Temozolomide plus rituximab in the treatment of recurrent central nervous system lymphoma:Case report and literature review

Objective： The aim of our study was to investigate the treatment of recurrent central nervous system lymphoma. Methods： A case of recurrent central nervous system lymphoma in a 46-year-old male was treated with temozolomide 150 mg/m2 per day for 5 days; rituximab 750 mg/m2 on dl and d8, injected from Ommaya capsule to lateral ventricle, cycles were repeated every 28 days. Results： The patient achieved complete remission and the side effects was light after the treatment. Conclusion： Using this therapy method had certain curative effect on recurrent central nervous system lymphoma. Further studies should be needed on its indication.

Clinical Studies on Primary Central Nervous System Lymphoma:A Report of 31 Cases

OBJECTIVE To analyze the clinical manifestations, neuroimaging and pathological characteristics of primary central nervous system lymphoma （PCNSL） with a normal immunity, and to explore the methods of treatment and diagnosis. METHODS The clinical, laboratory, imaging data and pathological findings and therapeutic efficacy of 31 cases with pathologically proved PCNSL, during a period from July 1995 to June 2006, were analyzed retrospectively. The method of surgery, used in combination with chemotherapy and radiotherapy, was evaluated in 18 cases versus a simple surgical procedure used in 5. Among the total cases, a CHOP regimen was employed in 11 and Teniposide （VM26） plus Semustine （me-CCUN） was used in 7 cases. RESULTS PCNSL had a variety of clinical features, so that its misdiagnosis rate was high. The main clinical findings of PCNSL included intracranial hypertension and （focal） neurologic impairment. No positive result was found in the CSF cellular examination. All of the 31 cases were B-cell lymphoma. Twenty-four of the 31 cases were followed-up, with a follow-up period from 6 to 98 months. The median period of survival of the group who underwent surgery in combination with chemotherapy and radiotherapy was 20 months, while the group with simple surgical therapy was 10 months. CONCLUSION Specific clinical manifestations were usually absent in the patients with PCNSL, giving an uncertain preoperative diagnosis and a poor prognosis. Pathological examination is the only reliable method for a final diagnosis of the disease. The main objective of surgical therapy is to relieve the intracranial hypertension caused by the tumor. Recurrence may occur in a short period following the simple operation. Therefore combined therapy, i.e. surgery plus additional radiotherapy and chemotherapy, should be adopted. This is the key point for extending survival time and improving the quality of life.

Clinical analysis of primary central nervous system lymphoma with non-immune deficiency in 17 patients

Objective: The aim of this study was to investigate the clinical manifestations of primary central nervous system lymphoma (PCNSL) with non-immune deficiency and explore effective methods for its diagnosis and treatment. Methods: The clinical, imaging and pathological data from 17 cases with PCNSL in our hospital from March 2006 to April 2009 were analyzed. The immunologic function test for all 17 cases was confirmed as normal. Four of them received stereotactie brain biopsy while the other patients were given full or partial resection. Fifteen of them were given both radiotherapy and chemotherapy after surgery. High-dose Methotrexate (HD-MTX) (2.0g/m2) was used via intravenous infusion once per week for three times. From week 4, patients began radiotherapy. Six cases with abnormal cerebrospinal fluid were given whole central nervous system radiotherapy, and 9 cases with normal cerebrospinal fluid were given only whole brain radiotherapy. Two of them were without any additional treatment after surgery. Sixteen of 17 cases were followed up for 9-48 months. Therapeutic efficacy, toxic and side effect were investigated. Results: Six cases, who were given HD-MTX chemotherapy and whole central nervous system radiotherapy, had grade 3 leukopenia, but other toxic and side effect above grade 3 were not observed. Two patients having no chemotherapy and radiotherapy recurred in one month, but there was only one recurred case in three months among 15 cases who had both chemotherapy and radiotherapy. One of them lost fellow-up. The 2-year survival rate was 69.2%. Conclusion: There is no specific clinical manifestation for PCNSL. The pathological examination is a reliable method to confirm PCNSL. Recurrence may occur after surgery alone, however, the combination of HD-MTX chemotherapy and radiotherapy is an effective and safe therapeutic option, which might improve the treatment efficiency and survival rate.

