40例早期胃外黏膜相关淋巴组织淋巴瘤的治疗结果

目的 分析胃外黏膜相关淋巴组织(MALT)淋巴瘤的临床特征和预后.方法 回顾性分析40例首程治疗的Ⅰ_E～Ⅱ_E期原发胃外MALT淋巴瘤,其中男女比例为1:2,中位年龄54岁.原发病部位为肠道10例,眼附属器9例,甲状腺8例,肺5例,韦氏环2例及其他部位6例.Ⅰ_E期27例,Ⅱ_E期13.17例患者接受放疗(其中7例合并化疗),18例接受化疗(未合并放疗),5例单纯手术切除.结果 中位随访58个月.5年总生存率和无进展生存率分别为86%和82%.Ⅰ_E期和Ⅱ_E期5年总生存率分别为92%和76%(χ~23.66,P=0.060),无进展生存率分别为85%和76%(χ~2=1.04,P=0.300).原发眼附属器MALT淋巴瘤的5年总生存率和无进展生存率均为100%.17例接受放疗的患者无局部区域复发,局部区域控制率为100%,而23例未接受放疗者局部区域复发率为13%(3例).结论 Ⅰ_E期胃外MALT淋巴瘤可取得较好的治疗效果,放疗仍是标准治疗手段,原发眼附属器MALT淋巴瘤预后最好.     

原发韦氏环黏膜相关淋巴组织淋巴瘤的临床特点和长期治疗结果

目的 分析原发韦氏环黏膜相关淋巴组织(MALT)淋巴瘤的临床特点和长期治疗结果。方法 回顾分析本院初治的 10例原发韦氏环MALT淋巴瘤,其中男 1例、女 9例,Ⅰ_E期 7例、Ⅱ_E期 3例。单纯放疗 3例,7例在放疗前加 1~4周期化疗或再加辅助化疗。放疗中位剂量40 Gy,化疗方案以CHOP方案为主。结果 中位年龄 58岁,均无全身症状,只有 1例乳酸脱氢酶升高。疗后完全缓解率100%。中位随访90个月,全组 5年总生存率、疾病相关生存率和无进展生存率分别为90%、100%和80%。1例死于直肠癌肝转移,1例脑部复发经放疗挽救治愈。结论 韦氏环MALT淋巴瘤与其他胃外MALT淋巴瘤有类似临床特点,放疗为主的治疗取得了出色的长期疗效。     

ⅠE期原发眼附属器黏膜相关淋巴组织淋巴瘤的放疗效果分析

目的 评价ⅠE期原发眼附属器黏膜相关淋巴组织淋巴瘤放疗剂量效应和预后。方法 回顾分析2003-2015年单纯放疗 93例(117只眼)ⅠE期原发眼附属器黏膜相关淋巴组织淋巴瘤患者资料。通过眼球突出度的改变观察剂量效应。采用Kaplan Meier法计算生存率并Logrank法检验和单因素预后分析。结果 照射剂量≥27 Gy者眼球突出度缓解率为69%(29/42),眼球突出度缓解中位剂量7.2(5.4~19.8) Gy。全组患者局部控制率100%。全组患者5、10年总生存率分别为92%、82%,病因特异性生存率均为98%,无进展生存率分别为90%、88%。单因素分析显示国际预后指数、年龄是无进展生存的预后因素(P=0.04、0.04)。结论 原发眼附属器黏膜相关淋巴组织淋巴瘤对放疗敏感。单纯放疗可以获得局部控制和长期生存。处方剂量18 Gy可以获得满意的疗效。     

