In this editorial I comment on the article,published in the current issue of the World Journal of Clinical Oncology.Primary central nervous system lymphoma(PCNSL)is a disease of elderly and immunocompromised patients....In this editorial I comment on the article,published in the current issue of the World Journal of Clinical Oncology.Primary central nervous system lymphoma(PCNSL)is a disease of elderly and immunocompromised patients.The authors reported clinical results of 19 patients with PCNSL treated with zanubrutinib/high dose methotrexate(HD-MTX)until disease progression.They demonstrated that the combination of zanubrutinib with HD-MTX led to a marked clinical response and tolerability among these patients.They also observed that cerebrospinal fluid liquid biopsy to detect circulating tumor DNA may be a good option for evaluating treatment response and tumor burden in patients with PCNSL.PCNSL is a challenging disease for treatment as these patients present with different neurological states and comorbidities.Treatment has evolved over the years from whole brain radiotherapy to HD-MTX followed by autologous stem cell transplant.Gradually,treatment of patients with PCNSL is going to become individualized.展开更多
Primary central nervous system lymphoma(PCNSL)is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes.This article aims to present a brief profile of PCNSL diagnosis...Primary central nervous system lymphoma(PCNSL)is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes.This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients.The authors retrieved information from the PubMed database up to September 2019.The annual incidence of PCNSL increased over the last four decades.The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate,which is now the backbone of all first-line treatment polychemotherapy regimens.Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-kB,B cell antigen receptor,and Janus kinases/signal transducer and activator of transcription proteins signal pathways.Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy.In particular,lenalidomide and ibrutinib have demonstrated durable efficiency.Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups,but there is still room to improve outcome by optimizing current chemotherapy and novel agents.展开更多
Introduction:Primary central nervous system lymphoma(PCNSL)is extremely rare in pediatric population.We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.Case presenta...Introduction:Primary central nervous system lymphoma(PCNSL)is extremely rare in pediatric population.We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.Case presentation:A 3-year-old girl presented with gait disturbance.A contrast-enhanced magnetic resonance image of the brain showed a solitary bulky mass in the left cerebellar hemisphere,hydrocephalus and cerebellar tonsillar hernia.Surgical resection was performed and the patient was diagnosed with primary central nervous system lymphoblastic B cell lymphoma.Then the patient received regular chemotherapy,including 6 cycles of chemotherapy containing high-dose methotrexate(HD-MTX).The patient remains alive 15 months after the diagnosis with no evidence of active disease,but suffered twice chronic subdural hematoma,which was treated by burr hole drainage.Conclusion:Lymphoblastic B cell lymphoma is a rare histologic subtype of pediatric PCNSL.Chemotherapy containing HD-MTX remains the most effective treatment.The patient should avoid head impact after surgical resection of the tumor to prevent chronic subdural hematoma.展开更多
