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Primary CNS Lymphoma

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Latest Research Publications
CNS Tumours

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).

Schalk E, Zeremski V, Fischer T
Impact of lymphopenia on prognosis of patients with primary central nervous system lymphoma.
Eur J Cancer. 2017; 75:280-283 [PubMed] Related Publications

Yun J, Iwamoto FM, Sonabend AM
Primary Central Nervous System Lymphoma: A Critical Review of the Role of Surgery for Resection.
Arch Cancer Res. 2016; 4(2) [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Primary central nervous system lymphomas (PCNSL) are rare CNS tumors that carry a poor prognosis, with most patients suffering recurrence. Progress has been made in the treatment of this pathology, notably with the widespread use of systemic high dose methotrexate. However, unlike most other malignant CNS neoplasms, surgery for cytoreduction is not routinely performed for this disease, mainly as a result of negative experiences decades ago. Since these studies were published, the availability of intraoperative monitoring, MR imaging and neuro-navigation as well as surgical adjuncts such as fluorescence- guided resection have greatly improved the safety of intracranial procedures. More recent data is suggestive of a potential survival benefit for resection of single PCNSL lesions when patients are subsequently treated with modern regimen high-dose methotrexate, yet this evidence is limited, and should be interpreted conservatively.
METHODS AND FINDINGS: A systematic review of the literature was performed to identify trials evaluating surgical options for the treatment of PCNSL.
CONCLUSION: In this review, we provide a critical overview of the evidence favoring and discouraging resection for PCNSL. This literature suffers from several biases and limitations that must be considered in the context of the extrapolation of this literature into clinical decision-making.

Tanimizu M, Kobayashi K
Neurolymphomatosis Appeared Following Primary Central Nervous System Lymphoma.
Intern Med. 2017; 56(4):463-464 [PubMed] Related Publications

Yamanaka R, Morii K, Sano M, et al.
Long-term survivors of primary central nervous system lymphoma.
Jpn J Clin Oncol. 2017; 47(2):101-107 [PubMed] Related Publications

Zeynalova PA, Tumyan GS, Dolgushin MB, Akhmedov MI
Work-up and management of a high-risk patient with primary central nervous system lymphoma.
Cancer Biol Med. 2016; 13(4):514-518 [PubMed] Free Access to Full Article Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare disorder that, in 95% of cases, represents diffuse large B-cell lymphoma. As such, making an accurate diagnosis is important. At present, stereotactic-guided biopsy is a recognized method of choice for tissue analysis. However, the diagnostic work-up for high-risk patients is determined by their performance status. Here, we report a case of PCNSL in a high-risk patient, for whom diagnosis was established by cerebrospinal fluid cytology and flow cytometry, which significantly shortened a diagnostic work-up period and allowed for the immediate treatment of the patient.

Balci MA, Pamuk GE, Unlu E, et al.
Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature.
Lupus. 2017; :961203317691370 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

Alessandro L, Pastor Rueda JM, Villalonga JF, et al.
[Retrospective study of 48 cases of primary central nervous system lymphoma].
Medicina (B Aires). 2017; 77(1):17-23 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is an infrequent form of non-Hodgkin lymphoma restricted to the CNS. More than 90% are type B and mainly affect patients aged 50-70 years. Immunodeficiency is the most important risk factor. The aim of our study was to evaluate the immune status, clinical presentation and findings in complementary studies of PCNSL patients. A retrospective analysis of 48 cases treated in our center between January 1992 and May 2015 was performed. Median age at diagnosis was 61 years (range 25-84); with male predominance (2.1:1). Forty one cases (85%) were immunocompetent patients. Brain MRI findings showed parenchymal involvement in 45 cases (94%), 43% with frontal lobe and 35% basal ganglia, 4% had meningeal involvement and 2% had ophthalmic involvement at diagnosis. Fifty-five percent had restricted signal on diffusion weighted imaging and contrast enhancement was found in 89%. Pyramidal syndrome was the main initial clinical manifestation (56%). There were abnormal findings in 62% of CSF samples, but in only 11.1% positive cytology results were detected. The most frequent type was diffuse large B-cell lymphoma (83%), being B-cell type the most common form between them (96%). In our series PCNSL was more frequent in immunocompetent elderly male subjects. At initial evaluation, clinical manifestations and MRI findings were variable. The initial suspicion of this entity would allow an early diagnosis, avoiding empirical treatments that may confuse or delay diagnosis.

