Clinicopathological Features, Survival And Prognostic Factors Of Primary Central Nervous System Lymphomas: Trends in Incidence of Primary Central Nervous System Lymphomas and Primary Malignant Brain Tumors in A Well-Defined Geographical Area; Population-Based Data from the Danish Lymphoma Registry, Lyfo, And the Danish Cancer Reistry

It has been claimed that Primary Central Nervous System Lymphoma! (PCNSL), a rare neoplasm accounting for only a small fraction of malignant brain tumors and extrancdal non-Hcdgkin lymphomas (NHL), occur with increasing frequency in immunologically normal as well as in immunocompromised individuals. In an attempt to characterize the clinicopathological features, outcome and prognostic factors of PCNSL we here report our experience in a large unselected series of patients from a well-defined region. In addition, we present data on trends in incidence of PCNSL and primary malignant brain tumors in a well-defined geographical area. In a Danish population-based NHL registry (LYFO) representing a population of 2.7 million all new cases of NHL were registered during the approximate I I-year period from 1st January 1983 to 3 1st May 1994. Incidence data of primary malignant tumors of the brain and central nervous system in western Denmark for the period 1971-1990 have been obtained from the Danish Cancer Registry. During the approximate I I-year period 3 124 new cases of NHL were registered. Of these, I 152 (37%) were extranodal and 48 were non-AIDS related PCNSL accounting for4.2% ofextranodal NHL and 1.5% of all NHL, respectively. The average annual incidence rate of non-AIDS related PCNSL during the period was I .56 cases per million population (age range: 15-85 yrs, median: 62 yrs, MIF ratio: I).In a 23-year period there was no trend towards an increasing incidence of non-AIDS related PCNSL in a well-defined population. PCNSL accounted for 1.7% of all primary malignant brain tumors. Incidence of primary malignant brain tumors was stable, except for age ranges over 70 years. However, diagnostic artifacts might be responsible for this apparent increase. Histologically, 85% were high grade. Using the Kiel classification centroblastic diffuse (60%) and immunoblastic lymphoma (13%) were the most common subtypes. Forty-three patients had 6-cell lymphoma and no T-cell lymphoma was detected. Forty-seven cases were diagnosed pre mortem. Treatment included surgical resection (26 patients), whole brain irradiation (WBRT) (43 patients) and chemotherapy (28 patients). Median survival for those receiving either WBRT or WkRT and chemotherapy was 8 months and 20 months, respectively (p = 0.78). Overall survival was 53%. 38% and 26% at I, 2 and 5 years. Cox-regression analysis identified only one factor having independent impact on survival in PCNSL performance score 2 2 (pc 0.001. RR = 5.8     

Increasing incidence and continued dismal outcome of primary central nervous system lymphoma in Norway 1989-2003 : time trends in a 15-year national survey

BACKGROUND: The incidence of primary central nervous system lymphoma (PCNSL) appears to be increasing in some countries, whereas it is stable in others. Many reports the last decades have suggested that there have been improvements in the treatment of PCNSL. The objective of this study was to analyze time trends in the incidence, clinical features, histologic diagnosis, treatment, and outcome of nonacquired immunodeficiency syndrome (non-AIDS) PCNSL in Norway from 1989 to 2003. METHODS: Patients were identified by a chart review of all patients who had a recorded diagnosis of PCNSL from 1989 to 2003 in The Norwegian Cancer Registry. The histologic and cytologic material from each patient was re-examined by pathologists. Time trends were analyzed according to year of diagnosis grouped into 3 5-year periods: 1989-1993, 1994-1998, and 1999-2003. RESULTS: There were 98 patients who had confirmed, newly diagnosed non-AIDS PCNSL in Norway from 1989 to 2003. The incidence rate increased during the consecutive 5-year periods from 0.89 per million during 1989 to 1993, to 1.74 per million during 1994 to 1998, and to 1.82 per million during 1999 to 2003 (P = .013). Diagnostic delay and overall survival did not improve with time. Survival decreased from 1999 to 2003 compared with survival from 1994 to 1998, which was explained in part by reduced performance status and fewer patients receiving combined chemotherapy and radiotherapy during 1999 to 2003. In multivariate analysis, age 相似文献   

