emphasized the indisputable role of high-dose methotrexate as backbone for any therapy regimen also in elderly patients. However, the durability of responses to primary chemotherapy is significantly shorter than in young patients. Recent data from a randomized phase II study for elderly PCNSL patients suggest that the combination of high-dose methotrexate, procarbazine, vincristine and cytarabine is superior to methotrexate in combination with temozolomide.
SUMMARY: Current efforts aim at treating elderly PCNSL patients within clinical trials that are specifically designed for this group of patients. Determining adapted consolidation and/or maintenance treatment to improve disease control in responding patients are the main challenges to be faced by future trials. Together with a better understanding of age-specific changes in the biology of PCNSL, this will pave the road for elderly tailored therapies.


Kranick SM, Goncalves PH, Stetler-Stevenson M, et al.
Paradoxical central nervous system immune reconstitution syndrome in acquired immunodeficiency syndrome-related primary central nervous system lymphoma.
Haematologica. 2015; 100(1):e21-4 [PubMed] Free Access to Full Article Related Publications


Ferreri AJ
Primary central nervous system lymphoma: three linked questions in the situation puzzle of radiotherapy.
Leuk Lymphoma. 2014; :1-3 [PubMed] Related Publications


Vassal F, Pommier B, Boutet C, et al.
Isolated primary central nervous system lymphoma arising from the optic chiasm.
Neurochirurgie. 2014; 60(6):312-5 [PubMed] Related Publications
A 58-year-old previously healthy woman rapidly developed progressive bilateral visual loss. Magnetic resonance imaging revealed a bulging appearance of the optic chiasm, with homogeneous enhancement after gadolinium administration, which suggested an optic glioma or inflammatory disease. In the absence of (para)clinical clues for a specific diagnosis despite extensive investigation, a biopsy of one optic nerve was performed, resulting in a diagnosis of non-Hodgkin B-cell lymphoma. There was no evidence of any other ocular or systemic involvement, therefore the conclusion was that this immunocompetent patient had a primary central nervous system lymphoma isolated in the anterior visual pathway. Treatment included two cycles of polychemotherapy (rituximab, methotrexate, carmustine, etoposide, methylprednisolone), followed by autologous peripheral blood stem cell transplantation and rituximab plus cytarabine consolidation therapy. Subsequently, the patient exhibited significant improvement in vision, and was still disease-free at the 1-year follow-up examination. The aim of the present paper was to provide well-documented clinical, radiological, and intraoperative features of isolated primary malignant lymphoma arising from the anterior visual pathway. A better recognition of this rare pathological entity is necessary for clinicians who may encounter similar presentations, as prompt management is crucial for both a visual and vital prognosis.


Milgrom SA, Yahalom J
The role of radiation therapy in the management of primary central nervous system lymphoma.
Leuk Lymphoma. 2014; :1-8 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is an aggressive neoplasm with a poor prognosis. Early studies of whole brain radiation therapy (WBRT) alone revealed a robust initial response but high rates of local recurrence with long-term follow-up. The addition of high-dose methotrexate (HDMTX)-based chemotherapy improved the durability of disease control. However, delayed neurotoxicity emerged as an important complication, mainly in elderly patients. Therefore, researchers have investigated eliminating WBRT or reducing its dose. Multiple studies of chemotherapy alone have demonstrated inferior disease control. On the other hand, a phase III trial reported that WBRT may be deferred until relapse without compromising survival; however, this trial is fraught with flaws. A recent study of immunochemotherapy and dose-reduced WBRT demonstrated excellent outcomes. Currently, this regimen is being studied in a multi-institutional trial by the Radiation Therapy Oncology Group. WBRT maintains an important position in the armamentarium against PCNSL. This article aims to describe its evolving role.