Cloney MB, Sonabend AM, Yun J, et al.
The safety of resection for primary central nervous system lymphoma: a single institution retrospective analysis.
J Neurooncol. 2017; [PubMed] Related Publications
Surgical resection is not the standard of care for primary central nervous system lymphoma (PCNSL), as historical studies have demonstrated unfavorable complication rates and limited benefits. Some recent studies suggest that resection may provide a therapeutic benefit, yet the safety of these procedures has not been systematically investigated in the setting of modern neurosurgery. We examined the safety of surgical resection for PCNSL. We retrospectively analyzed all patients with PCNSL treated at Columbia University Medical Center between 2000 and 2015 to assess complications rates following biopsy or resection using the Glioma Outcomes Project system. We identified predictors of complications and selection for resection. Well-validated scales were used to quantify patients' baseline clinical characteristics, including functional status, comorbid disease burden, and cardiac risk. The overall complication rate was 17.2% after resection, and 28.2% after biopsy. Cardiac risk (p = 0.047, OR 1.72 [1.01, 2.95]), and comorbid diagnoses (p = 0.004, OR 3.05 [1.42, 6.57]) predicted complications on multivariable regression. Patients who underwent resection had better KPS scores (median 70 v. 80, p = 0.0068, ∆ 10 [0.0, 10.00]), and were less likely to have multiple (46.5% v. 27.6%, p = 0.030, OR 1.42 [1.05, 1.92]) or deep lesions (70.4% v. 39.7%, p = 0.001, OR 1.83 [1.26, 2.65]). Age (p = 0.048, OR 0.75 per 10-year increase [0.56, 1.00]) and deep lesions (p = 0.003, OR 0.29 [0.13, 0.65]) influenced selection for resection on multivariable regression. Surgical resection of PCNSL is safe for select patients, with complication rates comparable to rates for other intracranial neoplasms. Whether there is a clinical benefit to resection cannot be concluded.

Enblad G, Martinsson G, Baecklund E, et al.
Population-based experience on primary central nervous system lymphoma 2000-2012: the incidence is increasing.
Acta Oncol. 2017; :1-12 [PubMed] Related Publications
BACKGROUND: Primary central nervous system lymphomas (PCNSL) are rare lymphomas with a poor prognosis. Recently, an increased incidence has been reported. The present study is a population-based study of all patients with PCNSL in the Uppsala/Örebro region of middle Sweden.
PATIENTS AND METHODS: All patients diagnosed with a PCNSL at Uppsala University Hospital 2000-2012 were identified. Altogether, 96 patients (50 women and 46 men) were included. The median age at diagnosis was 66 years (17-95).
RESULTS: There was a statistically significant increase in age-standardized incidence during the study period, 30 patients were diagnosed in the first half and 66 in the second half of the period. No patient had an HIV-infection. Two patients had undergone kidney transplantation and were treated with immunosuppressive drugs. A high proportion of the patients, 29%, had a history of an autoimmune or inflammatory disease. The prognosis was poor with a median survival of only four months. In the 70 (73%) patients treated with curative intention the median survival was 12 months. Patients treated with high-dose methotrexate, radiotherapy and/or temozolomide appeared to have a better survival. There was no improvement in survival during the study period or after the introduction of rituximab. There also was no difference in any of the analyzed variables that could explain the increased incidence.
CONCLUSION: In this population-based study we could confirm the previously described increased incidence of PCNSL. The prognosis remains poor despite the inclusion of treatment with rituximab during the study period. A high proportion of the patients had a history of an autoimmune or inflammatory disease not previously described but there was no increase during the study period.

Debata A, Yoshida K, Ujifuku K, et al.
Hyperglycemia is associated with poor survival in primary central nervous system lymphoma patients.
Tumori. 2016; :0 [PubMed] Related Publications
PURPOSE: Primary central nervous system lymphoma (PCNSL) is a type of non-Hodgkin lymphoma (NHL), and it has been postulated that metabolic disorder may contribute to NHL etiology. We retrospectively investigated the prognostic significance of hyperglycemia in patients with PCNSL. We evaluated glucose transporter type 1 (GLUT1) expression by immunohistochemistry and analyzed its association with hyperglycemia and survival.
METHODS: The medical and neuroradiologic records of 50 patients with PCNSL at our institution over the past 15 years were analyzed. Patients were divided into 3 groups based on mean fasting plasma glucose (FPG) levels: normal (>110 mg/dL), prediabetes (110-125 mg/dL), and diabetes (≥126 mg/dL). We defined prediabetes and diabetes groups as hyperglycemia.
RESULTS: Forty-four percent of patients were in the prediabetes and diabetes groups. One-year survival rates were 73%, 66%, and 43% in normal, prediabetes, and diabetes groups, respectively. Univariate analysis revealed that high age, female sex, poor performance status, high mean FPG, and monotherapy were associated with shorter survival. Multivariable Cox regression analyses showed that high mean FPG and monotherapy were significant predictors of shorter survival (p = 0.036 and p = 0.000, respectively). The GLUT1 immunohistopathologic staining was performed in 34 cases, 20 of which (58%) showed variable levels of GLUT1 expression at the cell membrane and/or cytoplasm. Prediabetes and diabetes groups had a higher percentage of GLUT1-positive cells compared with the normal group (p = 0.015).
CONCLUSIONS: These findings indicate that hyperglycemia is associated with poor survival. The putative biological mechanism might involve differential GLUT1 expression between hyperglycemic and normal states in patients with PCNSL.

