原发性中枢神经系统淋巴瘤35例临床及预后分析

目的：探讨原发中枢神经系统淋巴瘤（PCNSL）临床特点、诊治方案及临床疗效。方法：总结2001年1月-2008年1月收治的35例PCNSL患者,均经病理证实为B细胞来源非霍奇金淋巴瘤并接受放疗,其中25例放疗后接受化疗。结合文献对原发性中枢神经系统淋巴瘤患者的临床特点、病理学检查、影像学表现、治疗及预后进行回顾性分析。结果：本病以中老年人多见,发病急,病程短,病情进展快。临床表现复杂,颅内高压为主要表现之一。CT、MRI增强扫描病灶多呈均匀明显强化,可单发或多发。35例患者中位生存时间23月,1年生存率74．3％,3年生存率25．7％,5年生存率5．71％。肿瘤全切及局部切除者,生存率未见明显统计学差异（P=0．053）,加化疗疗效优于不加化疗（P=0．012）。结论：PCNSL临床表现多样,影像学缺乏特异性,极易误诊,确诊需要依靠病理学检查,最佳治疗方案是手术加放疗、化疗的联合治疗。PCNSL侵袭性强,生存期短,其预后主要与发病年龄、多灶性、一般状态有关。     

原发性中枢神经系统淋巴瘤临床及预后分析

目的:总结原发性中枢神经系统淋巴瘤(PCNSL)的临床特点、诊治方案及临床疗效.方法:回顾收活的21例PCNSL患者,均为病理确诊的B细胞来源非霍奇金淋巴瘤,其中5例接受单纯放疗,16例接受放化疗.对病理学检查、影像学表现、治疗及预后进行总结分析.结果:PCNSL以中老年人多见,发病急,病程短,病情进展快.临床表现复杂,颅内高压为主要表现之一.CT、MR增强扫描病灶多呈均匀明显强化,可单发或多发.21例患者中位生存时间22个月,1、3和5年生存率分别为76.2％、28.6％和4.76％.放化疗疗效优于单纯放疗,P=0.029.结论:PCNSL临床表现多样,影像学缺乏特异性,极易误诊,确诊需要依靠病理学检查,其最佳治疗方案是三维立体定向穿刺活检加放疗、化疗的联合治疗.PCNSL侵袭性强,生存期短,其预后主要与发病年龄、多灶性和体力状况有关.     

原发性中枢神经系统淋巴瘤28例临床分析

目的　探讨原发性中枢神经系统淋巴瘤 (PCNSL)的合理治疗方式及影响预后的因素。方法　回顾性分析 2 8例PCNSL的临床特征、治疗结果及预后因素 ,运用SPSS 10 .0软件包进行统计分析。结果　 2 8例PCNSL患者中 ,男性 18例 ,女性 10例 ,中位年龄 5 2岁。全组中位生存期 (MST) 2年 ,5年生存率 2 1.4 % ;单病灶 19例 ,多病灶 9例。病理检查为B细胞来源者占 78.6 % (2 2 / 2 8) ,病理类型以弥漫性大细胞淋巴瘤为主。依国际工作分类 (WF) ,中度恶性占 5 7.7% (15 / 2 8)。多因素分析结果显示 ,病灶的单发或多发是影响预后的独立因素 (P =0 .0 4 17)。对B细胞来源、WF高度恶性及多病灶的PCNSL患者 ,合并化疗的综合治疗效果较好 ,其中合并非常规化疗的疗效优于常规化疗疗效(P =0 .0 191)。结论　PCNSL较一般的NHL具有独特的预后因素 ;化疗在PCNSL的综合治疗中极为重要 ,临床应尽可能采用非常规的易透过血脑屏障的药物或给药方式     

中枢神经系统原发性淋巴瘤的诊断及预后

中枢神经系统原发性淋巴瘤大多发生于颅内，约占颅内肿瘤的１％～３％，７５％为弥漫性Ｂ大细胞淋巴瘤。本瘤恶性程度高，进展快，预后差。我院１９８０年１月至２０００年１月收治６例，术前误诊率１００％，现报告如下。１临床资料１．１临床表现及实验室检查６例中男性５例，女性１例，年龄２２～６２岁；病程１２天至１０个月。临床表现：头痛４例，偏瘫１例，截瘫１例。发生部位：左额顶部３例，左领部及枕部１例，右顶枕部１例，Ｔ。、Ｔ。－，、Ｔ；。脊髓节段水平１例。脑积液检查：蛋白升高，６例均未找到肿瘤细胞。４例作血清ＨＩ…     