Primary central nervous system lymphomas: incidence and survival in the Southern and Eastern Netherlands

BACKGROUND: An excessive increase in the incidence of primary central nervous system lymphoma (PCNSL) has been reported since the mid-1980s in the U.S. and U.K. Clinical studies have shown that radiotherapy and chemotherapy may prolong survival. In the current study, the authors describe the incidence, treatment, and survival of an unselected group of patients with PCNSL in the southern and eastern Netherlands. METHODS: Data regarding patients diagnosed between 1989-1994 were obtained from 4 population-based regional cancer registries in the southern and eastern Netherlands (n = 86) and the Eindhoven Cancer Registry for 1980-1988 (n = 6). Lymphomas were registered as PCNSL when a tissue diagnosis of CNS lymphoma was established for a patient with neurologic symptoms (i.e., lymphomas were not necessarily restricted to the CNS at the time of diagnosis). Only patients diagnosed during their lifetime with Stage I disease, Stage "IV" disease (i.e., diffuse CNS lymphoma), or disease of unknown stage were included (63 patients, 8 patients, and 15 patients, respectively, between 1989-1994). For 80 patients (93%) follow-up was complete until January 1, 1997. RESULTS: Between 1989-1994, an average World Standardized Rate of 2.3 cases and 1.7 cases per 1 million person-years, respectively, was reported for males and females. The median age of the patients at the time of diagnosis was 62 years, and was 66 years for patients with an unknown disease stage. In the area of the Eindhoven Cancer Registry the occurrence of PCNSL more than doubled from < 2% of all histologically confirmed primary CNS malignancies diagnosed between 1980-1985 to approximately 4% of cases diagnosed between 1986-1994. The median survival of all the patients was 4.1 months; the median survival was 5.8 months for patients with limited (Stage I and Stage IV) disease and was 0.6 months for patients with an unknown stage of disease. Approximately 65% of the patients with limited disease received radiotherapy and approximately 35% of such patients received chemotherapy. Furthermore, chemotherapy was given more often to patients age < 60 years who tended to have a slightly better survival than patients age > or = 60 years. CONCLUSIONS: The increase in the incidence of PCNSL in the 1980s may be explained in large part by changes in diagnostics and registration. The relatively high incidence and low survival rate of PCNSL in the southern and eastern Netherlands reported in the 1990s may be due in part to the inclusion of patients with systemic lymphoma and immunodeficiency disorders. However, a significant improvement in the prognosis of patients with PCNSL in the southern and eastern Netherlands diagnosed in the 1990s is unlikely.     

Incidence and radiographic findings of primary central nervous system lymphoma in immunocompetent patients in southern Thailand

AIMS AND BACKGROUND: In the last two decades there have been conflicting reports concerning a rising incidence of primary central nervous system lymphoma (PCNSL) in immunocompetent patients. This study is being conducted to review the incidence and radiographic findings of PCNSL in a large tertiary care institution in southern Thailand. PATIENTS: A review was carried out in Songklanagarind University Hospital of the clinical records, CT and MRI images of immunocompetent patients who had histologically proven PCNSL diagnosed between January 1993 and December 2004. RESULTS: A total of 25 PCNSL patients were diagnosed over a 12-year period. The incidence trend was rising but not to a statistically significant extent. The median age at diagnosis was 57.4 years. Based on the Working Formulation classification, diffuse large cell was the most common subtype. All of the 20 patients with available immunohistochemical results had B-cell lymphomas. The radiographic findings in our series show that the majority of patients had a single lesion (60%) at a supratentorial location (50%). Most of the lesions were well circumscribed. Based on the density of the lesions before administration of a contrast medium, 56% of the lesions on CT images appeared hyperdense. On T1-weighted MRI images, the lesions of our patients were most frequently hypointense (67%) while the T2-weighted images were more commonly hyperintense (60%) and contrast enhancement was found in all lesions. There were 21 patients who received whole brain radiotherapy with doses ranging between 4.2 and 6.0 Gy. The median survival time was 14.4 months. CONCLUSIONS: This study indicates that there has been no significant increase in PCNSL cases over the last 12 years. The recognition of characteristic imaging features of PCNSL may facilitate a stereotactic procedure before steroid administration. In addition, we provide evidence that radiotherapy alone is insufficient in PCNSL.     