原发纵隔B细胞淋巴瘤疗效分析

目的 分析原发纵隔B细胞淋巴瘤的治疗疗效和失败原因.方法 搜集14年间收治的病理让实的原发纵隔B细胞淋巴瘤46例,其中Ⅰ期14例,Ⅱ期23例,Ⅲ期3例,ⅣA期6例.21例接受手术治疗,其中开胸探查术19例,纵隔镜活检术2例.37例Ⅰ+Ⅱ期中,8例接受单纯化疗,29例接受化疗+放疗,Ⅲ+Ⅳ期以化疗为主.放疗多采用纵隔加颈部照射野,中位剂量为45.00 Gy(16.95～61.00 Gy).化疗采用CHOP方案27例,三代化疗方案(MACOP-B,ProMACE/CytaBOM,m-BACOD,PmMACE-MOPP)9例,高剂量化疗加自体外周血干细胞移植(HDCT/APBSCT)10例.化疗+美罗华16例.化疗周期多为6～8个.结果 全组患者首程治疗后达完全缓解19例,部分缓解14例,病变进展11例,未评价2例.全组11例死于肿瘤.全组5年总生存率为35%.Ⅰ+Ⅱ期和Ⅲ+Ⅳ期2年总生存率分别为79%和51%,5年总生存率分别为63%和0(X2=4.35,P=0.037),2年无进展生存率分别为63%、11%(x2=17.77,P=0.000).治疗后达完全缓解者5年总生存率为80%,部分缓解者为50%,病变进展者为0(X2=19.58,P=0.003).19例治疗中病变进展或治疗后复发,腹腔淋巴结受侵和远处结外器官受侵最常见.结论 晚期患者生存率低,需要探讨更为有效的全身治疗方案,放疗可改善局部控制率.     

77例胃的黏膜相关淋巴组织淋巴瘤的临床分析

目的 回顾分析原发于胃的黏膜相关淋巴组织淋巴瘤(MALT)的治疗结果、预后因素和失败类型.方法 搜集20余年间收治的原发胃MALT淋巴瘤病例,病理证实且为Ⅰ、Ⅱ、ⅡE期.77例患者进入分析,手术切除组60例(手术14例、手术+化疗32例、手术+化放疗4例、手术+放疗9例、化疗+手术+化疗1例),非手术切除组17例(化放疗11例、化疗5例、放疗1例).放疗采用常规放疗(20例)和三维适形放疗(5例).化疗采用CHOP、BACOP或COP方案(53例).结果 随访1～198个月,中位值57个月.全组患者5年总生存率为74%,5年无瘤生存率为70%,5年局部控制率为76%,5年无远处失败生存率为87%.单因素分析显示临床分期与总生存率显著相关(P=0.02),肿瘤大小(P=0.03)和手术程度(P=0.02)与无瘤生存率显著相关,临床分期(P=0.04)、肿瘤大小(P=0.01)和手术程度(P=0.03)与局部控制率显著相关,未发现与无远处失败生存率显著相关因素.Ⅰ、Ⅱ期患者手术治疗后失败主要在远处,非手术治疗患者的失败主要在局部.ⅡE期患者,无论手术组还是非手术组,失败主要在局部.结论 胃原发黏膜组织相关淋巴瘤采用手术和非手术治疗均可取得很好的治疗效果,临床分期是非常重要的预后因素.     

71例原发颅内中枢神经系统DLBCL预后分析

目的 探讨原发颅内中枢神经系统弥漫性大B细胞淋巴瘤(DLBCL)的预后因素。 方法 回顾分析1991—2015年间收治的经病理和临床证实的 71例原发颅内中枢神经系统DLBCL临床资料。全组患者均进行了化疗,59例进行了放疗,化疗方案以HD-MTX (HD-MTX,66/71)为主,放疗方案以全脑放疗 ±局部推量为主。Kaplan-Meier法计算生存率,Logrank法检验和单因素预后分析,Cox模型多因素预后分析。 结果 放化疗结束时 58例CR, 10例PR,3例PD。5年生存率为43%;5年无疾病进展率为34%。单因素分析显示年龄、KPS评分、单发与多发、是否放疗、放化疗完成时评价、有无复发是影响OS的因素(P=0.000~0.047),多因素分析显示年龄、KPS评分、有无复发是影响OS的因素(P=0.000~0.022)。单因素分析化疗方案、是否放疗、总放疗剂量、全脑剂量、放化疗完成时评价、有无复发是影响PFS的因素(P=0.000~0.028);多因素分析KPS评分、有无复发是影响PFS的因素(P=0.000~0.011)。 结论 年轻、KPS评分高、无复发患者总生存更好,单发、接受放疗、放化疗后疗效好的患者可能更好;KPS评分高、放化疗后疗效好、无复发患者PFS更好,接受含HD-MTX化疗、接受放疗、总的放疗剂量和全脑剂量越高患者PFS可能更好。化疗达CR后是否还放疗及放疗靶区、剂量需进一步研究。     