原发性中枢神经系统恶性淋巴瘤6例分析

原发性中枢神经系统恶性淋巴瘤(ｐｒｉｍａｒｙｃｅｎｔｒａｌｎｅｒｖｏｕｓｓｙｔｅｍｌｙｍｐｈｏｍａ,ＰＣＮＳＬ)少见,约占全部非霍奇金淋巴瘤的1%[1],近20年来其发病率有上升趋势,其预后明显比其它系统恶性淋巴瘤差。我院1994年1月至1999年8月共收治6例,均经手术与病理证实,现分析报告如下。1　临床资料1.1　一般资料本组男性4例,女性2例,年龄12～63岁,中位年龄44岁。临床表现:头晕、头痛4例,肢体活动障碍4例,共济失调1例,癫痫1例。ＣＴ、ＭＲＩ显示单发病灶5例,其中额叶3例,左中央区1例,小脑半球1例;额、颞多发病灶1例。ＣＴ显示:边…     

原发性中枢神经系统淋巴瘤预后相关因素研究进展

 原发性中枢神经系统淋巴瘤（PCNSL）是一种少见的结外恶性淋巴瘤,好发于免疫缺陷人群,但近年来在免疫功能正常人群中发病率逐年提高。其临床治疗缓解期短,易复发,预后不佳。研究发现PCNSL的预后相关因素包括：年龄、体力状态、放化疗的方式、初治对糖皮质激素的反应、组织病理和分子生物学标志等。     

原发中枢神经系统淋巴瘤17例临床分析

目的:原发性中枢神经系统淋巴瘤(PCNSL)是一种较少见的中枢神经系统恶性肿瘤.近年发病率有逐年增高的趋势,本文探讨其临床特点、诊治方案及临床疗效.方法:总结1999年9月～2007年12月收治的17例患者(男性8例,女性9例,年龄19～80岁),均经病理证实为B细胞来源非霍奇金淋巴瘤.全部患者接受放疗,其中15例放疗后接受化疗等综合治疗.结合文献对原发性中枢神经系统淋巴瘤患者的临床特点、病理学检查、影像学表现、治疗及预后进行回顾性分析.结果:本病以中老年人多见,发病急,病程短,病情进展快.临床表现复杂,颅内高压为主要临床表现之一.头颅CT以及MRI检查显示中枢侵及额叶多见,CT扫描多表现为较高密度肿块,MRI显示T1加权像多呈低信号,T2加权像多呈高信号.CT和MRI增强扫描病灶多呈均匀明显强化,可单发或多发,极少发生出血、钙化或囊变.PCNSL主要起源于B细胞,包括手术、放疗、化疗和免疫治疗的综合治疗效果较好.17例患者随访7～65个月,中位时间17个月,平均生存23.9个月.1、3年生存率分别为76.5%和23.5%,5年生存率仅有5.9%.结论:PCNSL是一组异质性肿瘤,侵袭性强,预后差,伴脊髓侵犯者则预后更差.其临床、影像学表现复杂多变,无特异性,术前诊断困难.确诊主要依靠病理检查,三维立体定向穿刺活检(定向活检)可显著提高诊断效率.其病程短,疗效不理想,最佳治疗方案是手术加放疗、化疗的联合治疗.虽然对放射及化学药物治疗敏感,但缺乏共识方案,较全身及其他部位淋巴瘤预后差.中等剂量放疗加足疗程化疗是有效的治疗方法,增加放射剂量未能改善肿瘤控制.综合治疗是提高本病疗效的关键.     

原发性中枢神经系统恶性淋巴瘤（附五例报告）

原发性中枢神经系统恶性淋巴瘤较少见，国内报道占中枢神经系统肿瘤的０．６％￣１．２％。作者１９８４年１月￣１９９３年７月共诊治５例，均行手术探查及病理活检确诊。作者结合文献，对本瘤的组织起源、病因、病理学特征、临床特点、诊断、治疗及预后作了简要讨论。     