Nakamura T, Yamashita S, Fukumura K, et al.
Genome-wide DNA methylation profiling identifies primary central nervous system lymphoma as a distinct entity different from systemic diffuse large B-cell lymphoma.
Acta Neuropathol. 2017; 133(2):321-324 [PubMed] Related Publications

Schlegel U, Korfel A
The challenge of adequate imaging surveillance in primary central nervous system lymphoma.
Neuro Oncol. 2016; [PubMed] Related Publications

DU CY, Yang RY, Li C, Duan LJ
[Clinicopathological Features of Primary Central Nervous System Lymphoma and Their Influence on Prognosis of Desease].
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016; 24(6):1776-1781 [PubMed] Related Publications
OBJECTIVE: To study the clinical features of of patients with primary central nervous system lymphoma(PCNSL) and their influence on prognosis.
METHODS: Forty-two cases of PCNSL hospitallized in our hospital from January 2012 to December 2015 were selected, and the laboratory analysis, imaging examination, bone marrow analysis and pathological examination all were performed, 26 cases were treated by lumbar puncture combined with intrathecal injection of drugs (Ara C, dexamethason and methotrexate), 8 cases were treated by methotrexate combined with rituximab, 8 cases voluntanly abandon treatment after being diagnosed as PCNSL.
RESULTS: Headache accrued in 12 cases, diplopia in 2 cases, dizziness in 6 cases, limb weakness in 10 cases, amnesia in 2 cases, inhibited speech in 4 cases.Out of 42 patients 4 cases of peripheral T cell lymphoma, 38 cases of B cell lymphoma; 30 cases of multiple lesions, 12 cases of solitary lesions, 8 cases of corpus callosum, 8 cases of thalamus, 8 cases of frontal lobe, parietal lobe, temporal lobe, thalamus and other lesions, 2 cases of hydrocephalus, 2 cases of cerebral hemorrhage; and the patients HIV were negative. 8 cases erythrocyte sedimentation rate were faster, IgG, IgE and IgM levels increased to varying degrees in 42 cases, and the blood routine, liver function and blood coagulation examinations showed normal; There was no significant difference in the influence of the lumbar injection, methotrexate dose, radiation therapy, and hematopoietic stem cell transplantation on survival time(P>0.05); but there was significant difference in the influence of rituximab and number of lesions on survival time (P<0.05); there were significant differences in the effects of age and protein content in cerebrospinal fluid on therapeutic effecacy(P<0.05).
CONCLUSION: There is no significant difference in the imaging examination and clinical manifestation between PCNSL and other intracranial tumors. The lumbar puncture is an effective way to identify, the age, cerebrospinal fluid protein and the number of lesions are the adverse factors influencing the prognosis.

Fossard G, Ferlay C, Nicolas-Virelizier E, et al.
Utility of post-therapy brain surveillance imaging in the detection of primary central nervous system lymphoma relapse.
Eur J Cancer. 2017; 72:12-19 [PubMed] Related Publications
BACKGROUND: The optimal follow-up strategy for primary central nervous system lymphoma (PCNSL) patients after first-line therapy is unclear. The goal of this study is to determine the utility of planned brain surveillance imaging in the detection of relapse in a retrospective cohort of PCNSL patients.
METHODS: Patients were consecutive PCNSL cases treated in Leon Berard Cancer Centre, Lyon, France, from 1985 to 2011. Histology was diffuse large B-cell lymphoma in 94%. Patients were treated by methotrexate (92%) and cytarabine (63%) based-chemotherapy followed by radiotherapy for 108 patients (51%). Clinical records were reviewed for details at relapse and relationship to planned imaging. The imaging follow-up strategy was performed according to each treating physicians.
RESULTS: Among 209 PCNSL patients, 127 complete response patients entered in post-treatment observation and 63 (50%) subsequently relapsed. Among the 125 evaluable patients, the majority of relapses (N = 49, 80%) was asymptomatic and identified before the planned brain imaging. Surveillance imaging detected relapses before symptoms in 12 patients who entered in post-therapy observation (10%). The median number of brain imaging during the follow-up was 7 (0-13). A total of 819 MRI/CT-scan were performed leading to the detection of 12 asymptomatic relapses. The one year OS rates were 41% and 58% for symptomatic and non-symptomatic relapses, respectively (P = 0.21).
CONCLUSION: The majority of PCNSL relapses occurred outside planned follow-up with no difference in patient outcome between symptomatic and asymptomatic relapses. The role of brain imaging for the detection of relapses in the follow-up of PCNSL patients remains to be clarified.