早期鼻腔NK/T细胞淋巴瘤治疗方法和预后分析

目的 探讨不同治疗方法对早期鼻腔NK/T细胞淋巴瘤预后的影响.方法 回顾分析15年问85例ⅠE、ⅡE期鼻腔NK/T淋巴瘤放疗及CHOP为主化疗的疗效.单纯化疗(单化组)20例,放疗后±化疗(放化组)17例(单纯放疗11例),化疗后放疗(化放组)48例.生存率计算采用Kaplan-Meier法,并Logrank法检验,Cox回归模型进行多因素分析.结果 全组5年生存率为40%,单化纽、放化组和化放组的分别为13%、54%和47%,放化纽和化放组均优于单化组(P=0.030和0.049).ⅠE局限组与超腔组的5年生存率分别为57%与28%(χ2=8.87,P=0.003),ⅡE期的为23%,与ⅠE超腔组相似(χ2=0.19,P=0.664).近期疗效达到完全缓解与未完全缓解的5年生存率分别为58%与12%(χ2=30.68,P=0.000).放疗剂量≤50 Gy与>50 Gy的完全缓解率分别为56%和86%(χ2=6.11,P=0.013),5年无复发生存率分别为89%与84%(χ2=0.36,P=0.551).首程化疗的68例中≤2、3～4、≥5个疗程者分别为18、20、30例,完全缓解率分别为0%、20%、33%(χ2=7.65,P=0.022).首程先化疗且≥3个疗程的50例和先放疗≥40 Gy的17例的完全缓解率分别为28%和88%(χ2=18.75,P=0.000).结节型和溃疡型的完全缓解率放疗均优于化疗(100%:38%,2X=7.92,P=0.005和100%:11%,χ2=14.40,P=0.000).多因素分析显示临床分期和近期疗效是影响预后的独立因素.结论 早期鼻腔NK/T细胞淋巴瘤首程应选择50 Gy放疗为宜.对于ⅠE期超腔与ⅡE期应酌情联合化疗,但CHOP方案效果欠佳.     

21例原发性中枢神经系统淋巴瘤临床分析及文献复习

目的 :原发性中枢神经系统淋巴瘤(Primary central nervous system lymphoma,PCNSL)指原发于中枢神经系统,无其它系统播散的淋巴瘤。近年来发病率呈上升趋势且预后较差。本研究目的在于探讨PCNSL的合理诊断方法及治疗方式。 方法 :回顾性分析自1993年6月至2006年6月经病理证实的21例原发性中枢神经系统淋巴瘤的临床特征、诊断方法及治疗方式。采用SPSS11.5软件包进行统计学分析。 结果 :男性12例,女性9例,中位年龄50岁(46～55岁)。单发病灶15例,多发者6例。CT、MRI检查示明显的肿物占位效应,边界清楚,伴周围水肿。病理检查均为B细胞淋巴瘤,脑脊液未见癌细胞。3例行立体定向穿刺活检,18例行手术治疗完全或部分切除肿瘤。17例术后或立体定向穿刺术后行放、化疗综合治疗。10例术后先行放疗后行化疗,7例先行化疗后行放疗。Kaplan-Meier分析全组中位生存期(Mediansurvivaltime,MST)22.6个月,2年生存率44.5%。经Log-Rank检验,先放后化组与先化后放组的生存期未见统计学差异(P=0.0547)。前者中位生存期36个月,2年生存率66.7%;后者中位生存期19个月,2年生存率35.7%。 结论 :原发性中枢神经系统淋巴瘤采用化疗和放疗综合治疗有望提高生存率。手术的意义仅仅在于取活检,并不能延长生存期。对于影像学高度怀疑的病例应行立体定向穿刺活检明确诊断,不必手术。放化疗的顺序对于患者总生存期并无影响。但先放后化组与先化后放组的2年生存率、中位生存期仍然存在较大差异。先放后化组好于先化后放组。     

原发中枢神经系统淋巴瘤40例治疗与预后分析

目的 比较不同治疗方法对原发中枢神经系统淋巴瘤患者的有效性及安全性.方法 收集40例原发中枢神经系统淋巴瘤患者,分为3组,其中单纯手术组10例、手术联合化疗组15例、手术联合放化疗组15例,对3组进行生存分析比较.结果 随访7~53个月,中位生存时间手术联合放化疗组(45个月)和手术联合化疗组(30个月)高于单纯手术组(17个月),差异均有统计学意义(P均<0.05).3 a生存率手术联合放化疗组(72.2%)高于手术联合化疗组(40.8%)和单纯手术组(27.3%,P均<0.05).结论 手术联合放化疗可有效治疗原发中枢神经系统淋巴瘤,且患者耐受性好.     