原发性中枢神经系统淋巴瘤的临床特征及预后分析

目的:探讨原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma,PCNSL）患者的临床病理和分子学特征及预后影响因素。方法:收集2013年03月至2021年05月山西省人民医院35例PCNSL患者的临床资料及组织标本,总结其临床病理特征并应用二代测序技术检测淋巴瘤相关基因,分析年龄、病灶部位、乳酸脱氢酶（lactate dehydrogenase,LDH）、免疫组化等指标对预后的影响,比较不同基因突变组及治疗方案之间的总生存（overall survival,OS）差异。结果:最常见的基因突变为MYD88（66.7%,12/18）,IGLL5、PIM1（38.9%,7/18）;PIM1突变组、IGLL5突变组OS差于未突变组,差异均有统计学意义（P=0.019和0.021）;多因素Cox回归分析显示LDH、不同的治疗方案是PCNSL患者独立的预后影响因素（P=0.007和0.002）;接受大剂量甲氨蝶呤（methotrexate,MTX）、利妥昔单抗（rituximab,R）治疗组OS明显优于未接受组,差异均有统计学意义（P=0.005和＜0.001）;使用布鲁顿酪氨酸激酶抑制剂（broughton tyrosine kinase inhibitor,BTKi）组与未接受组相比,有生存获益的趋势,但未显示出统计学差异。结论:血清LDH升高与不良预后显著相关,PIM1及IGLL5突变患者生存较差,含MTX、R治疗方案可改善患者生存。     

原发性中枢神经系统恶性淋巴瘤5例报告

原发性中枢神经系统恶性淋巴瘤(CNSPML)较其他部位的淋巴瘤及继发性神经系统的淋巴瘤均少见.我科1987年12月～1998年12月共收治淋巴瘤521例,其中CNSPML5例,均经手术及病理证实,现分析如下:     

原发性中枢神经系统淋巴瘤患者预后的影响因素分析

目的 探讨原发性中枢神经系统淋巴瘤(PCNSL)患者预后的影响因素.方法 收集82例PCNSL患者的临床资料,包括性别、年龄、病灶数量、病灶部位、病灶直径、治疗方法、意识状态、体力状况(PS)评分等一般资料,以及脑脊液(CSF)常规检测指标(CSF蛋白质、CSF氯化物)和生化检测指标[血清乳酸脱氢酶(LDH)、β2微球...     

71例原发性中枢神经系统淋巴瘤的临床、影像学与病理学特征

背景与目的：原发性中枢神经系统淋巴瘤（primary central nervous system lymphoma, PCNSL）是临床少见的非霍奇金淋巴瘤,近年来发病率有所上升.本研究的目的是探讨PCNSL的临床、影像学与病理学特征,提高临床对PCNSL的认识.方法：回顾性分析经病理证实的71例PCNSL的临床资料.结果：71例患者中男性45例,女性26例,男女比例为1.73：1;其中50岁以上患者44例（62.0%）,平均年龄52岁;就诊时间在1个月内者51例（71.8%）;临床症状多表现为颅内压力增高及局灶性神经系统损害的定位体征;单发病灶47例（66.2%）;MRI示T1WI低信号或等信号,T2WI高信号或等信号,大多数病灶显像增强后呈明显强化;所有病例病理均为B细胞性.结论：PCNSL多发于中老年人,男性多见,病程短,单发病灶多见,病灶多呈明显均匀强化,绝大多数为B细胞性,临床表现及影像学缺乏特异性,确诊需依赖于病理.     

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。     

A new prognostic model using absolute lymphocyte count in patients with primary central nervous system lymphoma

PurposePrimary central nervous system lymphoma (PCNSL) is an aggressive and rare extranodal non-Hodgkin lymphoma (NHL). Absolute lymphocyte count (ALC) has been suggested to have a prognostic value in several subtypes of NHL. We evaluated the prognostic significance of clinical factors, including ALC, in patients with PCNSL to develop a new prognostic model.MethodsWe analysed prognostic factors, including ALC, at diagnosis in 81 PCNSL patients receiving high-dose methotrexate-based therapy.ResultsThe median ALC at diagnosis was 1210 × 10⁶/L (range, 210–3610), with lymphopenia (≤875 × 10⁶/L) being detected in 27 (33.3%) patients. In the multivariate analysis, Eastern Cooperative Oncology Group performance status (ECOG PS) >1 (hazard ratio [HR] 3.18, P = 0.003), age >50 years (HR 4.23, P = 0.012), and lymphopenia at diagnosis (HR 2.83, P = 0.008) remained independent prognostic factors for low overall survival (OS). Lymphopenia was also a significant prognostic factor for progression-free survival (HR 3.17, P = 0.001). By means of a new three-factor prognostic model using ECOG PS >1, age >50 years, and presence of lymphopenia, with 1 point assigned to each factor, we successfully classified the patients into three risk groups: low (0 and 1), intermediate (2), and high (3). The 5-year OS rates of the patients in the low-, intermediate-, and high-risk groups were 74.3%, 21.7%, and 12.5%, respectively (P < 0.001).ConclusionsLow ALC is a useful indicator of poor prognosis in patients with PCNSL. The proposed three-factor model should be validated in large-scale studies.     