Liu J, Wang Y, Sun X, et al.
Promoter methylation attenuates SHP1 expression and function in patients with primary central nervous system lymphoma.
Oncol Rep. 2017; 37(2):887-894 [PubMed] Related Publications
The Src homology region 2 domain-containing phosphatase-1 (SHP1) is a critical negative regulator involved in the JAK/STAT signaling pathway. The SHP1 gene has been proposed as a candidate tumor suppressor in solid and hematological malignancies and promoter methylation is an important biological process in controlling tumorigenesis. However, the detailed roles of SHP1 promoter methylation in the pathogenesis of primary central nervous system lymphoma (PCNSL) is largely unknown. In the present study, we evaluated the correlation between SHP1 expression and promoter methylation in patients with PCNSL. Thirty-three patients with PCNSL were included. We evaluated SHP1 protein expression levels by immunohistochemistry and the SHP1 promoter methylation profile by pyrosequencing analysis. For cases (n=8) with a good yield of total protein, SHP1 phosphorylation (pSHP1) and STAT3 protein expression levels were further analyzed by western blot analysis to uncover the molecular impact of SHP1 promoter methylation on downstream signaling pathways. In this study, a lower expression of SHP1 protein level was observed in 16/33 cases (48.5%) of PCNSL. SHP1 promoter methylation was predominant in 29/33 cases (87.9%) with a mean methylation level of 31.7±36.5%. The mean methylation level of the SHP1 promoter was significantly elevated in patients with a lower SHP1 protein expression, compared with those showing a higher SHP1 protein expression (50.3±38.9 vs. 14.2±24.0%, p=0.004). Further analysis showed that SHP1 protein expression was significantly decreased in patients with a higher SHP1 promoter methylation status (p=0.001), and such attenuation was correlated with a downregulation of pSHP1 (p=0.005) and an upregulation of STAT3 protein expression (p=0.020). Our data demonstrated that epigenetic alterations in the promoter region downregulated SHP1 expression in PCNSL patients. SHP1 promoter methylation was correlated with tyrosine phosphorylation and activation of transcription factor STAT3, which may contribute to the pathogenesis of PCNSL. Therapeutical regimens with epigenetic modifiers may be a potential option for patients with PCNSL.

Huang YC, Jou JR
Intravitreal injections of methotrexate in treatment of primary central nervous system lymphoma with intraocular involvement.
Kaohsiung J Med Sci. 2016; 32(12):638-639 [PubMed] Related Publications

Giulino-Roth L, Abla O, Batchelor TT
Management of primary central nervous system lymphoma in children.
Hematology Am Soc Hematol Educ Program. 2016; 2016(1):386-389 [PubMed] Related Publications
A 14-year-old boy with no significant past medical history presents with headaches and vomiting and is found to have a 2 × 3-cm left parietal lobe mass. A stereotactic biopsy reveals diffuse large B-cell lymphoma (DLBCL). Cerebrospinal fluid cytology, as well as bone marrow biopsies are negative, and a whole-body positron emission tomography/computed tomography scan does not demonstrate other areas of disease. The primary medical team asks how you would treat this patient.

Batchelor TT
Primary central nervous system lymphoma.
Hematology Am Soc Hematol Educ Program. 2016; 2016(1):379-385 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. The majority of PCNSL cases occur in the immunocompetent host, the focus of this review. The prognosis of PCNSL is inferior to that of other NHL subtypes including other organ-specific subtypes of extranodal NHL. The 5- and 10-year survival proportions for PCNSL are 29.3% and 21.6%, respectively. The diagnosis and management of PCNSL differs from that of other primary brain cancers and NHL in other parts of the body.