Trends in survival from primary central nervous system lymphoma, 1975-1999: a population-based analysis

BACKGROUND: The age-adjusted incidence of primary central nervous system lymphoma (PCNSL) has increased since the 1970s, and treatment for this disease has evolved considerably. The objective of this study was to examine time trends in overall survival and disease-specific mortality in a population-based cohort of patients with PCNSL. METHODS: We identified patients diagnosed with PCNSL from 1975-1999 in the Surveillance, Epidemiology, and End Results (SEER) cancer registries. To assess time trends, year of diagnosis was classified in 5-year intervals: 1975-1980, 1981-1985, 1986-1990, 1991-1995, and 1996-1999. Overall survival distributions were estimated via Kaplan-Meier methodology and a competing risk analysis was used to assess PCNSL-specific mortality. We used information on underlying cause of death to distinguish likely immunocompetent patients from those whose PCNSL was related to human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS). We also examined survival stratified by age at diagnosis. RESULTS: From 1975-1999, 2462 patients were diagnosed with PCNSL in SEER. Median survival was 4 months (95% CI 4, 5) for the entire cohort and 9 months (95% CI 8, 11) for the immunocompetent cohort (n = 1565). In the immunocompetent cohort, 965 of 1323 (73%) deaths were attributed to PCNSL. No significant time trend was observed in either overall or PCNSL-specific survival. CONCLUSIONS: Overall survival for patients with PCNSL has not improved consistently in the past three decades despite important therapeutic advances during this time. Although results from clinical trials suggest progress in the treatment of PCNSL, survival improvements are not reflected in this population-based cohort.     

Natural history and prognostic factors for survival in patients with acquired immune deficiency syndrome (AIDS)-related primary central nervous system lymphoma (PCNSL)

The incidence of primary central nervous system lymphoma (PCNSL) is increasing rapidly. It will be the most common primary malignant neoplasm of the brain by the year 2000. PCNSL is an important lethal complication in acquired immunodeficiency syndrome (AIDS) patients. Our objective was to study the natural history and prognostic factors for survival in patients with AIDS-related PCNSL. This is a retrospective cohort study of 75 patients with the diagnosis of AIDS-related PCNSL followed at Jackson Memorial Hospital/University of Miami. Medical records were abstracted for information about age, gender, race, and ethnicity. The method of diagnosis, treatment, and outcome of AIDS and PCNSL in this group were examined. Univariate and multivariate analyses were performed to identify prognostic factors for survival. The median age was 37 years. Males comprised 84% of the patients and 55% of the patients were Hispanic. The most common human immunodeficiency virus (HIV) risk factors were homosexuality and multiple sexual partners. The median cluster designation (CD) 4 count was 15/microl and the median lactic dehydrogensase (LDH) was 1.5x normal. Computed-assisted tomographic (CT) scans of the brain showed multiple lesions in 44% of the patients. Single-photon emission CT scan (SPECT) Thallium-201 of the brain was performed in two-thirds of patients. The most common histologies were immunoblastic and large cell lymphoma. Cranial radiation was given to 72% of the patients, and 55% of them did not complete treatment. The median survival of the group was 1.3 months. Univariate and multivariate analysis showed that longer survival was associated with good performance status (ECOG = 1 to 2 vs. 3 to 4). The presence of prior opportunistic infections, risk factors for AIDS, CD4 counts, level of LDH, ethnicity, gender, duration of symptoms before diagnosis, and race did not influence survival. PCNSL is a neoplasm with a very poor prognosis and short survival even with CNS radiation therapy. Performance status appears to be the main prognostic factor for survival. No significant differences in presentation or outcome were detected between the Hispanic and non-Hispanic patients.     