Lee BS, Juthani RG, Healy AT, et al.
Hyperosmolar and methotrexate therapy avoiding surgery in the acute presentation of primary central nervous system lymphoma.
Surg Neurol Int. 2014; 5(Suppl 4):S175-80 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is an aggressive type of extra-nodal non-Hodgkin lymphoma. Without treatment, PCNSL is associated with significant morbidity and mortality, including rapid neurological deterioration. In contrast to other high-grade intracranial neoplasms, PCNSL is considered to have a high response rate to conventional medical therapy, especially in younger patients, and therefore warrants particular attention in terms of nonsurgical treatment.
CASE DESCRIPTION: We report a case of the medical management of acute deterioration due to rapidly growing PCNSL with mass effect to highlight the efficacy of temporization with hyperosmolar therapy while awaiting the known rapid effects of dexamethasone and methotrexate (MTX) treatment. Surgical intervention was avoided, and tumor response was rapid. The patient had corresponding clinical resolution of symptoms of elevated intracranial pressure with return to neurologic baseline.
CONCLUSIONS: Despite the evidence that PCNSL responds well to steroids and MTX, the rapidity of onset with which this occurs can vary. In patients presenting with mass effect and rapid neurologic decline, there is little evidence to support medical over surgical intervention. Herein we present an illustrative case of a large PCNSL lesion presenting with rapid decline. With clinical improvement in one day and a 50% reduction in tumor volume over less than seven days, the authors present the specific time frame with which PCNSL responds to medical therapy and a safe strategy for medical temporization.


Li Y, Liu F, Liu Y, Zhang J
[The diagnosis and treatment of primary central nervous system lymphoma].
Zhonghua Xue Ye Xue Za Zhi. 2014; 35(8):771-3 [PubMed] Related Publications


Bierman PJ
Surgery for primary central nervous system lymphoma: is it time for reevaluation?
Oncology (Williston Park). 2014; 28(7):632-7 [PubMed] Related Publications
The overall survival rate for primary central nervous system lymphoma (PCNSL) has improved significantly. This prolongation in survival is related mainly to advances in chemotherapy regimens and radiotherapy. In contrast, the role of surgery in PCNSL has been limited because tumors are frequently not amenable to resection and because most studies have failed to show that resection is beneficial. A recent analysis of the German Primary CNS Lymphoma Study Group 1 (G-PCNSL-SG-1) trial has challenged this convention by showing that the survival of patients with PCNSL may actually be prolonged if tumors are resected. While there are a number of weaknesses in the analysis, many authorities now feel that an attempt at gross total resection is reasonable for patients with solitary lesions that can be removed without morbidity. However, even if resection does benefit some patients, the diagnosis of lymphoma is almost always made in retrospect, and there are few occasions when a neurosurgeon will actually need to make a decision whether or not to resect a known PCNSL.


Lee SY, Okoshi Y, Kurita N, et al.
Prognosis factors in Japanese elderly patients with primary central nervous system lymphoma treated with a nonradiation, intermediate-dose methotrexate-containing regimen.
Oncol Res Treat. 2014; 37(7-8):378-83 [PubMed] Related Publications
BACKGROUND: Nearly half of all patients with primary central nervous system lymphoma (PCNSL) are known to be aged over 60 years. However, clinical factors affecting treatment outcomes in elderly patients are understudied.
METHODS: We analyzed 38 patients with PCNSL older than 60 years. All patients were treated with a nonradiation, intermediate-dose methotrexate-containing regimen between March 2005 and May 2013 at the University of Tsukuba Hospital.
RESULTS: The 3-year overall survival and progression-free survival rates were 56.2% (95% confidence interval (CI) 36.2-76.2%) and 29.8% (95% CI 9-50.6%), respectively, with a median follow-up of 36.5 months. We found that an age > 75 years, a Karnofsky performance score < 70, altered mentation, and a creatinine clearance (CrCl) > 90 ml/min were significant (p < 0.05) factors associated with a worse survival, by univariate analysis. Multivariate analysis revealed that CrCl (p < 0.05; hazard ratio (HR) = 3.39; 95% CI 1.08-10.68) and altered mentation (p < 0.05; HR = 6.27; 95%CI 1.37-28.83) were independent significant association factors. The most frequent adverse event was myelosuppression, with grade 3-4 hematologic toxicities in 28 patients. No delayed neurotoxicities were observed.
CONCLUSION: More intensive therapy may be introduced in selected patients with poor prognosis factors to improve outcomes.