Malikova H, Koubska E, Weichet J, et al.
Can morphological MRI differentiate between primary central nervous system lymphoma and glioblastoma?
Cancer Imaging. 2016; 16(1):40 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare, aggressive brain neoplasm that accounts for roughly 2-6% of primary brain tumors. In contrast, glioblastoma (GBM) is the most frequent and severe glioma subtype, accounting for approximately 50% of diffuse gliomas. The aim of the present study was to evaluate morphological MRI characteristics in histologically-proven PCNSL and GBM at the time of their initial presentation.
METHODS: We retrospectively evaluated standard diagnostic MRI examinations in 54 immunocompetent patients (26 female, 28 male; age 62.6 ± 11.5 years) with histologically-proven PCNSL and 54 GBM subjects (21 female, 33 male; age 59 ± 14 years).
RESULTS: Several significant differences between both infiltrative brain tumors were found. PCNSL lesions enhanced homogenously in 64.8% of cases, while nonhomogeneous enhancement was observed in 98.1% of GBM cases. Necrosis was present in 88.9% of GBM lesions and only 5.6% of PCNSL lesions. PCNSL presented as multiple lesions in 51.9% cases and in 35.2% of GBM cases; however, diffuse infiltrative type of brain involvement was observed only in PCNSL (24.1%). Optic pathways were infiltrated more commonly in PCNSL than in GBM (42.6% vs. 5.6%, respectively, p <0.001). Other cranial nerves were affected in 5.6% of PCNSL, and in none of GBM. Signs of bleeding were rare in PCNSL (5.6%) and common in GBM (44.4%); p < 0.001. Both supratentorial and infratentorial localization was present only in PCNSL (27.7%). Involvement of the basal ganglia was more common in PCNSL (55.6%) than in GBM (18.5%); (p < 0.001). Cerebral cortex was affected significantly more often in GBM (83.3%) than in PCNSL (51.9%); mostly by both enhancing and non-enhancing infiltration.
CONCLUSION: Routine morphological MRI is capable of differentiating between GBM and PCNSL lesions in many cases at time of initial presentation. A solitary infiltrative supratentorial lesion with nonhomogeneous enhancement and necrosis was typical for GBM. PCNSL presented with multiple lesions that enhanced homogenously or as diffuse infiltrative type of brain involvement, often with basal ganglia and optic pathways affection.

Carnevale J, Rubenstein JL
The Challenge of Primary Central Nervous System Lymphoma.
Hematol Oncol Clin North Am. 2016; 30(6):1293-1316 [PubMed] Article available free on PMC after 01/12/2017 Related Publications
Primary central nervous system (CNS) lymphoma is a challenging subtypes of aggressive non-Hodgkin lymphoma. Emerging clinical data suggest that optimized outcomes are achieved with dose-intensive CNS-penetrant chemotherapy and avoiding whole brain radiotherapy. Anti-CD20 antibody-based immunotherapy as a component of high-dose methotrexate-based induction programs may contribute to improved outcomes. An accumulation of insights into the molecular and cellular basis of disease pathogenesis is providing a foundation for the generation of molecular tools to facilitate diagnosis as well as a roadmap for integration of targeted therapy within the developing therapeutic armamentarium for this challenging brain tumor.

Fastré S, London F, Lelotte J, et al.
Primary central nervous system lymphoma of T-cell origin: an unusual cause of spinal cord disease.
Acta Neurol Belg. 2016; [PubMed] Related Publications

Kim J, Kim YZ
A Case of Primary Central Nervous System Lymphoma Located at Brain Stem in a Child.
Brain Tumor Res Treat. 2016; 4(2):155-159 [PubMed] Article available free on PMC after 01/12/2017 Related Publications
Primary central nervous system lymphoma (PCNSL) is an extranodal Non-Hodgkin's lymphoma that is confined to the brain, eyes, and/or leptomeninges without evidence of a systemic primary tumor. Although the tumor can affect all age groups, it is rare in childhood; thus, its incidence and prognosis in children have not been well defined and the best treatment strategy remains unclear. A nine-year old presented at our department with complaints of diplopia, dizziness, dysarthria, and right side hemiparesis. Magnetic resonance image suggested a diffuse brain stem glioma with infiltration into the right cerebellar peduncle. The patient was surgically treated by craniotomy and frameless stereotactic-guided biopsy, and unexpectedly, the histopathology of the mass was consistent with diffuse large B cell lymphoma, and immunohistochemical staining revealed positivity for CD20 and CD79a. Accordingly, we performed a staging work-up for systemic lymphoma, but no evidence of lymphoma elsewhere in the body was obtained. In addition, she had a negative serologic finding for human immunodeficient virus, which confirmed the histopathological diagnosis of PCNSL. She was treated by radiosurgery at 12 Gy and subsequent adjuvant combination chemotherapy based on high dose methotrexate. Unfortunately, 10 months after the tissue-based diagnosis, she succumbed due to an acute hydrocephalic crisis.