Changing incidence and survival in patients with aids-related non-Hodgkin's lymphomas in the era of highly active antiretroviral therapy (HAART)

To determine role of highly active antiretroviral therapy (HAART) and additional factors in incidence and outcome of patients with AIDS-related non-Hodgkin's lymphomas (NHL) we retrospectively analyzed 257 cases of AIDS-related NHL (24 low-grade, 168 high-grade B-cell, 6 high-grade T-cell, and 59 primary CNS lymphomas (PCNSL) among 2004 patients with HIV-infection treated at the University Hospital of Frankfurt, Germany from January 1983 to May 1999. Data were evaluated by univariate and multivariate analyses, using overall survival as end point. Patients received CHOP-like therapy as standard treatment. Until May 1999 incidence of all diagnosed cases of NHL was decreasing (1991-94: 14.2% versus 1995-5/99: 12.8%). Mainly, the incidence of low-grade NHL and PCNSL clearly decreased whereas the incidence of high-grade B-cell NHL increased compared to all diagnosed cases of NHL (1983-86: 53.3% versus 1995-5/99: 78.6%). One-year survival probability of all screened patients with AIDS related NHL was 54%, while 5-year survival rate remained 5%. We found age <25 years, development of NHL in the years before 1990, IVDU, CD4 counts <150/microl, PCNSL as well as NHL as the AIDS index disease, to be highly significant independent predictors of poor survival, including increased hazard ratios. In the era of HAART incidence of NHL is decreasing, mainly the incidence of low-grade NHL and PCNSL. Overall survival of patients has been prolonged with HAART. This development is mainly due to improvement of antiretroviral therapy, rather than to any fundamental changes in the chemotherapeutic treatment of NHL. Therefore, new treatment approaches for AIDS-related NHL should focus on more efficient antiretroviral therapy in association with combination chemotherapy.     

Epstein–Barr virus (EBV)-associated primary central nervous system lymphoma: is incidence of EBV expression associated with median survival time?

The frequency and clinical features of Epstein–Barr virus (EBV)-associated primary central nervous system lymphoma (PCNSL) in elderly patients were investigated in this study. Thirty-three PCNSL cases were enrolled in the retrospective study. Biopsies were performed, and tissue was embedded in paraffin and sectioned. In-situ hybridization of EBV-encoded small RNAs was then conducted. Specimens were scored as having one of three possible results: negative (no EBV-positive cells), slightly positive (<50% EBV-positive cells), and strongly positive (>50% EBV-positive cells). Fifteen cases were negative for EBV expression. Sixteen cases were slightly positive, and two cases (68 and 79 years of age) were strongly positive. The incidence of strongly positive EBV expression in PCNSL was 6.1%. The incidence of strongly positive EBV expression in PCNSL patients ≥65 years of age was 13%. Median survival time differed significantly among PCNSL patients treated with high-dose methotrexate and radiotherapy. Importantly, the strongly EBV-positive PCNSL cases had the worst outcomes, and the EBV-negative PCNSL cases had the best outcomes. These results suggest that EBV infection may affect the treatment outcome of PCNSL. In the future, examination of EBV expression in PCNSL patients who receive individualized treatment may be useful.     