Weller M
Primary central nervous system lymphoma in the elderly.
Oncol Res Treat. 2014; 37(7-8):376-7 [PubMed] Related Publications


Cho BJ, Yu HG
Risk factors for intraocular involvement in patients with primary central nervous system lymphoma.
J Neurooncol. 2014; 120(3):523-9 [PubMed] Related Publications
To determine the risk factors for intraocular involvement in patients with primary central nervous system lymphoma (PCNSL), a retrospective chart review was performed on 136 patients who were pathologically diagnosed with PCNSL. The patients were investigated for demographics, clinical manifestation, and the profile of immunohistochemical tumor biomarkers, as well as for the presence of intraocular involvement of lymphoma at diagnosis or during follow-up. The mean age of the entire cohort was 58.6 ± 12.4 years, and the mean follow-up period was 31.1 ± 30.8 months. Twenty-nine (21 %) patients had an intraocular involvement, among which 20 (69 %) patients presented with intraocular involvement at diagnosis of PCNSL and 9 (31 %) patients developed intraocular involvement after a mean period of 32.4 ± 33.6 months. Of the patients with intraocular involvement, 8 (28 %) had no visual symptom at the diagnosis of ocular invasion. Between those with and without intraocular involvement, no significant differences were found with respect to the age, sex, and follow-up period as well as cerebrospinal fluid spread and bone marrow involvement. Among the immunohistochemical biomarkers, the Ki-67 proliferation index was significantly higher in patients with intraocular involvement than in patients without (P = 0.021), but the other investigated biomarkers did not show a significant difference between the two groups. A Ki-67 level ≥80 % was a risk factor for the intraocular involvement in patients with PCNSL (odds ratio, 2.63). Median overall survival was 39.0 months in the entire cohort and was not significantly different between those with and without intraocular involvement (P = 0.959).


Thorer H, Zimmermann M, Makarova O, et al.
Primary central nervous system lymphoma in children and adolescents: low relapse rate after treatment according to Non-Hodgkin-Lymphoma Berlin-Frankfurt-Münster protocols for systemic lymphoma.
Haematologica. 2014; 99(11):e238-41 [PubMed] Free Access to Full Article Related Publications


Ahmed Z, Ramanathan RK, Ram S, et al.
Unusual relapse of primary central nervous system lymphoma at site of lumbar puncture.
Case Rep Hematol. 2014; 2014:161952 [PubMed] Free Access to Full Article Related Publications
Primary CNS lymphoma (PCNSL) is a rare non-Hodgkin's lymphoma confined to the CNS. Local relapse of this disease is common, but extracranial or subcutaneous metastasis is rare with only a few cases being reported in literature. We report a 63-year-old male patient, who responded well to treatment for PCNSL but relapsed two and half years later with a lumbosacral nodule at the site of a previous lumbar puncture due to microscopic tumor seeding. Clinicians treating patients with PCNSL must remain alert to the possibility of extracranial solitary relapse even after the resolution of initial disease because prompt treatment can result in a good outcome.


Nakamaki T
[Recent advances in the treatment of primary central nervous system lymphoma].
Brain Nerve. 2014; 66(8):969-79 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare, aggressive form of lymphoma, accounting for approximately 1-2% of all cases of lymphoid neoplasms in Japan. The current standard treatment for these tumors, which arise in pharmacological sanctuaries isolated by the blood brain barrier, is chemo-radiation with high-dose methotrexate (3g/m(2)) and cytarabine (3g/m(2)) followed by whole brain radiotherapy (WBRT) (45Gy). To overcome the limitations of and neurotoxicities associated with WBRT, several novel therapeutic strategies have been developed. These include either upfront intensive chemotherapy with high-dose alkylating agents combined with stem cell transplantation or less toxic immunochemotherapy with molecular targeted agents such as anti-CD20 monoclonal antibody. The roles of these therapies for PCNSL are being assessed in ongoing randomized clinical trials and will be further explored in the future.