原发骨非霍奇金淋巴瘤临床分析

目的:探讨原发骨非霍奇金淋巴瘤的临床特点、诊断、治疗及预后。方法:选择1970年11月～2003年2月我院收治的21例原发于骨的非霍奇金淋巴瘤,其中Ⅰ期14例(66.7%),Ⅱ期2例(9.5%),Ⅳ期5例(23.8%)。弥漫性大B细胞型12例(57.1%),混合细胞型4例(19.0%),小淋巴细胞型1例(4.8%),T细胞型1例(4.8%),未分型3例(14.3%)。采用单纯放疗6例(28.6%),放疗和化疗综合治疗15例(71.4%)。结果:中位随访86个月,2例(9.5%)局部复发,9例(42.9%)出现远处受侵,3年、5年、10年无进展生存率分别为56.7%、38.9%、29.1%;3年、5年、10年总生存率分别为69.1%、42.2%、42.2%。结论:原发于骨的非霍奇金淋巴瘤首选放疗和化疗的综合治疗;放疗剂量推荐45Gy～46Gy。     

42例原发性中枢神经系统淋巴瘤患者预后影响因素分析

背景与目的：原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma,PCNSL）是发生在脑、脊髓、脑膜或眼的罕见侵袭型非霍奇金淋巴瘤,无CNS之外的部位累及。PCNSL与其他类型淋巴瘤相比,患者生存期短,预后差,且复发率高,未经治疗的患者的中位生存期仅为3个月。近年来研究发现C-MYC、BCL-2、BCL-6、Ki-67等指标在一定程度上影响PCNSL患者预后。因此,通过分析PCNSL相关蛋白表达、治疗方式及其他临床因素对患者预后的影响, 希望为该病的临床治疗及预后评价进一步积累资料。方法：回顾性分析自2013年6月—2021年5月于大连医科大学附属第二医院治疗的42例经病理学检查明确诊断为原发性中枢神经系统弥漫大B细胞淋巴瘤患者的临床资料,包括性别、年龄、病灶数量、美国东部肿瘤协作组（Eastern Cooperative Oncology Group,ECOG）评分、血清乳酸脱氢酶（lactate dehydrogenase,LDH）、病灶是否累及深部脑组织、治疗方案、病理学Hans分型及C-MYC、BCL-2、BCL-6、Ki-67等生物标志物,结合随访调查,了解患者生存时间及生存状况,应用Kaplan-Meier法及log-rank检验分析影响患者无进展生存期（progression-free survival,PFS）和总生存期（overall survival,OS）的预后相关因素,多因素分析采用COX回归模型。结果：42例PCNSL患者中位发病年龄61岁,男女比例为1.33∶1.00,颅脑增强MRI病灶多呈均匀明显强化。所有患者均接受含有大剂量甲氨蝶呤（high-dose methotrexate,HD-MTX）方案化疗,治疗后评价完全缓解（complete response,CR）20例、部分缓解（partial response,PR）5例,疾病稳定（stable disease,SD）11例,疾病进展（progressive disease,PD）6例。中位PFS为21个月,中位OS为34个月,1年PFS率为63.7%,2年PFS率为47.0%;1年OS率为70.8%,2年OS率为55.6%。单因素分析结果显示,影响PFS的因素是HD-MTX多药联合化疗、鞘内化疗及联合利妥昔单抗。影响OS的因素是ECOG评分≥2、C-MYC（+）、BCL-2及C-MYC双表达、HD-MTX多药联合化疗、鞘内化疗及联合利妥昔单抗。多因素分析结果显示：利妥昔单抗治疗是影响PFS的独立预后因素（P=0.020）,ECOG评分、利妥昔单抗是影响OS的独立预后因素（P=0.007;P=0.046）。与未接受巩固治疗的患者相比,接受巩固治疗患者的中位PFS及OS较高;进一步的亚组分析显示,自体干细胞移植（autologous stem cell transplantation,ASCT）组的中位PFS及OS较全脑放疗（whole brain radiation therapy,WBRT）组高,但差异无统计学意义。结论：PCNSL多发于中老年人,男性多于女性,影像学缺乏特异性。ECOG评分≥2与PCNSL患者较差的OS相关。C-MYC（+）、BCL-2及C-MYC双表达可作为指导危险分层的预后标志物。以HD-MTX为基础的多药联合化疗已经成为PCNSL的首选治疗手段,利妥昔单抗的应用可延长生存期。在全身化疗的基础上,联合局部鞘内化疗可以改善预后。进一步的巩固治疗主要包括ASCT及WBRT,可延长PFS及OS,ASCT可以取得与WBRT相似的疗效,且可避免WBRT的晚期神经毒性,但本研究中因样本量及随访时间的限制,未得出明确的统计学结果。     