原发性中枢神经系统淋巴瘤的临床特征分析

 目的　分析原发性中枢神经系统淋巴瘤（PCNSL）的临床特征,探讨影响疾病的预后因素,并对不同的治疗方案进行评价。方法　回顾性分析初发PCNSL患者的临床资料、治疗经过及随访结果,应用Log-rank进行单因素分析,应用COX回归模型进行生存资料的多因素分析。结果　共收集PCNSL初发病例64例,中位年龄54.9岁,男性多于女性,肿瘤单发62 %（40／64）,深部病变占54 %（33／61）。在我科诊治的具有完整治疗资料的患者26例,其中19例患者初始治疗为单纯化疗,6例为全颅放疗（WBRT）后1个月进行化疗,1例患者初治时仅行WBRT。中位生存时间为17个月,血红蛋白≥9 g／L患者的生存时间长于血红蛋白＜9 g／L患者。年龄>60岁、性别、体能状态、病变部位等因素对预后无明显影响。应用含大剂量甲氨蝶呤（HD-MTX）或替尼泊苷的方案化疗者的预后优于未使用者,化疗联合放疗可能有助于改善患者的预后（χ2＝3.24,P＝0.07）,应用CHOP方案（环磷酰胺、多柔比星、长春新碱、泼尼松）、利妥昔单抗、鞘内注射化疗药物等与预后关系不大;多因素分析提示HD-MTX是影响PCNSL患者生存时间的独立有利因素,颅内病灶部位、病灶的多少、是否联合放化疗等均不是影响预后的独立因素。结论　PCNSL预后较差,应用HD-MTX、替尼泊苷等药物可改善患者的预后,贫血尤其中重度贫血患者预后不良。     

Immunohistochemistry for c-myc and bcl-2 overexpression improves risk stratification in primary central nervous system lymphoma

Overexpression of bcl-2 and c-myc are defining features of double-expressor-lymphoma (DEL) but may also occur separately in patients with primary central nervous system lymphoma (PCNSL). Despite all progress in optimizing treatment regimen, there is lack of sufficient risk stratification models. Here, we first describe the relationship between DEL biology, the National Comprehensive Cancer Network International Prognostic Index (NCCN-IPI), treatment response, disease progression, and mortality in PCNSL. In this study, we determined c-myc and bcl-2 status immunohistochemically in samples of 48 patients with newly diagnosed PCNSL and followed these patients for a median interval of 6.2 years. Twelve, 18, and 17 patients harbored none, one, or both DEL features. Corresponding overall response rates after first-line therapy were strongly associated with DEL biology (100%, 42%, and 44% in patients with 0, 1, or 2 DEL features). Patients with one or both DEL features had a 5-fold and 13-fold higher 5-year risk of progression and/or death than patients without DEL features. These associations prevailed after adjusting for the NCCN-IPI. DEL improved the discriminatory capability of the NCCN-IPI (P = .0001). Furthermore, we could show that addition of DEL biology to the NCCN-IPI significantly improved the score's discriminatory potential both toward progression-free survival (increase in Harell's c = 0.15, P = .005) and overall survival (increase in Harell's c = 0.11, P = .029). In conclusion, DEL biology is a strong and simple-to-use predictor of adverse outcome in PCNSL. Addition of DEL to the NCCN-IPI improves its prognostic potential. Disease progression from PCNSL harboring both DEL features is invariably fatal. This defines a novel PCNSL patient subset with a great unmet need for improved therapy.     

Human immunodeficiency virus-related primary central nervous system lymphoma: factors influencing survival in 111 patients

BACKGROUND: The current study evaluated factors influencing survival in patients diagnosed with human immunodeficiency virus (HIV)-related primary central nervous system lymphoma (PCNSL), with a focus on the effects of therapeutic radiotherapy (RT) and highly active antiretroviral therapy (HAART). METHODS: A retrospective chart review of patients with a diagnosis of HIV-related PCNSL at one of five university hospitals between 1987 and 1998 was performed. Clinical details including antiretroviral agent use, brain imaging scan results, RT use, and survival outcomes were recorded. RESULTS: One hundred eleven patients with HIV-related PCNSL were identified. The annual incidence decreased significantly between 1992 and 1995 and between 1996 and 1998 (P = 0.04). The median survival period was 50 days (mean, 109 days; range, 4-991 days), with improved survival for patients diagnosed after 1993. Patients treated with two or more antiretroviral agents had improved survival (P = 0.01), as did patients who received RT (P < 0.0001). For patients who received RT, completion of the prescribed course and treatment to > or = 30 Gray (Gy) independently predicted a more favorable outcome. RT used in conjunction with antiretroviral therapy involving two or more agents had an additive positive effect on survival. For patients who did not receive RT, poor performance status and encephalopathy predicted a shorter survival duration. CONCLUSIONS: The results of the current study suggest that HAART and treatment with RT to > or = 30 Gy improve survival for patients with HIV-related PCNSL. This combination of therapies may provide a standard of care as the basis for further trials of chemotherapy, novel adjunctive treatment, and quality of life assessment.     