Inflammatory myofibroblastic tumor of the central nervous system:A case report

BACKGROUND An inflammatory myofibroblastic tumor(IMT)occurring in the central nervous system is very rare,and thus its pathogenesis is unknown.This case report and literature review aimed to explore the pathogenesis,clinical features,imaging findings,pathological characteristics,immunohistochemical characteristics,diagnoses,treatments,and risks of postoperative recurrence of IMT in the central nervous system.CASE SUMMARY A 67-year-old woman was admitted to the hospital with an exophthalmic protrusion and double vision in the left eye that had persisted for 3 mo.Magnetic resonance imaging(MRI)showed a 2.4 cm×1.3 cm heterogeneous large mass in the bottom of the left anterior cranial fossa,which was closely related to the dura mater.Before surgery,we suspected the mass to be meningioma.The entire mass was successfully removed under neuronavigation and electrophysiological monitoring,and postoperative pathology indicated an IMT with extensive infiltration of chronic inflammatory cells and scattered multinucleated giant cells.Head MRI at the 3-mo follow-up showed that the tumor at the bottom of left anterior cranial fossa had been completely resected without recurrence.CONCLUSION From the histological,immunohistochemical,and genetic analyses,the present case suggests that the pathogenesis of IMT-CNS is related to autoimmunity.

Primary isolated central nervous system acute lymphoblastic leukemia with BCR-ABL1 rearrangement:A case report

BACKGROUND BCR-ABL1 fusion gene is associated with a poor prognosis and a high incidence in central nervous system(CNS)leukemia.CNS invasion which detected at the initial diagnosis is commonly with bone marrow infiltration.It is uncommon for the leukemia cells to be located primarily in the CNS without bone marrow involvement.CASE SUMMARY We here report the rare initial presentation of CNS-restricted BCR-ABL-positive acute lymphoblastic leukemia in a 30-year-old female patient who clinically manifested with leukemic meningitis,with no involvement in peripheral blood or bone marrow.Identification of abnormal phenotypes of blast cells,and BCR-ABL1 rearrangement in the cerebrospinal fluid alone established the diagnosis of primary CNS-isolated acute lymphocytic leukemia.The patient received a combination of intrathecal therapy and high-dose chemotherapy.But the benefits of the treatments were short-lived and she experienced recurrence.CONCLUSION Flow cytometry in combination with molecular genetic analysis improved diagnostic accuracy.New approaches that may enhance the efficacy of the existing therapies and cure CNS leukemia are required.

Primary central nervous system lymphoma:status and advances in diagnosis,molecular pathogenesis,and treatment

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.

Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab

Background:With current chemotherapy treatment,>90%of survival has been obtained for Burkitt lymphoma(BL).In this study,the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive(CNS+)BL.Methods:This retrospective study consecutively enrolled 78 CNS+BL patients in Beijing Children’s Hospital(BCH)from 2007 to 2019 who received the BCH B-cell non-Hodgkin’s lymphoma regimen(modified by French-American-British mature lymphoma B-cell 96[FAB/LMB96]C1 arm±rituximab).Clinical characteristics,methods of disease detection in the CNS,and outcomes were evaluated.Univariate and multivariate analyses were used to assess prognostic factors.Results:The median age of 65 boys and 13 girls at the time of diagnosis was 5.7 years(ranging from 1 to 14 years).Patients were followed up for a median time of 34 months(ranging from 1 to 72 months).Bone marrow invasion was found in 38(48.7%)patients.There were 48(61.5%),44(56.4%),and 25(32%)patients with cranial nerve palsy,intracerebral mass(ICM),and parameningeal extension,respectively.Abnormal cerebrospinal fluid(CSF)morphology and CSF immunophenotype appeared in 15(19.2%)and 15(19.2%)patients,respectively.There were 69(88.5%)patients treated with chemotherapy combined with rituximab,and nine patients were treated solely with chemotherapy.Finally,five patients died of treatment-related infection,recurrence occurred for 13,and one developed a second tumor.The 3-year overall survival and event-free survival rates were 78.9%±4.7%and 71.4%±6.0%,respectively.Treatment with chemotherapy only,ICM positivity,and>4 organs involved at diagnosis were independent risk factors.Conclusions:Rituximab combined with a modified LMB96 regimen has greatly increased the efficacy of treatment for Chinese children with CNS+BL,and with the continuous collection of outcome data,treatment-related complications are decreasing.For further verification,a large sample multicentre randomized controlled study should be performed to explore a treatment scheme for Chinese children with even greater efficacy.

