Research progress on prognostic factors of primary central nervous system lymphoma / 中国输血杂志

Primary central nervous system lymphoma (PCNSL) is a rare and special kind of non-Hodgkin lymphoma. Chemotherapy based on high-dose methotrexate (HD-MTX) is considered a standard therapy for newly diagnosed PCNSL, but the prognosis of PCNSL is poor due to its high invasiveness and recurrence rate. Risk stratification and prognosis assessment of PCNSL in diagnosis is particularly important in clinical treatment. At present, MSKCC (the Memorial Sloan Kettering Cancer Center), the International Extranodal Lymphoma Study Group (IELSG) and Nottingham/Barcelona scoring models are mostly used to classify the risk and evaluate the prognosis of PCNSL. With the further understanding of immunohistochemistry and molecular genetics of PCNSL, potential prognostic factors continue to emerge.

Prospective study of preliminarily diagnosed primary central nervous system lymphoma treated with an R-FPD regimen / 中国肿瘤临床

Objectives: To evaluate the safety, efficacy, and feasibility of using rituximab in combination with fotemustine, pemetrexed, and dexamethasone (R-FPD) in primary central nervous system lymphoma (PCNSL). To undertake an initial exploration of the significance of biomarkers in PCNSL. Methods: This was a prospective, single-center, single-arm, phase Ⅱ clinical trial. Patients newly diagnosed with PCNSL from the First Affiliated Hospital of Zhengzhou University from July 2018 to July 2019 were enrolled. All patients underwent first-line chemotherapy with R-FPD. The primary study endpoints were: objective response rate (ORR), disease control rate (DCR), progression-free survival (PFS), and overall survival (OS). The secondary study endpoint was: adverse reactions (ADR). Results: Twelve patients were included in the study. After four cycles of treatment, six patients had complete remission, two had partial remission, one was stable, and three progressed. The ORR was 66.7%, DCR was 75%, and median PFS was 7 months (95% confidence interval: 4.4-9.6 months). The median OS was 10.5 months (95% confidence interval: 6.1-14.9 months). The main adverse reaction due to R-FPD chemotherapy was hematologic toxicity, and grade III- neutropenia and thrombocytopenia were observed (16.7% and 25%, respectively). High expression of c-myc protein (>40%) did not appear to be significantly associated with prognosis. Conclusions: R-FPD is an effective and safe protocol for the treatment of newly diagnosed PCNSL patients. There was no significant relationship between high c-myc expression and prognosis.

Primary hypothalamic lymphoma with extensive ventricular enhancement and hydrocephalus

@#Primary central nervous system lymphoma (PCNSL) is an aggressive malignancy arising exclusively in the central nervous system (CNS). PCNSL represents 1-5% of intracranial neoplasms and approximately 1% of all non-Hodgkin lymphomas.1,2 The clinical manifestation varies, depending on the location and size of the tumor. Biopsy is usually required in the diagnosis of PCNSL, and chemotherapy and/or radiotherapy were the most commonly used therapy.3 Here we report a rare case of primary hypothalamic lymphoma with extensive ventricular enhancement and hydrocephalus

Analysis of prognostic factors of the first relapsed/refractory primary central nervous system lymphoma / 中国肿瘤临床