Incidence of and survival from Wilms' tumour in adults in Europe: data from the EUROCARE study

Wilms' tumour, or nephroblastoma, is an embryonal cancer of the kidney that occurs mainly in young children. This is a very rare tumour among adults, with an incidence rate of less than 0.2 per million per year. The aims of this study were to report the survival of adults diagnosed with nephroblastoma in Europe and to analyse time trends and geographic variations in survival. All the adults (age range 15-99 years) diagnosed with a Wilms' tumour during 1983-1994 and registered by one of the 22 cancer registries in 16 countries contributing to the EUROCARE (European cancer registries study on cancer patients' survival and care) database were analysed. Relative survival at 1 and 5 years after diagnosis was estimated by age, sex, geographic area, period of diagnosis and tumour stage. A total of 143 patients, with a median age of 34 years, were included in the analysis. Crude annual incidence rates varied geographically between 0.17 and 0.27 per million. Overall relative survival was 69.9% (95% confidence interval (CI) 61.8-78.0%) at 1 year and 47.3% (38.2-56.4%) at 5 years. Survival was 2.1-fold higher for women than for men (95% CI 1.3-3.5). There was a non-significant trend for better survival for younger patients and localised tumours, but no improvement in survival by period of diagnosis. Survival was not different between geographic areas. Our results suggest a poorer outcome of nephroblastoma in adults compared with published results in children. This may, at least partly, be explained by the rarity of this diagnosis. Prognosis may be improved by the use of specific treatment guidelines for nephroblastoma in adults.     

Post-treatment T1 shortening in primary CNS lymphoma

The incidence of primary central nervous system lymphoma (PCNSL) has increased over the past two decades. The MR imaging appearance of PCNSL plays a central role in the initial diagnosis, management and follow-up of patients. The purpose of this study was to describe the presence and frequency of the pre-contrast T1 hyperintensity (T1h) that is sometimes identified in the region of enhancing neoplastic disease following treatment of PCNSL. We also explored possible causes for this phenomenon that, to the best of our knowledge, has not been previously described. The MR imaging and relevant medical records of 221 patients with pathologically confirmed PCNSL were retrospectively reviewed. Only patients with both treatment and follow-up imaging at our institution were eligible for inclusion in the study. Patients with evidence of post-procedural blood products (pre-contrast bright T1 lesions) prior to the initiation of therapy were excluded. Out of 221 patients, 119 met the eligibility criteria and were included in this investigation. Following treatment, 75 patients (63 %) developed pre-contrast T1h not attributable to blood products. All patients with this finding had been treated with methotrexate chemotherapy. The development of pre-contrast T1h following treatment for PCNSL is common. The hyperintense T1 signal in these patients may be caused by the biochemical response of tumor cells to treatment. To assess the prognostic significance of this novel finding, additional studies focusing on disease recurrence and patient survival are warranted.     

The CSF IL-10 concentration is an effective diagnostic marker in immunocompetent primary CNS lymphoma and a potential prognostic biomarker in treatment-responsive patients

IntroductionWe aimed to confirm the diagnostic value and to evaluate the pre- and post-therapeutic prognostic value of cerebrospinal fluid (CSF) concentrations of interleukin (IL)-10 and IL-6 in patients with diffuse large B-cell primary central nervous system lymphoma (PCNSL).Patients and methodsIL-10 and IL-6 concentrations were measured in 79 patients with PCNSL at diagnosis and in 40 control individuals. Fifty-four PCNSL patients underwent repeat assessments starting at diagnosis.ResultsThe IL-10 concentration distinguished PCNSL from other neurologic diseases with a sensitivity of 88.6% and a specificity of 88.9% with a cutoff of 4 pg/ml. In a multivariate analysis of PCNSL patients, CSF involvement was associated with a higher IL-10 concentration (mean log (IL-10) of 4.4 versus 2.5 pg/ml, respectively, p = 0.0004). The pre-therapeutic IL-10 concentration had no prognostic impact on outcome. The IL-10 concentration decreased after treatment for most patients tested. Among patients with complete remission or partial remission, as evaluated by magnetic resonance imaging (MRI), a persistent detectable IL-10 level in the CSF at the end of treatment was associated with a negative impact on progression-free survival (PFS) (1-year PFS: 15%, 95% confidence interval [CI]: 2.5–38% versus 59%, 95% CI: 32–78%, respectively, p = 0.0004).ConclusionOur study confirmed that IL-10 is a useful biomarker for the diagnosis of PCNSL. We highlight new findings showing that the IL-10 level in the CSF could be used as a surrogate marker for CSF involvement and that the post-treatment IL-10 concentration could complement standard MRI for therapeutic response assessment in PCNSL.     