目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的影像学诊断、治疗及预后,提高临床对PCNSL的认识。方法回顾性分析经术后病理证实的30例免疫正常的原发性中枢神经系统淋巴瘤患者的临床资料。单纯手...目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的影像学诊断、治疗及预后,提高临床对PCNSL的认识。方法回顾性分析经术后病理证实的30例免疫正常的原发性中枢神经系统淋巴瘤患者的临床资料。单纯手术8例,手术+化疗14例,手术+化疗+放疗8例。Kaplan-Meier法分析患者生存期。结果PCNSL临床表现主要为颅内压增高和神经功能缺损为主,误诊率高达93.3%。24例PCNSL(占80.0%)MRI明显均匀强化,表现为团块状及结节状强化,或出现具有特异性的"缺口征"、"尖角征"。病理检查均为B细胞淋巴瘤,以弥漫性大B淋巴瘤最多见。Kaplan-Meier分析全组中位生存期32.0个月,2年生存率46.7%。手术+化疗(中位生存期29个月)及手术+化疗+放疗组(34个月)的生存期高于单纯手术组(3个月)。手术+化疗组(64.3%)及手术+化疗+放疗组(62.5%)的2年生存率均高于单纯手术组(0)。放化疗患者肿瘤全切组与次全切组的生存率未见明显的统计学差异(P>0.05)。结论原发性中枢神经系统淋巴瘤影像学缺乏特异性,易误诊;单纯手术治疗不是首选,应尽早采取以放、化疗为主的全身综合治疗。展开更多
背景和目的:原发性中枢神经系统淋巴瘤(Primary central nervous system lymphoma,PCNSL)是一种罕见的原发并局限于脑和脊髓的非霍奇金氏淋巴瘤,治疗上主要采用高剂量MTX(HD-MTX)为主的化疗及放射治疗。本文探讨HD-MTX为主的化疗及放疗...背景和目的:原发性中枢神经系统淋巴瘤(Primary central nervous system lymphoma,PCNSL)是一种罕见的原发并局限于脑和脊髓的非霍奇金氏淋巴瘤,治疗上主要采用高剂量MTX(HD-MTX)为主的化疗及放射治疗。本文探讨HD-MTX为主的化疗及放疗对PCNSL的疗效及毒性。方法:回顾性分析1991～2004年间我院收治的17例PCNSL患者的临床资料。其中,单纯CHOP方案化疗2例,含HD-MTX方案化疗12例,MTX/Ara-C/DXM鞘内注射9例。13例患者接受放射治疗。其中,单纯全脑放疗2例,全脑+局部缩野照射8例,全中枢放疗2例;侵犯野照射1例。结果:随访时间最长120个月,中位随访时间24个月。死亡4例,2例死于肿瘤进展,1例死于复发,1例死于放化疗后感染。结论:含HD-MTX为主的化疗联合全脑照射是PCNSL治疗的主要模式,治疗毒性可以耐受。放疗在PCNSL治疗中的地位有待进一步研究。展开更多
目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点及诊治措施。方法总结我院1993年7月至2005年9月收治的16例免疫正常的PCNSL患者的临床资料。结果16例PCNSL,男11例,女5例,中位年龄52岁。...目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点及诊治措施。方法总结我院1993年7月至2005年9月收治的16例免疫正常的PCNSL患者的临床资料。结果16例PCNSL,男11例,女5例,中位年龄52岁。就诊时主要临床表现为颅内压增高、偏瘫和癫痫,肿瘤多位于幕上的额顶颞叶。11例行手术,4例行立体定向活检,均给予放化疗,2000年1月以前的10例患者行VM-26+BCNU化疗,2000年1月以后的6例患者均给予大剂量甲氨蝶呤化疗。2000年以前的10例患者已全部死于肿瘤复发,中位生存期为16个月。2000年以后的6例患者已有3例死亡,中为生存期为29个月。结论原发性中枢神经系统淋巴瘤是一种恶性肿瘤,无特殊临床表现和影像学特征。颅内压增高为主要表现,大剂量甲氨蝶呤化疗联合放疗治疗PCNSL有效。展开更多
目的原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是少见的非霍奇金淋巴瘤,近年来其发病率呈上升趋势,增长速度在颅内肿瘤中最高。该病临床特征无特异性,具有病程短、进展快、生存期短和预后差的特点。故...目的原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是少见的非霍奇金淋巴瘤,近年来其发病率呈上升趋势,增长速度在颅内肿瘤中最高。该病临床特征无特异性,具有病程短、进展快、生存期短和预后差的特点。故本文旨在通过总结PCNSL的治疗现状及研究进展,以提高对该病的认识。方法应用PubMed、CNKI数据库等检索,以"原发性中枢神经系统淋巴瘤,治疗方法,化疗,放疗,靶向治疗,自体造血干细胞移植"为检索词,检索1992-2017年的相关文献。纳入标准:与PCNSL治疗有关的文献。排除标准:(1)综述类文献;(2)研究资料缺失。根据上述标准最终共纳入分析45篇,英文文献39篇,中文文献6篇。结果 PCNSL目前尚无最佳治疗方案,以大剂量甲氨蝶呤为主的联合化疗成为PCNSL的一线方案。化疗联合放疗可延长生存期,但带来的神经毒性大。大剂量化疗联合自体造血干细胞移植可作为年轻初发及复发难治患者的选择。利妥昔单抗、替莫唑胺、噻替哌和培美曲赛等延长了PCNSL的生存期。新的靶点药物有待进一步研究。结论 PCNSL的治疗仍以甲氨蝶呤为基础的联合化疗为主,靶点药物治疗的疗效有所期待。展开更多
原发性中枢神经系统淋巴瘤(primary control nervous system lymphoma, PCNSL)是在脑、脊髓、脑膜或眼发生的罕见侵袭型非霍奇金淋巴瘤,无中枢神经系统之外的部位累及。PCNSL绝大多数来源于B淋巴细胞,极少数来源于T淋巴细胞。90%是弥漫...原发性中枢神经系统淋巴瘤(primary control nervous system lymphoma, PCNSL)是在脑、脊髓、脑膜或眼发生的罕见侵袭型非霍奇金淋巴瘤,无中枢神经系统之外的部位累及。PCNSL绝大多数来源于B淋巴细胞,极少数来源于T淋巴细胞。90%是弥漫大B细胞型,另有其他少见类型Burkitt或T细胞淋巴瘤。PCNSL占所有非霍奇金淋巴瘤的1%,占原发性颅内肿瘤的3%。目前尚无标准治疗方案。以大剂量甲氨蝶呤为基础的联合化疗已经成为首选治疗手段,巩固治疗主要包括全脑放疗和自体造血干细胞移植。针对PCNSL分子机制的新型治疗药物也取得了很大进展。本文重点对PCNSL目前的治疗研究进展进行综述。展开更多