Autologous Stem Cell Transplantation in Central Nervous System Lymphoma: A Multicenter Retrospective Series and a Review of the Literature

BackgroundCentral nervous system (CNS) lymphoma is associated with poor outcomes. Autologous stem cell transplantation (ASCT) has been reported to improve outcomes when used as a consolidation strategy in primary CNS lymphoma (PCNSL) and as a salvage strategy in patients with disease relapse limited to the CNS. Herein, we describe our experience of using ASCT in PCNSL and secondary CNS lymphoma (SCNSL).Patients and MethodsWe evaluated clinical outcomes of 18 patients from 2 major academic centers with a median age of 55 (range, 46-72) years. Thirteen patients had PCNSL and 5 patients had SCNSL. Most of the cases were in the first (CR1) or second (CR2) complete remission (CR1 = 7, CR2 = 7) at the time of ASCT. Carmustine with thiotepa (n = 12, 67%) was the most commonly prescribed preparative regimen.ResultsThe median follow-up from ASCT for surviving patients was 12 (range, 0.9-115) months. The 2-year progression-free survival (PFS) and overall survival (OS) were 74% (95% confidence interval [CI], 48%-99%) and 80% (95% CI, 55%-100%), respectively. Two-year non-relapse mortality was 0%. The 2-year cumulative incidence of relapse/progression was 27% (95% CI, 10%-72%). In subgroup analysis of PCNSL patients, 2-year PFS, OS, and relapse were 71% (95% CI, 38%-100%), 71% (95% CI, 38%-100%), and 29% (95% CI, 9%-92%), respectively.ConclusionIn this retrospective study of patients with CNS lymphoma, consolidation with ASCT after high-dose methotrexate-based chemotherapy is safe and effective in reducing disease relapse.     

Clinical analysis of patients with primary and secondary extranodal natural killer/T-cell lymphoma of central nervous system

Extranodal natural killer (NK)/T-cell lymphoma (NKTL) is a rare non-Hodgkin lymphoma that rarely arise exclusively in or metastasizes to the central nervous system (CNS). Globally, CNS involvement of NKTL heralds a serious prognosis and there is no standard treatment. 19 of 414 patients (4.59%) with ENKL followed were diagnosed with CNS involvement between 2006 and 2020. Two patients had primary CNS (PCNS) NKTL, and 17 patients had secondary CNS (SCNS) invasion. A total of 9 patients survived and 10 patients died. The median overall survival time was 55 months, and the median survival time after CNS invasion was 17 months. The 5-year cumulative survival probability was 45.7%. In conclusion, CNS risk evaluation and prophylaxis treatment can be carried out for patients with NK/T-cell lymphoma prognostic index risk group III/IV. In terms of treatment, systemic therapy based on methotrexate combined with radiotherapy and intrathecal chemotherapy can be selected.     

Relevance of intraocular involvement in the management of primary central nervous system lymphomas.

BACKGROUND: Reported data regarding intraocular lymphoma (IOL) management are anecdotal. Cases of IOL included in an international multicentre series of 378 immunocompetent patients with primary central nervous system lymphomas (PCNSLs) were reviewed. PATIENTS AND METHODS: Staging included slit-lamp examination in 170 patients: IOL was diagnosed in 22 cases (13%). A concomitant brain lesion was detected in 21 cases. Planned treatment was chemotherapy followed by radiotherapy in 13 cases, chemotherapy alone in three and radiotherapy, followed by or not by chemotherapy in five; one patient was not treated. Chemotherapy included high-dose methotrexate in 12 cases. Ten patients received intrathecal chemotherapy. Radiotherapy consisted of whole brain irradiation, followed by or not by a tumour bed boost; ocular irradiation was planned in 15 cases. Irradiation in one patient without brain lesions was limited to the orbits only (50 Gy). RESULTS: IOL was positively correlated to systemic symptoms and meningeal disease. Fifteen patients (71%) achieved an objective response; 16 patients experienced a failure (2-year failure-free survival 34+/-10%). Failures involved the eyes in eight cases, with a 2-year time to ocular relapse of 59+/-11%. Ocular failure was less common in patients treated with chemotherapy plus ocular irradiation and was associated with a significantly shorter survival. Seven patients are alive [median follow-up 53 months, 2-year overall survival (OS): 39+/-11%] , five of whom were treated with ocular irradiation. The patient with isolated IOL is alive and disease-free at 14 months. OS of the entire series was similar to that of PCNSL patients with negative slit-lamp examination. CONCLUSIONS: IOL is usually associated with concomitant brain disease and shows a survival similar to that of the rest of PCNSLs. Chemotherapy combined with ocular irradiation resulted in better control of ocular disease, which seems to be associated with survival. In view of the potential role of ocular irradiation, the use of chemotherapy alone in phase II trials should be critically reconsidered in PCNSL patients with ocular disease.     