Is primary CNS lymphoma really becoming more common? A population-based study of incidence, clinicopathological features and outcomes in Alberta from 1975 to 1996

Background: The incidence of primary CNS lymphoma (PCNSL) is believed to be increasing in immunocompetent patients but this may not be universally true. The objective of this study was to determine in a population if the incidence of PCNSL is increasing, if the histologic subtypes are changing, and to describe the clinicopathologic and outcome characteristics of PCNSL.Patients and methods: We identified all Alberta residents with a histologic diagnosis of PCNSL from 1 January 1975 to 31 December 1996 using the Alberta Cancer Registry. Annual age- standardized incidence rates (ASIR), clinicopathologic and outcome characteristics were determined.Results: There were 50 immunocompetent PCNSL patients; the median age was 64 and 30 were male. Their median survival was 10.15 months. Histology was available for review in 37 (74%) patients: 19 (51%) were diffuse large cell, 16 (43%) were immunoblastic and 2 (5%) were unclassifiable malignant lymphomas. The ASIR ranged from 0.178–1.631/106 and no change in ASIR was found (test for trend, P = 0.26) for gender or age. The ASIR of malignant gliomas did not change either but increased for all other non-Hodgkin's lymphoma (94.95–138.76106; test for trend, P = 0.0001) The number of brain biopsies increased from 1979–1985 (test for trend, P < 0.0001) but remained stable from 1986–1996 (test for trend, P = 0.99).Conclusions: Unlike several other populations, PCNSL is not becoming significantly more common in Alberta. If this difference is real (i.e., not due to differences in cancer registry coding practices etc.) comparisons between Albertans and other populations in whom the incidence is rising may provide clues regarding the etiology of PCNSL.     

Survival among patients with primary central nervous system lymphoma, 1973–2004

Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin’s lymphoma that occurs in immunocompetent and human immunodeficiency virus (HIV) patients. Despite treatment advances, previous reports have produced conflicting information about survival trends over time. Using the Surveillance, Epidemiology, and End Results (SEER) data, 2,557 patients diagnosed with PCNSL between 1973 and 2004 were identified and classified by HIV status. Potential predictors of survival were evaluated using log-rank tests. Hazard ratios and 95% confidence intervals (CIs) were computed using a Cox proportional hazards regression model. The cohort included 1,732 (67.7%) HIV-negative patients and 825 (32.3%) HIV-positive patients. Median overall survival was 12 months (95% CI 10, 13) among HIV-negative patients. In this group, median survival increased over time, from 7.5 months (95% CI 6, 14) for patients diagnosed in the 1970s, to 14 months (95% CI 11, 20) for patients diagnosed in the 2000s. Independent predictors of mortality included older age (hazard ratio [HR] 1.03 [95% CI 1.02, 1.03]), earlier year of diagnosis (HR 0.98 [95% CI 0.98, 0.99]), male sex (HR 1.20 [95% CI 1.08, 1.34), married status (HR 0.70 [95% CI 0.63, 0.78]), and receipt of radiation therapy (HR 0.69 [95% CI 0.61, 0.77]). HIV positivity was a powerful adverse prognostic factor in the overall cohort (HR 4.55 [95% CI 4.01, 5.16]). Despite treatment advances, survival among PCNSL patients in the United States remains poor. However, in the subset of PCNSL patients who are HIV-negative, survival has improved over time.     