文摘In this editorial I comment on the article,published in the current issue of the World Journal of Clinical Oncology.Primary central nervous system lymphoma(PCNSL)is a disease of elderly and immunocompromised patients.The authors reported clinical results of 19 patients with PCNSL treated with zanubrutinib/high dose methotrexate(HD-MTX)until disease progression.They demonstrated that the combination of zanubrutinib with HD-MTX led to a marked clinical response and tolerability among these patients.They also observed that cerebrospinal fluid liquid biopsy to detect circulating tumor DNA may be a good option for evaluating treatment response and tumor burden in patients with PCNSL.PCNSL is a challenging disease for treatment as these patients present with different neurological states and comorbidities.Treatment has evolved over the years from whole brain radiotherapy to HD-MTX followed by autologous stem cell transplant.Gradually,treatment of patients with PCNSL is going to become individualized.
文摘Primary central nervous system lymphoma(PCNSL)is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes.This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients.The authors retrieved information from the PubMed database up to September 2019.The annual incidence of PCNSL increased over the last four decades.The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate,which is now the backbone of all first-line treatment polychemotherapy regimens.Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-kB,B cell antigen receptor,and Janus kinases/signal transducer and activator of transcription proteins signal pathways.Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy.In particular,lenalidomide and ibrutinib have demonstrated durable efficiency.Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups,but there is still room to improve outcome by optimizing current chemotherapy and novel agents.
文摘Introduction:Primary central nervous system lymphoma(PCNSL)is extremely rare in pediatric population.We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.Case presentation:A 3-year-old girl presented with gait disturbance.A contrast-enhanced magnetic resonance image of the brain showed a solitary bulky mass in the left cerebellar hemisphere,hydrocephalus and cerebellar tonsillar hernia.Surgical resection was performed and the patient was diagnosed with primary central nervous system lymphoblastic B cell lymphoma.Then the patient received regular chemotherapy,including 6 cycles of chemotherapy containing high-dose methotrexate(HD-MTX).The patient remains alive 15 months after the diagnosis with no evidence of active disease,but suffered twice chronic subdural hematoma,which was treated by burr hole drainage.Conclusion:Lymphoblastic B cell lymphoma is a rare histologic subtype of pediatric PCNSL.Chemotherapy containing HD-MTX remains the most effective treatment.The patient should avoid head impact after surgical resection of the tumor to prevent chronic subdural hematoma.