利妥昔单抗联合适形放疗治疗术后原发中枢神经系统淋巴瘤的疗效分析

 【摘要】　目的 探讨利妥昔单抗结合全颅放射治疗、局部三维适形放疗（3D-CRT）加量治疗术后原发中枢神经系统淋巴瘤（PCNSL）的疗效。方法　PCNSL术后患者23例,年龄均＜60岁,全颅放射治疗处方剂量32.4 Gy,局部病灶采用3D-CRT推加剂量至50.4 Gy,放射治疗第1天开始使用利妥昔单抗（375 mg／m2）,每周1次,连续使用6周。用Kaplan-Meier法分析患者的生存情况。结果　23例患者中,完全缓解19例（82.6 ％）,部分缓解3例（13.0 ％）;无进展生存14例（60.9 ％）,无病生存期26个月（17～34个月）,总生存期为40个月（29～55个月）;无Ⅲ～Ⅳ级神经毒性出现。结论 利妥昔单抗联合全颅放疗、病灶部位3D-CRT推量的方案治疗年龄＜60岁的术后PCNSL患者可以获得较长的生存期,而且治疗的不良反应低。     

原发性中枢神经系统淋巴瘤的放射治疗进展

原发性中枢神经系统淋巴瘤(PCNSL)是指发生在脑、脊髓、脑膜或眼的罕见侵袭型非霍奇金淋巴瘤,以弥漫大B细胞淋巴瘤占绝大多数,其中又以Non-GCB亚型多见。未经治疗的患者中位生存期仅为3个月,单纯的手术切除肿瘤并没有明显的生存获益。早期单独使用全脑放疗(WBRT),缓解率高,但持续时间短,且延迟性神经系统不良反应是一...     

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.     

The clinical characteristics and treatment outcome of 57 children and adolescents with primary central nervous system germ cell tumors

Primary central nervous system germ cell tumors （CNS-GCTs） in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years （range, 2.7 to 18.0 years） at diagnosis; 43 （75.4%） had non-germinomatous germ cell tumors （NGGCTs） and 14 （24.6%） had germinomas; 44 （77.2%） had localized disease and 13 （22.8%） had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB （cisplatin, etoposide, and bleomycin） protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months （range, 1.2 to 139 months）. Fourteen patients died of relapse or disease progression. The 3-year event-free survival （EFS） and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs （P = 0.064）. The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively （P = 0.042）. Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.     

Bcl-6 predicts improved prognosis in primary central nervous system lymphoma

BACKGROUND: In systemic non-Hodgkin lymphoma (NHL), tumors that resemble germinal center B-cells are less aggressive than tumors that resemble postgerminal center B-cells. However, the value of B-cell differentiation markers in primary central nervous system lymphoma (PCNSL) is less clear. The objectives of this study were to characterize the immunophenotypes of PCNSL samples and to determine their utility in predicting clinical outcomes. METHODS: The immunohistochemical staining profile of PCNSL was determined from 66 immunocompetent patients. Then, the authors evaluated whether the expression of these proteins was associated with progression-free or overall survival. RESULTS: Only 8% of PCNSL samples were positive for the cluster designation (CD) germinal center marker CD10. Another germinal center marker, bcl-6, was positive in 47% of samples. Ninety-four percent of samples expressed significant levels of the postgerminal center marker melanoma-associated antigen (MUM-1). In univariate analyses, only bcl-6 staining had a significant effect on progression-free survival (median, 20.5 months in bcl-6-positive patients vs 10.1 months in bcl-6-negative patients; P = .02). There was a nonsignificant trend toward improved overall survival in patients who had bcl-6 expressing tumors. Older age and poorer performance status, as observed previously, were associated with reduced survival. CONCLUSIONS: Bcl-6 expression was associated with a better prognosis in patients with PCNSL.