Primary central nervous system lymphoma: a single-centre experience of 55 unselected cases

AimsCurrent treatment for primary central nervous system lymphoma (PCNSL) involves high-dose methotrexate (HDMTX) with or without radiotherapy. Many published studies describing this approach include a highly selected group of patients. We report a single-centre experience of unselected cases of PCNSL.Materials and methodsWe retrospectively reviewed the case notes of 55 consecutive patients diagnosed with biopsy-proven PCNSL between 1995 and 2003 at Addenbrooke's Hospital Cambridge, UK. We describe the treatment and outcome, including survival, treatment-related toxicity and long-term functional disability.ResultsAt diagnosis, 45% of patients were considered unfit to receive treatment with HDMTX, owing to poor performance status or comorbidity. These patients had a median survival of 46 days and may not have been included in other published studies. The remaining patients were treated with a chemotherapy regimen, which included HDMTX. Patients who received at least one cycle of a chemotherapy containing HDMTX had a median survival of 31 months. Forty per cent did not complete planned chemotherapy owing to toxicity, disease progression or death. The median survival of patients treated with HDMTX aged 60 years compared with patients aged under 60 years was 26 months vs 41 months (P = 0.07), respectively. Younger patients treated with HDMTX, who achieved complete remission with chemotherapy, had a median survival of 56 months. We identified a high incidence of functional disability among survivors, resulting from a combination of the tumour itself, the neurosurgical procedure required for diagnosis and the late neurotoxicity of combined chemoradiotherapy.ConclusionThe treatment of PCNSL is associated with significant early and late toxicity. Further attempts to improve treatment should address mechanisms to reduce this toxicity. In particular, the benefit of radiotherapy in patients who achieve complete remission with HDMTX will remain uncertain until it is addressed in a multicentre, randomised trial.     

Breast cancer survival in Rizal, Philippines, 1996-1997

The department of health-Rizal cancer registry (DOH-RCR) was the first population-based cancer registry in the Philippines, established in 1974. Even though cancer is reportable by legislation, cancer registration is pursued by active methods. Data on survival from cancer of the breast registered in 1996-1997 are reported. Followup was carried out by passive and active methods. The proportion of cases with a histological confirmation of cancer diagnosis was 90%; death certificates only (DCOs) constituted 6%; 81% of the total registered were included for the survival analysis. Complete follow-up at five years from the incidence date was 30%. Relative survival rates at one, three and five years were 89%, 56% and 37%, respectively. Five-year age-standardized relative survival was 35%. Five-year relative survival by age group did not display any pattern or trend, and was fluctuating. A majority of cases were diagnosed with a regional spread of disease (44%) followed by localized stage (17%). Five-year absolute survival ratesby extent of disease were localized (65%), regional (35%), distant metastasis (12%) and unknown (35%). Thetrend of 5-year survival for breast cancer decreased from 46% in 1987 to 37% in 1996-1997.     

基于SEER 数据库分析原发性中枢神经系统淋巴瘤患者的预后影响因素

目的:探讨原发性中枢神经系统淋巴瘤（PCNSL）患者的预后影响因素。方法:基于美国国立癌症研究所的监测、流行病学、结果数据库(SEER)选择2004至2015 年确诊为PCNSL的患者,采用COX比例风险模型进行单因素和多因素分析确定PCNSL患者的独立预后因素,Kaplan-Meier法绘制生存曲线,Log-rank检验比较不同组别患者的生存率差异。结果:单因素分析显示发病年龄、婚姻状态、原发部位、病理类型、B症状、HIV感染情况、治疗方式与原发性中枢神经系统淋巴瘤患者生存预后密切相关,多因素分析显示发病年龄、婚姻状态、原发部位、病理类型、HIV感染情况、治疗方式是影响PCNSL患者生存预后的独立因素。结论:发病年龄、婚姻状态、原发部位、病理类型、HIV感染情况、治疗方式是影响原发性中枢神经系统淋巴瘤患者生存预后的独立因素。