Evers G, Kamp M, Warneke N, et al.
5-Aminolaevulinic Acid-Induced Fluorescence in Primary Central Nervous System Lymphoma.
World Neurosurg. 2017; 98:375-380 [PubMed] Related Publications
OBJECTIVE: Diagnosis of primary central nervous system lymphoma (PCNSL) is usually confirmed by brain biopsy and subsequent neuropathologic workup. 5-Aminolevulinic acid (5-ALA)-induced fluorescence has been established for diagnostic and therapeutic purposes in glioma treatment during the last few years and is discussed for use in other cranial tumors. Its role in diagnosis and treatment of PCNSL is still elusive.
METHODS: This retrospective study includes clinical, magnetic resonance imaging, pathologic and surgical data of selected 11 patients with PCNSL at two university hospitals within the last 4 years undergoing surgical treatment for resection because of imminent mass effect or suspected cerebral glioma. Patients received 5-ALA for fluorescence-guided resection preoperatively.
RESULTS: The 11 subjects age ranged from 59 to 81 years. Postsurgical pathologic workup revealed malignant B cell lymphoma with morphologic features of diffuse large B cell lymphoma. Eight of these 11 patients with PCNSL showed a clear fluorescence induced by 5-ALA. After surgical resection, patients were treated with combination chemotherapy regimens.
CONCLUSION: In patients with glioma, the use of 5-ALA is known to be associated with increased extent of resection and survival benefit. Our data and retrospective analysis of a larger patient cohort suggest that the use of 5-ALA in PCNSL should be included in a surgical approach, if this is reconsidered for select patients within a clinical study. In addition, even photodynamic therapy in combination with 5-ALA might be studied.

Yin L, Lin ZC, Yue JL, et al.
[Manifestations of PET/CT Imaging in Primary Central Nervous System Lymphoma].
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016; 24(5):1416-1420 [PubMed] Related Publications
OBJECTIVE: To investigate the PET/CT features of primary central nervous system lymphoma(PCNSL).
METHODS: A retrospective analysis of PET/CT imaging manifestations and clinical data was performed in 4 PCNSL cases which were confirmed by surgery or stereotactic pathologic biopsy.
RESULTS: The pathologic biopsy in 4 patients all showed diffuse large B cell lymphoma with a total of 8 lesions. All cases were performed with (18)F-FDG PET/CT imaging except 1 patient simultaneously with (18)F-FLT PET/CT imaging in the brain. Single lesion was found in 2 cases which both were located in the left frontal lobe. Multiple lesions were found in 2 cases, out of them 1 case located in the corpus callosum and the right lateral ventricle Angle, and the other 1 located in dual lateral ventricle and pineal region. In (18)F-FDG PET/CT image, 4 patients were characterized by high metabolism, even higher than the surrounding brain tissue. The patient which underwent (18)F-FLT PET/CT imaging was also presented with high metabolism and had better Target and non-target ratio(T/N). 3 cases with surrounded by edema and 2 cases with area of cystic necrosis.
CONCLUSION: PCNSL are characterized by high metabolism in (18)F-FDG PET/CT images and can better contrast with the surrounding normal brain. Combined use of other tracer such as (18)F-FLT can improve the diagnostic value of PET/CT in PCNSL.

Kuitunen H, Tokola S, Siniluoto T, et al.
Promising treatment results with blood brain barrier disruption (BBBD) based immunochemotherapy combined with autologous stem cell transplantation (ASCT) in patients with primary central nervous system lymphoma (PCNSL).
J Neurooncol. 2017; 131(2):293-300 [PubMed] Related Publications
Primary central nervous system lymphoma (PCNSL) is a rare brain tumour with a dismal prognosis. Several phase II studies with high-dose methotrexate-based regimens have shown promising early results, but in all hospital-based data published so far, the disease outcome has been poor. Patients with relapsed or refractory disease have a dismal prognosis. We performed retrospective analysis to evaluate results and tolerabilities of BBBD therapy in combination with high-dose therapy supported by autologous stem cell transplantation. We analysed 25 patients (age range: 40-71 years) who were treated in first or second line with BBBD therapy. When we started BBBD treatment, patients had relapsed or refractory PCNSL or they did not tolerate Bonn-like therapy. In recent years, some of the patients were treated in first line. We found promising response rates. Altogether 19 (76 %) of the patients achieved a complete response (CR). Two-year progression-free survival (PFS) and overall survival (OS) rates were 61 and 57 % respectively and the five-year OS was 47 %. Patients who were treated with a five-drug therapy had a very promising prognosis. The CR rate was 100 % in first-line therapy and 60 % in relapsed cases. These findings suggest that BBBD is a promising therapy for PCNSL, especially for patients in first line, but also for patients with relapsed or refractory disease after conventional chemotherapy, who commonly have a very poor prognosis. Treatment-related toxicity was generally manageable. Thus, BBBD followed by ASCT could be a treatment of choice in transplant-eligible patients with PCNSL.