Diagnosis, treatment, and misdiagnosis analysis of 28 cases of central nervous system echinococcosis

Background:To explore central nervous system(CNS)involvement in this disease,from the perspectives of diagnosis,treatment,and misdiagnosis Methods:Twenty-eight patients with CNS echinococcosis were included in this retrospective study,including 18 males(64.3%)and 10(35.7%)females.The average age of all the patients were 23.5 years(ranged 4–60 years).Twenty-three(23)patients(82.1%)received the first surgical resection in our hospital.Five(5)patients(17.9%)gave up surgical treatment for multiple-organ hydatidosis and previous surgery history at other hospitals,and albendazole was applied for a long-term(3–6 months)adjunct therapy for the 5 patients.The average follow-up time was 8 years.Results:For the 28 patients,23 cases received surgical treatments,and the diagnosis was confirmed by pathological examinations.The diagnosis of 4 cases of brain echinococcosis and 2 cases of spinal cord echinococcosis could not be confirmed,resulting in a misdiagnosis rate of 21.4%(6/28).For the pathological examination,a total of 17 cases were infected with Echinococcus granulosus(including 2 cases of spinal cord echinococcosis),and 6 cases were infected with Echinococcus alveolaris.Conclusion:The diagnosis should be specifically considered in endemic regions.The clinical features of CNS hydatidosis were intracranial space-occupying lesions.For the treatment,the surgical removal of cysts should be necessary.In addition,the adjuvant therapy with drug and intraoperative prophylaxis is also suggested.The misdiagnosis may have resulted from atypical clinical features and radiographic manifestations,as well as the accuracy of hydatid immunologic test.

Intraocular lymphoma

Intraocular lymphoma （IOL） is a rare lymphocytic malignancy which contains two main distinct forms. Primary intraocular lymphoma （PIOL） is mainly a sub- type of primary central nervous system lymphoma （PCNSL）. Alternatively, IOL can originate from outside the central nervous system （CNS） by metastasizJng to the eye. These tumors are known as secondary intraocular lymphoma （SlOL）. The IOL can arise in the retina, uvea, vitreous, Bruch＇s membrane and optic nerve. There are predominantly of B-cell origin; however there are also rare T-cell variants. Diagnosis remains challenging for ophthalmologists and pathologists, due to its ability to masquerade as noninfectious or infectious uveitis, white dot syndromes, or occasionally as other metastatic cancers. Laboratory tests include flow cytometry, immunocytochemistry, interleukin detection （IL-10： IL-6, ratio 〉1）, and polymerase chain reaction （PER） amplification. Methotrexate-based systemic chemotherapy with external beam radiotherapy and intravitreal chemotherapy with methotrexate are useful for controlling the disease, but the prognosis remains poor. Therefore, it is important to make an early diagnose and treatment. This review is focused on the clinical manifestations, diagnosis, treatment and prognosis of the IOL.

原发性中枢神经系统非霍奇金淋巴瘤1例报道并文献复习

目的探讨原发性中枢神经系统非霍奇金淋巴瘤(PCNSL)的病因,诊断方法及治疗措施。方法结合文献回顾性分析我院收治的1例PCNSL患者的临床及影像学资料,进行诊断及鉴别诊断。结果病理活检明确诊断为PCNSL,患者依方案行化疗治疗,随访1年未见病变复发。结论 PCNSL是一种罕见的颅内肿瘤,发病隐匿,对放化疗敏感,临床需与多种颅内肿瘤相鉴别。