Objective: To analyze the clinical characteristics of patients with relapsed/refractory primary central nervous system lym-phoma (PCNSL) and to explore the factors that influence the prognosis, in order to provide evidence for the clinical diagnosis and treat-ment. Methods: Sixty-four patients with relapsed/refractory PCNSL diagnosed from October 2006 to August 2015 were selected. The clinical features, treatment plans, and laboratory examination data were retrospectively analyzed. Cox regression was used for multi-variate analysis. Results: Univariate and multivariate analyses showed that progression-free survival of first time (PFS1)≤1 year and Kar-nofsky performance status (KPS) score<70 points were independent prognostic factors in patients with first relapsed/refractory PCNSL. The median PFS2 and overall survival of second time (OS2) were 19 and 21 months, respectively, in patients with PFS1≥1 year, where-as the median progression free survival of second time (mPFS2) and OS2 were 10 and 14 months, respectively, in patients with PFS1<1 year. The median PFS2 (mPFS2) in patients with first relapse/refractory KPS score≥70 points and those with KPS score<70 points were 40 and 10 months, respectively, and the median OS2 were 43 and 12 months, respectively. The median PFS for the methotrexate (MTX) and non-MTX groups was 18 and 10 months, respectively. Multivariate analysis showed that the salvage therapy was a relevant factor influencing the patient's PFS. However, univariate analysis showed that the median OS2 in the MTX and non-MTX groups was 23 and 12 months, respectively, with significant difference but without any correlation with prognosis. Conclusions: progression-free sur-vival (PFS)≤1 year and KPS score<70 were independent prognostic factors in patients with first relapsed/refractory PCNSL. Patients with relapsed/refractory PCNSL who continuously received high-dose MTX-based treatment may have improved long-term treatment outcomes.

Differentiation between Glioblastoma and Primary Central Nervous System Lymphoma Using Dynamic Susceptibility Contrast-Enhanced Perfusion MR Imaging: Comparison Study of the Manual versus Semiautomatic Segmentation Method

BACKGROUND: Normalized cerebral blood volume (nCBV) can be measured using manual or semiautomatic segmentation method. However, the difference in diagnostic performance on brain tumor differentiation between differently measured nCBV has not been evaluated. PURPOSE: To compare the diagnostic performance of manually obtained nCBV to that of semiautomatically obtained nCBV on glioblastoma (GBM) and primary central nervous system lymphoma (PCNSL) differentiation. MATERIALS AND METHODS: Histopathologically confirmed forty GBM and eleven PCNSL patients underwent 3T MR imaging with dynamic susceptibility contrast-enhanced perfusion MR imaging before any treatment or biopsy. Based on the contrast-enhanced T1-weighted imaging, the mean nCBV (mCBV) was measured using the manual method (manual mCBV), random regions of interest (ROIs) placement by the observer, or the semiautomatic segmentation method (semiautomatic mCBV). The volume of enhancing portion of the tumor was also measured during semiautomatic segmentation process. T-test, ROC curve analysis, Fisher's exact test and multivariate regression analysis were performed to compare the value and evaluate the diagnostic performance of each parameter. RESULTS: GBM showed a higher enhancing volume (P = 0.0307), a higher manual mCBV (P = 0.018) and a higher semiautomatic mCBV (P = 0.0111) than that of the PCNSL. Semiautomatic mCBV had the highest value (0.815) for the area under the curve (AUC), however, the AUCs of the three parameters were not significantly different from each other. The semiautomatic mCBV was the best independent predictor for the GBM and PCNSL differential diagnosis according to the stepwise multiple regression analysis. CONCLUSION: We found that the semiautomatic mCBV could be a better predictor than the manual mCBV for the GBM and PCNSL differentiation. We believe that the semiautomatic segmentation method can contribute to the advancement of perfusion based brain tumor evaluation.

A clinicopathological study of primary central nervous system lymphomas & their association with Epstein-Barr virus.