Lu S, Gao Q, Yu J, et al.
Utility of dynamic contrast-enhanced magnetic resonance imaging for differentiating glioblastoma, primary central nervous system lymphoma and brain metastatic tumor.
Eur J Radiol. 2016; 85(10):1722-7 [PubMed] Related Publications
PURPOSE: The study aimed to investigate the use of dynamic contrast-enhanced magnetic resonance imaging (MRI)-derived permeability parameters for the differentiation of glioblastoma multiformes (GBMs), primary central nervous system lymphomas (PCNSLs), and brain metastatic tumors (MTs).
MATERIALS AND METHODS: Seventy-five patients with histopathologically confirmed GBMs (n=38), PCNSLs (n=16) and MTs (n=21) underwent dynamic contrast-enhanced MRIs before surgery. The volume transfer constant K(trans), the flux rate constant between extravascular extracellular space and plasma Kep, the extravascular extracellular volume Ve and the fractional plasma volume Vp were measured within the entire contrast-enhancing tumor by extended Tofts model. A one-way analysis of variance was used to compare all of the parameters among these three tumors, followed by the post-hoc test. Receiver operating characteristic curves were constructed to evaluate the diagnostic performance of the permeability parameters.
RESULTS: Mean K(trans) value and Ve value were significantly higher in PCNSLs than in GBMs (P<0.001 and P=0.011) and MTs (P<0.001 and P<0.001). No significant difference was observed in all of the permeability parameters between GBMs and MTs. According to the receiver operating characteristic analyses, both K(trans) and Ve had good diagnostic performance for discriminating between PCNSLs and GBMs (the area under the curve: 0.847 and 0.785, respectively), as well as between PCNSLs and MTs (the area under the curve: 0.851 and 0.884, respectively).
CONCLUSIONS: The K(trans) and Ve derived from dynamic contrast-enhanced MRI facilitate the differentiation of PCNSLs from GBMs and MTs.
Related: Non Hodgkin's Lymphoma

Okita Y, Narita Y, Miyakita Y, et al.
Health-related quality of life in outpatients with primary central nervous system lymphoma after radiotherapy and high-dose methotrexate chemotherapy.
Mol Clin Oncol. 2016; 5(3):179-185 [PubMed] Article available free on PMC after 01/12/2017 Related Publications
Chemoradiotherapy for primary central nervous system lymphoma (PCNSL) is associated with a considerable risk of long-term neurotoxicity. The present study aimed to assess the health-related quality of life (HRQOL) of outpatients with PCNSL who have received radiotherapy and high-dose methotrexate (HDMTX) chemotherapy, and to determine the factors that cause a decline in HRQOL and interfere with home living. A total of 37 patients were surveyed 0.9-14.2 years after their initial diagnosis and treatment. Each patient completed a multi-part HRQOL questionnaire that was used to examine the associations of HRQOL scores with leukoencephalopathy, Karnofsky performance status (KPS) scores, age, history of recurrence and HDMTX-based chemoradiotherapy. The results demonstrated that the history of recurrence, number of cycles of MTX chemotherapy and age affected the development of leukoencephalopathy. Reductions in KPS score were associated with a history of recurrence (P=0.03), but not with leukoencephalopathy (P=0.8). KPS score, leukoencephalopathy and age were significantly associated with a decline in HRQOL score. A decline in the HRQOL associated with a reduction in KPS score was also observed by multivariate analyses. Deterioration of the HRQOL among outpatients with PCNSL post-chemoradiotherapy was significantly associated with older age (≥66 years) and decreased KPS score. Older patients with a history of recurrence had a higher risk for deteriorated QOL due to development of leukoencephalopathy. Therefore, it is recommended that clinicians monitor the KPS score among outpatients with PCNSL. QOL examination for older patients with a lower KPS score was found to be particularly important for identifying any obstacles for home living.

Gupta NK, Nolan A, Omuro A, et al.
Long-term survival in AIDS-related primary central nervous system lymphoma.
Neuro Oncol. 2017; 19(1):99-108 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
BACKGROUND: The optimal therapeutic approach for patients with AIDS-related primary central nervous system lymphoma (AR-PCNSL) remains undefined. While its incidence declined substantially with combination antiretroviral therapy (cART), AR-PCNSL remains a highly aggressive neoplasm for which whole brain radiotherapy (WBRT) is considered a standard first-line intervention.
METHODS: To identify therapy-related factors associated with favorable survival, we first retrospectively analyzed outcomes of AR-PCNSL patients treated at San Francisco General Hospital, a public hospital with a long history of dedicated care for patients with HIV and AIDS-related malignancies. Results were validated in a retrospective, multicenter analysis that evaluated all newly diagnosed patients with AR-PCNSL treated with cART plus high-dose methotrexate (HD-MTX).
RESULTS: We provide evidence that CD4+ reconstitution with cART administered during HD-MTX correlates with long-term survival among patients with CD4 <100. This was confirmed in a multicenter analysis which demonstrated that integration of cART regimens with HD-MTX was generally well tolerated and resulted in longer progression-free survival than other treatments. No profound differences in immunophenotype were identified in an analysis of AR-PCNSL tumors that arose in the pre- versus post-cART eras. However, we detected evidence for a demographic shift, as the proportion of minority patients with AR-PCNSL increased since advent of cART.
CONCLUSION: Long-term disease-free survival can be achieved in AR-PCNSL, even among those with histories of opportunistic infections, limited access to health care, and medical non-adherence. Given this, as well as the long-term toxicities of WBRT, we recommend that integration of cART plus first-line HD-MTX be considered for all patients with AR-PCNSL.