28例原发性中枢神经系统淋巴瘤临床特征分析

目的 分析原发性中枢神经系统淋巴瘤（PCNSL）的临床特点,提高对PCNSL的诊治水平。方法 收集我院经病理及免疫组化确诊的PCNSL患者28例,回顾性分析患者临床诊治过程中的变化及特点。结果 28例中,经立体定向穿刺活检确诊5例,经开颅手术活检确诊7例,其余16例均经开颅单个病灶全切除或次全切除后病理确诊。确诊后,单纯化疗9例,生存期为13-43（25.22±9.35）个月;化疗＋放疗14例,生存期为10-62（31.14±14.48）个月。结论 PCNSL临床特点多变,临床表现缺乏特异性,病理确诊前诊断困难。临床表现结合特异性颅脑MRI影像可提示淋巴瘤。手术目的主要是减压并获得病理。肿瘤总体预后较差,确诊后放化疗是主要治疗手段。

31例原发性中枢神经系统恶性淋巴瘤临床分析

目的:分析免疫正常的原发性中枢神经系统恶性淋巴瘤患者的临床表现、影像学特点和病理表现并探讨其诊断、治疗方法。方法:回顾分析1995年7月至2006年6月经病理证实的31例原发中枢神经系统恶性淋巴瘤病例的临床、实验室检查、影像学、病理结果及治疗效果。18例采用手术+放疗+化疗方法,5例单纯手术治疗。其中化疗CHOP方案11例,替尼泊苷(VM26)+司莫司汀(me-CCNU)7例。结果:原发性中枢神经系统恶性淋巴瘤临床表现复杂,无特异性,主要为颅内压增高和神经功能缺损为主,误诊率高。脑脊液检查无阳性结果。31例均为B细胞淋巴瘤。31例中获随访24例,随访时间6～98个月,其中手术+放疗+化疗组中位生存期20个月,单纯手术组中位生存期10个月。结论:原发性中枢神经系统恶性淋巴瘤缺乏特异性临床表现,术前难以确诊,预后不良。病理检查是确诊的唯一方法。应采取综合治疗。手术的主要目的是解除肿瘤引起的颅内高压,单纯手术后,短时间内复发,应该采取手术、放疗、化疗综合治疗,这是延长生存期和改善生存质量的关键。

原发中枢神经系统淋巴瘤3例临床分析

目的探讨原发性颅内淋巴瘤的临床特点及诊疗措施.方法对临床收治的3例原发性颅内淋巴瘤临床资料进行回顾性分析,并参考近年国内外文献总结探讨.结果 2例给予手术近全切除,术后给予放化疗并随访,暂无复发.1例未行手术治疗,给予激素冲击治疗,复查肿瘤明显缩小.结论原发性颅内淋巴瘤为恶性肿瘤,术前明确诊断困难,预后较差,手术目的在于明确,性质,术后应施与积极放疗和化疗,提高患者生存质量.

原发中枢神经系统恶性淋巴瘤的诊断及治疗

目的:探讨原发中枢神经系统恶性淋巴瘤(PCNSL)的临床、影像学表现,治疗方案选择和预后。方法:对24例PCNSL的诊断、治疗过程、预后进行回顾性分析。结果:PCNSL的临床表现以颅内压增高、局灶占位性病变损伤症状为主,肿瘤可单发或多发;肿瘤影像学缺乏特异性,确诊依靠病理学诊断。结论:PCNSL恶性度高,预后不良,手术全切率低;对确诊病例采用个体化的手术方案结合术后放、化疗是治疗本病的关键,可显著延长患者生存时间。

HIV相关淋巴瘤诊治进展

HIV相关淋巴瘤是常见的HIV相关肿瘤之一,在联合抗反转录病毒治疗(combination antiretroviral therapy,cART)出现之前,其治疗效果极差。在cART时代,HIV相关淋巴瘤的基础及临床研究取得了重大进展,这些为挖掘治疗新靶点提供了依据,使优化HIV相关淋巴瘤的诊治成为可能。本文对近年来HIV相关淋巴瘤的诊断和治疗进展进行综述。