Relapse of primary central nervous system lymphoma: clinical features, outcome and prognostic factors

Data on relapsed primary central nervous system lymphoma (PCNSL) are limited. We have evaluated the clinical characteristics and outcome of relapsed PCNSL patients from two German trials. Patients with relapsed disease after primary treatment were studied. Primary therapy consisted of high-dose methotrexate-based chemotherapy in all patients. Treatment for relapse was not predetermined. After a median follow-up of 22.5 months, 52 (36%) patients with relapse were identified among 143 patients with complete remission (CR) after primary treatment. The median disease-free survival was 10.25 (3–47.5) months. The median age at relapse was 59 years. Forty-four of 51 evaluable patients relapsed within the CNS, 6 systemically and one both cerebrally and systemically. The median survival time after first relapse was 4.5 (0.5–40.5) months. Karnofsky performance status (KPS) at relapse (P = 0.004), site of relapse (isolated systemic versus other, P = 0.049) and treatment for relapse (versus no treatment, P = 0.001) were independent prognostic factors for survival after relapse in multivariate analysis. Survival of patients with relapsed PCNSL is poor despite high response rates to salvage therapy. Good KPS, isolated systemic relapse and treatment for relapse were significantly associated with longer survival.     

Second malignancies in patients with primary central nervous system lymphoma

Background

Primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphoma with distinctive biological behaviors. The evolving treatment of PCNSL has greatly improved the outcome for patients with this disease and has stimulated interest in second malignancies (SMs) in patients diagnosed with PCNSL.

Methods

The records of 129 cases of PCNSL at Mayo Clinic, diagnosed between January 1, 1988, and November 26, 2012, were reviewed. Data on clinical characteristics, laboratory parameters, treatments, outcomes, and SMs were collected. The mean follow-up time was 44.8 months (range, 0.5–240 months; median, 28.0 months).

Results

Altogether, 28 cases with 30 (23.26%) SMs were identified. Twenty (15.50%) patients had prior or synchronous SM. Ten (7.76%) patients developed a subsequent primary cancer after PCNSL. The most common sites of prior or synchronous SMs were prostate (4/20), skin (4/20), and gastrointestinal (3/20). The most common site of the subsequent SM was skin (4/10). Two cases were identified with both prior SM and subsequent SM.

Conclusions

Second malignancies in cases with PCNSL were not uncommon and occurred in nearly a quarter of our cohort. Nonmelanoma skin cancers were frequently seen. Therefore, screening for SMs should also be considered in long-term follow-up of patients with PCNSL. In addition, the high incidence of subsequent cancer, synchronous cancer, and frequently seen nonmelanoma skin cancers may all indicate an immunosuppressed state in patients with PCNSL.     

Characteristics and outcomes of elderly patients with primary central nervous system lymphoma

BACKGROUND:

Approximately 50% of all patients with primary central nervous system lymphoma (PCNSL) are aged ≥65 years; however, this group is relatively understudied, and to the authors's knowledge, optimal treatment for older patients is not well defined.

METHODS:

This was a retrospective review of PCNSL patients aged ≥65 years who were treated at Memorial Sloan‐Kettering Cancer Center between 1986 and 2008. A multivariate analysis of demographic and clinical variables on prognosis and receipt of treatment was performed.

RESULTS:

One hundred seventy‐four patients between the ages of 65 and 89 years were identified; there was a slight predominance of women (52.9%). One hundred forty‐eight patients were treated with chemotherapy at the time of diagnosis (98% with methotrexate‐based therapy) and 31 of these patients also received whole‐brain radiotherapy (WBRT). Sixteen patients received WBRT alone. A radiographic response to chemotherapy was noted in 76% of patients. Ninety patients developed disease progression after initial treatment; 74 received salvage therapy and 48% of these patients responded to salvage treatment. The median overall survival was 25 months (range, 18‐33 months), and the 3‐year survival rate was 36%. Approximately 20.1% of patients were alive for ≥11 years. WBRT was delivered more frequently before 1998, and patients with a history of prior malignancy were less likely to receive WBRT. Age and performance status were identified as the most important predictors of survival. Treatment‐related neurotoxicity at 2 years was strongly associated with receipt of WBRT (P = .0002).

CONCLUSIONS:

PCNSL in the elderly remains sensitive to methotrexate‐based chemotherapy and aggressive treatment may be warranted both at the time of diagnosis and disease recurrence. Cancer 2010. © 2010 American Cancer Society.