Alvarez-Pinzon AM, Wolf AL, Swedberg H, et al.
Primary Central Nervous System Lymphoma (PCNSL): Analysis of Treatment by Gamma Knife Radiosurgery and Chemotherapy in a Prospective, Observational Study.
Cureus. 2016; 8(7):e697 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare cancer accounting for less than 3% of primary brain and central nervous system (CNS) tumors. Tissues involved include the brain parenchyma, leptomeninges, eyes, and spinal cord. High-dose methotrexate (MTX) is the gold standard for newly diagnosed PCNSL. However, Gamma Knife radiosurgery (GKRS) may be efficacious as a co-adjuvant treatment. The purpose of this prospective observational cohort study is to determine the effectiveness of MTX in combination with GKRS in the treatment of PCNSL.
METHODS: This is a prospective, observational cohort study evaluating the treatment of histologically confirmed PCNSL with MTX as a single agent in a dose of 8 g/m2 (control) and treatment with MTX, plus GKRS. Strict inclusion and exclusion criteria were employed. Primary outcomes were measured by survival rate. Secondary outcomes were assessed by the tumor's responsiveness to treatment and reduction in size as noted on imaging.
RESULTS: Between January 2007 and January 2012, 128 charts were evaluated. Included in this evaluation were 73 chemotherapy (control) and 55 chemotherapy, plus GKRS, patients (variable). The follow-up period was 24 to 49 months (mean: 36.9 months). There were no statistically significant differences in patient demographics or histology diagnosis. Patients were treated with GK doses ranging from 11 Gy to 16 Gy (median: 11 Gy). The median survival rate from initial diagnosis was 26.8 months in the chemotherapy group and 47.6 in the chemotherapy, plus GKRS, group (p-value: 0.0034). All lesions showed a complete response after GKRS when evaluated using magnetic resonance imaging after three to eight weeks (mean range: 6.3 weeks).
CONCLUSIONS: The use of GKRS is non-invasive, safe, and shows rapid success, improving the prognosis of the patient. This noninvasive treatment modality should be considered as an option for patients with PCNSL. In our study, GKRS as a co-adjuvant therapy to high-dose methotrexate was statistically significant for greater tumor control, enhanced overall survival period, and a lesser number of complications.

Fu L, Chen L, Wei N, et al.
[Analysis of the Clinical Characteristics and Prognostic Factors of 31 Cases of Primary Central Nervous System Lymphoma].
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2016; 24(4):1061-5 [PubMed] Related Publications
OBJECTIVE: To investigate the clinical characteristics, therapeutic outcomes and prognostic factors of primary central nervous system lymphoma (PCNSL).
METHODS: Clinical records of 31 cases of PCNSL were collected, the clinical charactenstics were analyzed retrospectively. Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous variables was assessed via the Cox proportional hazard model.
RESULTS: The median age was 52 years, with a ratio of male to female 1:1. Headache/dizzy/limb numbness were the most common presentation and the lesions of PCNSL were primarily located at the frontal, parietal, temporal lobes and corpus callosum. All the cases were pathologically diffuse large B cell lymphoma (DLBCL), 6 cases were the type of germinal center (GC) and 25 cases of non-GC type, after craniotomy operation and biopsy. Among 31 cases, 17 patients accepted the combined treatment, 3 patients underwent simple chemotherapy, 8 patients received simple radiotherapy, the other patients accepted support therapy. The median follow-up for surviving patients was 24 months. The 1-, 3-, and 5-year survival rates were 80.6%, 55.1%, and 36.4%, respectively. The median overall survival time in the combined treatment group was significantly longer than that in simply radiotherapy. There was no significant difference in OS between the groups with and without rituximab. ECOG PS≥2 and elevated serum LDH predicted inferior survival.
CONCLUSION: The clinical manifectation of PCNSL is various and complicated, and for the time being there is no optimal treatment scheme. The overall survival time of the combined treatment is longer than that in simply radiotherapy. ECOG PS≥2 and elevated serum LDH often are poor prognostic factors.

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