原发性中枢神经系统淋巴瘤24例临床分析

目的:探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)的临床特征、治疗及预后。方法:回顾性分析24例PCNSL患者的临床特征及治疗方案,并随访复发率及生存状况。结果: 24例PCNSL患者中,术前考虑为淋巴瘤的仅有6例,其余病例均为术后病理证实。术后病理结果为弥漫性大B细胞性淋巴瘤16例,中枢神经系统浆细胞瘤4例,浆细胞性骨髓瘤4例。术后20例患者进行了放化疗,2例进行了单纯化疗,2例由于经济原因再未进行任何辅助治疗。病变发生部位在大脑半球者16例,发生于小脑相应部位者4例,发生于脊髓者4例。术后病情稳定伤口拆线后,24例患者均转入血液科继续治疗。定期随访发现,其中16例生存(KPS评分为60~80 分),6例术后2个月内死亡,2例失访。结论: PCNSL 在中枢神经系统肿瘤中少见且极易误诊,多发于中老年男性,其病理结果大多数为弥漫性大B细胞性淋巴瘤。手术治疗既有利于疾病的确诊也可显著缓解患者颅内高压症状,再联合放化疗,可在一定程度上提高患者生活质量并延长生存期限。

伴发下丘脑功能异常的PCNSL 1例报告

淋巴瘤是起源于淋巴造血组织的恶性肿瘤,可以发生于身体的任何部位。原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)是一种罕见的结外形式的非霍奇金淋巴瘤,约占所有淋巴瘤的1%,占所有原发性中枢神经系统肿瘤的1%~3%[1]。PCNSL临床表现多种多样,本文通过报道1例以纳差、尿频为首发症状,伴发多种下丘脑功能紊乱表现的PCNSL,并通过文献复习总结本病特点,帮助大家认识此病,以期早期诊断、早期治疗,更好改善患者预后。

Progress in Research on 5-HTTLPR under Stress and Thyroid Papillary Carcinoma

The central nervous system plays an important role in regulating thyroid hormone homeostasis. 5-HT is one of the important neurotransmitters involved in energy balance regulation. 5-HT transporters affect 5-HT function by regulating SERT protein expression and transcriptional activity. Based on the biological correlation between thyroid hormone and thyroid tumors, 5-HTTLPR is associated with thyroid tumors under stress. Animal models of thyroid disease have shown that some of the transmitters in the brain’s monoaminergic nervous system have changed, and when the monoamine transporter gene expression is altered, it is found that this disorder is more pronounced. This article discusses whether thyroid papillary carcinoma patients are associated with 5-HTTLPR and whether the mechanism of thyroid papillary carcinoma “gene x environment” has an effect. Briefly describe the role of 5-HTTLPR in the development of patients with papillary thyroid carcinoma, and provide a basis for clinical diagnosis and treatment of papillary thyroid cancer.

原发性中枢神经系统淋巴瘤诊断及治疗专家共识(2024年版)

原发性中枢神经系统淋巴瘤(PCNSL)是罕见类型的结外非霍奇金淋巴瘤,病灶范围局限于脑实质、脊髓、软脑膜和眼。年发病率为(0.4~0.5)/10万。95%以上患者的病理类型为弥漫大B细胞淋巴瘤,表现为典型血管中心性生长模式。世界卫生组织(WHO)将PCNSL归类为弥漫大B细胞淋巴瘤的一个独特亚型,属于免疫豁免部位的大B细胞淋巴瘤。PCNSL是所有非霍奇金淋巴瘤中预后最差的类型,5年预估总生存率仅为30.5%。由于PCNSL临床罕见,对该疾病的认识尚未统一,诊治有待进一步规范。为加强我国临床医师对PCNSL的认知,提高诊断和治疗水平,中华医学会血液学分会淋巴细胞疾病学组、中国临床肿瘤学会(CSCO)淋巴瘤专家委员会相关专家参考国内外指南、共识并结合最新的研究进展,讨论并制定本共识。

中枢神经系统淋巴瘤2例并文献复习

恶性淋巴瘤为一组高度异质性疾病,常可合并有中枢的侵犯而呈中枢神经系统（central nervoussystem,CNS）淋巴瘤表现。原发中枢神经系统淋巴瘤（primary central nervous system lymphoma,PCNSL）则是一种只发生于CNS的恶性侵袭性淋巴瘤,其临床表现没有特异性。本文结合我科近年来收治的2例以CNS为首发症状的淋巴瘤,对PC-NSL进行分析及相关文献复习。