Olivier G, Clavert A, Lacotte-Thierry L, et al.
A phase 1 dose escalation study of idarubicin combined with methotrexate, vindesine, and prednisolone for untreated elderly patients with primary central nervous system lymphoma. The GOELAMS LCP 99 trial.
Am J Hematol. 2014; 89(11):1024-9 [PubMed] Related Publications
Treatment of primary central nervous system lymphoma (PCNSL) in elderly patients remains unsatisfactory. To develop a new high-dose methotrexate (HD-MTX)-based regimen including idarubicin, a phase 1 multicenter dose escalation study was conducted to determine the maximum-tolerated dose (MTD) of idarubicin. Thirty-five immunocompetent patients with PCNSL were enrolled. The median age was 65 years (range, 60-70 years). MTX and vindesine (VDS) were given at the fixed dose of 3 g/m(2) (6-hr intravenous [IV]) and 3 mg/m(2) IV on day 1, respectively. Prednisolone (PRED) was given at the fixed dose of 60 mg/m(2) (IV or orally) on days 1-5. Idarubicin was escalated in increments of 2 mg/m(2) with doses ranging from 12-18 mg/m(2) IV on day 1. Treatment was repeated three times every 3 weeks. Dose-limiting toxicity (DLT) was defined as grade 4 neutropenia for more than 7 days, thrombocytopenia grade 4 or nonhaematological toxicity more than grade 2. The MTD of idarubicin was reached at 16 mg/m(2) . At this level, the main haematological toxicities were thrombocytopenia grade 4: 5% and neutropenia grade 3 or 4 (52%); the main nonhaematological toxicities were grade 3 or 4 infectious disease (5%) and grade 2 renal failure (9%). For the study population, median overall and progression-free survival were 19 and 13 months, respectively. Our study suggests that the MTD of idarubicin in combination with HD-MTX, VDS, and PRED, should be 16 mg/m(2) . Further studies will be necessary to challenge a standard treatment in elderly patients with PCNSL.

Related: Idarubicin Non Hodgkin's Lymphoma Methotrexate Vindesine


Kim BH, Kim IH, Park SH, et al.
Low-dose whole brain radiotherapy with tumor bed boost after methotrexate-based chemotherapy for primary central nervous system lymphoma.
Cancer Res Treat. 2014; 46(3):261-9 [PubMed] Free Access to Full Article Related Publications
PURPOSE: The purpose of this study is to evaluate the outcome of low-dose whole brain radiotherapy (WBRT) with tumor bed boost after methotrexate-based chemotherapy in the management of primary central nervous system lymphoma (PCNSL).
MATERIALS AND METHODS: We retrospectively analyzed 64 patients with pathologically proven PCNSL between 2000 and 2011. Methotrexate-based chemotherapy with a median of five cycles was followed by radiotherapy to the whole brain and to the initial tumor bed. The median dose to the whole brain and to the tumor bed was 27 Gy (range, 18 to 36 Gy) and 50.4 Gy (range, 45 to 54 Gy), respectively.
RESULTS: With a median follow-up period of 27 months, 55 patients (85.9%) achieved complete response (CR). The 5-year overall survival (OS) and progression-free survival (PFS) rates were 52.6% and 39.3%, respectively. In univariate analysis, factors associated with OS were age, performance status, involvement of deep structure, and CR to sequential chemoradiotherapy (CRT). These variables remained as significant factors for OS in multivariate analysis. CR to sequential CRT was the only positive factor associated with PFS (p=0.009). Neurologic toxicity was more common in elderly patients older than 60 years (p=0.025).
CONCLUSION: Low-dose WBRT with tumor bed boost after methotrexate-based chemotherapy might be an effective method for management of PCNSL.


Hatakeyama N, Hori T, Yamamoto M, et al.
Primary central nervous system lymphoma in an immunocompetent 12-year-old boy.
Int J Hematol. 2014; 100(3):211-3 [PubMed] Related Publications


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