Background & objectives: Primary central nervous system lymphomas (PCNSLs) are relatively uncommon, accounting for 2-3 per cent of primary brain tumours. Majority of these are diffuse large B cell lymphomas (DLBCL) occurring both in immunocompromised and immunocompetent patients. We undertook this study to classify PCNSL into germinal centre (GC) and non-germinal centre (NGC) type based on Hans classification and to find the role of Epstein-Barr virus (EBV) in pathogenesis both by conventional immunohistochemistry (IHC) and chromogenic in situ hybridization (CISH). Methods: The consecutive cases of PCNSL during a 10 years period were analysed by IHC for CD45, CD20, CD3, B-cell lymphoma 2 and 6 (Bcl-2 and Bcl-6), B-cell specific octamer binding protein-1 (BOB-1), multiple myeloma oncogene-1 (MUM-1), EBV latent-membrane protein 1 (LMP-1), cyclin-D1, CD10, CD5 and CD23, as well as by CISH for EBV. Results: During a period of 10 years, 65 PCNSL were diagnosed which comprised 0.69 per cent (65/9476) of all intracranial tumours. The mean age of presentation was 49 yr with sex ratio (M:F) of 1.4:1. Most common location was supratentorial region with predominant involvement of frontal lobe. Single lesions were seen in 38 (58.4%) and multifocal lesions in 27 (41.5%) patients. None of the patients were immunocompromised. All cases were B cell immunophenotype and were DLBCL except one case of follicular lymphoma. According to Hans classification, majority of them were NGC (n=51, 79.6%) and 13 (20.3%) were GC type. Bcl-2 expression was noted in 34 (52.3%) tumours. EBV was positive in three (4.6%) cases; two were detected both by IHC and CISH and one case by CISH only. Interpretation & conclusions: In Indian population, PCNSL occurs mainly in immunocompetent patients, and a decade earlier than in western population. Immunophenotyping revealed that all cases were DLBCL with predominance of NGC type. No prognostic difference was seen between GC and NGC DLBCL. Association of EBV was rare and this virus was possibly not involved in the pathogenesis of PCNSL in immunocompetent individuals. CISH was an easy, economical and less cumbersome method for detection of EBV in PCNSL.

Advances in the treatment of primary central nervous system lymphoma / 中国肿瘤临床

Primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphoma. Its pathological type mainly includes dif-fuse large B cell lymphoma. In MRI images, the condition is usually manifested as solitary deep lesions in brain parenchyma, with vari-ous forms of reinforcement. To date, chemotherapy based on high-dose methotrexate administration is the first-line therapy of PCNSL. Combined radiotherapy and chemotherapy can prolong the overall survival of patients. However, this approach simultaneously causes higher incidence of neurotoxicity. High-dose chemotherapy followed by autologous stem cell transplantation is recommended for re-current and refractory PCNSL. Temozolomide and rituximab can be used as treatment options because of their low toxicity and good tolerance. The prognosis of PCNSL depends on several factors, such as serum LDH concentration, age, ECOG/KPS score、cerebro-spi-nal fluid (CSF) protein concentration and tumor location.

Primary Central Nervous System Lymphoma Mimicking Behcet's Disease

No abstract available.

Temporary Spontaneous Remission in Primary CNS Lymphoma

Spontaneous remission in untreated primary central nervous system lymphoma is rare. A 66-year-old man was admitted with dizziness and gait disturbance. Initial fluid-attenuated inversion-recovery images revealed hyperintensities in the upper brainstem, left temporal lobe, and right occipital lobe. The patient's symptoms and lesions disappeared spontaneously after 1 month. However, he was readmitted after 4 months with right hemiparesis. Magnetic resonance imaging revealed a homogenous enhanced lesion in the left basal ganglia with a vasogenic pattern. This disease warranted biopsy, which revealed large B-cell lymphoma.

Primary Central Nervous System Lymphoma: A Notable Differential Diagnosis of Leukoencephalopathy

We report a 61-year-old man presented with hyperventilation in the absence of alteration of consciousness. Brain MRI showed diffuse T2-high signal intensities in the white matter of the bilateral hemispheres and brainstem with some focal enhancements without mass effect or edema. Methionine PET demonstrated hypermetabolism in the right thalamus and cerebellum. Primary central nervous system lymphoma (PCNSL) was confirmed pathologically by stereotaxic biopsy. PCNSL can take the form of leukoencephalopathy in MRI, and methionine PET would be helpful for the differentiation of PCNSL from non-tumorous conditions.