文摘目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的影像学诊断、治疗及预后,提高临床对PCNSL的认识。方法回顾性分析经术后病理证实的30例免疫正常的原发性中枢神经系统淋巴瘤患者的临床资料。单纯手术8例,手术+化疗14例,手术+化疗+放疗8例。Kaplan-Meier法分析患者生存期。结果PCNSL临床表现主要为颅内压增高和神经功能缺损为主,误诊率高达93.3%。24例PCNSL(占80.0%)MRI明显均匀强化,表现为团块状及结节状强化,或出现具有特异性的"缺口征"、"尖角征"。病理检查均为B细胞淋巴瘤,以弥漫性大B淋巴瘤最多见。Kaplan-Meier分析全组中位生存期32.0个月,2年生存率46.7%。手术+化疗(中位生存期29个月)及手术+化疗+放疗组(34个月)的生存期高于单纯手术组(3个月)。手术+化疗组(64.3%)及手术+化疗+放疗组(62.5%)的2年生存率均高于单纯手术组(0)。放化疗患者肿瘤全切组与次全切组的生存率未见明显的统计学差异(P>0.05)。结论原发性中枢神经系统淋巴瘤影像学缺乏特异性,易误诊;单纯手术治疗不是首选,应尽早采取以放、化疗为主的全身综合治疗。
文摘背景和目的:原发性中枢神经系统淋巴瘤(Primary central nervous system lymphoma,PCNSL)是一种罕见的原发并局限于脑和脊髓的非霍奇金氏淋巴瘤,治疗上主要采用高剂量MTX(HD-MTX)为主的化疗及放射治疗。本文探讨HD-MTX为主的化疗及放疗对PCNSL的疗效及毒性。方法:回顾性分析1991～2004年间我院收治的17例PCNSL患者的临床资料。其中,单纯CHOP方案化疗2例,含HD-MTX方案化疗12例,MTX/Ara-C/DXM鞘内注射9例。13例患者接受放射治疗。其中,单纯全脑放疗2例,全脑+局部缩野照射8例,全中枢放疗2例;侵犯野照射1例。结果:随访时间最长120个月,中位随访时间24个月。死亡4例,2例死于肿瘤进展,1例死于复发,1例死于放化疗后感染。结论:含HD-MTX为主的化疗联合全脑照射是PCNSL治疗的主要模式,治疗毒性可以耐受。放疗在PCNSL治疗中的地位有待进一步研究。
文摘目的探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特点及诊治措施。方法总结我院1993年7月至2005年9月收治的16例免疫正常的PCNSL患者的临床资料。结果16例PCNSL,男11例,女5例,中位年龄52岁。就诊时主要临床表现为颅内压增高、偏瘫和癫痫,肿瘤多位于幕上的额顶颞叶。11例行手术,4例行立体定向活检,均给予放化疗,2000年1月以前的10例患者行VM-26+BCNU化疗,2000年1月以后的6例患者均给予大剂量甲氨蝶呤化疗。2000年以前的10例患者已全部死于肿瘤复发,中位生存期为16个月。2000年以后的6例患者已有3例死亡,中为生存期为29个月。结论原发性中枢神经系统淋巴瘤是一种恶性肿瘤,无特殊临床表现和影像学特征。颅内压增高为主要表现,大剂量甲氨蝶呤化疗联合放疗治疗PCNSL有效。
文摘目的原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是少见的非霍奇金淋巴瘤,近年来其发病率呈上升趋势,增长速度在颅内肿瘤中最高。该病临床特征无特异性,具有病程短、进展快、生存期短和预后差的特点。故本文旨在通过总结PCNSL的治疗现状及研究进展,以提高对该病的认识。方法应用PubMed、CNKI数据库等检索,以"原发性中枢神经系统淋巴瘤,治疗方法,化疗,放疗,靶向治疗,自体造血干细胞移植"为检索词,检索1992-2017年的相关文献。纳入标准:与PCNSL治疗有关的文献。排除标准:(1)综述类文献;(2)研究资料缺失。根据上述标准最终共纳入分析45篇,英文文献39篇,中文文献6篇。结果 PCNSL目前尚无最佳治疗方案,以大剂量甲氨蝶呤为主的联合化疗成为PCNSL的一线方案。化疗联合放疗可延长生存期,但带来的神经毒性大。大剂量化疗联合自体造血干细胞移植可作为年轻初发及复发难治患者的选择。利妥昔单抗、替莫唑胺、噻替哌和培美曲赛等延长了PCNSL的生存期。新的靶点药物有待进一步研究。结论 PCNSL的治疗仍以甲氨蝶呤为基础的联合化疗为主,靶点药物治疗的疗效有所期待。
文摘原发性中枢神经系统淋巴瘤(primary control nervous system lymphoma, PCNSL)是在脑、脊髓、脑膜或眼发生的罕见侵袭型非霍奇金淋巴瘤,无中枢神经系统之外的部位累及。PCNSL绝大多数来源于B淋巴细胞,极少数来源于T淋巴细胞。90%是弥漫大B细胞型,另有其他少见类型Burkitt或T细胞淋巴瘤。PCNSL占所有非霍奇金淋巴瘤的1%,占原发性颅内肿瘤的3%。目前尚无标准治疗方案。以大剂量甲氨蝶呤为基础的联合化疗已经成为首选治疗手段,巩固治疗主要包括全脑放疗和自体造血干细胞移植。针对PCNSL分子机制的新型治疗药物也取得了很大进展。本文重点对PCNSL目前的治疗研究进展进行综述。
