中枢神经系统原发性淋巴瘤32例临床分析

目的探讨中枢神经系统原发性淋巴瘤的诊断、治疗方法选择及预后。方法采用手术、病理及免疫组织化学方法证实32例淋巴瘤均来源于中枢神经系统,并进行临床分析。结果中枢神经系统原发性淋巴瘤多位于颅内幕上,也可发生于椎管内,多呈浸润性生长,边界较清楚,影像学检查无特异性表现,确诊要靠病理学检查;单纯手术不能延长患者的生存期,放疗、化疗有一定疗效,多数预后差。结论中枢神经系统原发性淋巴瘤恶性程度高、发展快,早期手术、病理证实后采用放疗和化疗是延长患者生存期的关键。     

原发性中枢神经系统淋巴瘤的治疗

目的探讨原发性中枢神经系统淋巴瘤的诊疗方法。方法回顾性分析2004年4月至2011年4月收治的35例原发性中枢神经系统淋巴瘤患者的临床资料,并结合国内外最新文献,分析其临床特点及诊疗方法。28例患者行手术治疗,5例行立体定向穿刺检查,2例行腰椎穿刺脑脊液脱落细胞学检查,所有患者均行放化疗。结果35例患者均证实为恶性淋巴瘤。治疗后临床症状改善32例,2例无改善,1例加重。结论原发性中枢神经系统淋巴瘤较为罕见,手术、放疗和化疗等综合治疗有望提高其疗效。     

原发性中枢神经系统淋巴瘤影像学及病理学特征临床研究

目的探讨原发性中枢神经系统淋巴瘤的临床表现、影像学和病理学特征。方法回顾分析6例经术后病理学检查证实的原发性中枢神经系统淋巴瘤患者的临床资料,分析其临床表现、影像学和组织病理学特征。结果原发性中枢神经系统淋巴瘤临床表现多样,以头痛、头晕多见。6例患者中肿瘤单发5例、多发1例,共7个病灶,病灶位于大脑半球4个、小脑1个、侧脑室1个、丘脑1个,其中2个病灶累及胼胝体。病灶多呈类圆形或不规则形,边界较清晰,肿瘤周围水肿和占位效应相对较轻。CT显示肿瘤呈高密度,T1WI呈等或略低信号、T2WI呈等或稍高信号、增强扫描肿瘤呈均匀强化。所有患者均行手术治疗。光学显微镜观察,肿瘤细胞围绕血管周围分布,形成"袖套"样浸润;免疫组织化学表型分析,肿瘤均来源于B淋巴细胞。结论原发性中枢神经系统淋巴瘤影像学和病理学表现具有一定特异性,主要病理学类型为弥漫性大B细胞淋巴瘤,组织学形态和免疫组织化学表型分析是明确诊断的"金标准"。     

位于蝶骨嵴的原发性中枢神经系统淋巴瘤临床分析

目的探讨位于蝶骨嵴处原发性中枢神经系统淋巴瘤(PCNSL)的诊断及治疗。方法回顾性分析3例位于蝶骨嵴的PCNSL病人的临床资料。2例接受手术治疗,二期均接受化疗。1例行立体定向活检后接受联合放化疗。结果手术病人镜下肿瘤全切除,随访25个月均存活。接受放化疗病人肿瘤体积变小,生存期仅为20个月。病理结果均为PCNSL。结论位于蝶骨嵴的PCNSL少见,需结合临床特点和影像学表现来综合判定,早期提供合理综合治疗方案以改善预后。     

原发性中枢神经系统淋巴瘤5例报告

目的回顾分析5例原发性中枢神经系统淋巴瘤（PCNSL）患者的临床资料，探讨其合理治疗方式及影响预后的因素。方法对本病的临床特征，病理学检查，治疗方法结果及预后因素进行分析。结果PCNSL患者的临床表现复杂，颅高压和神经系统损害的症状和体征为主要表现。结论PCNSL的临床表现或影像学均缺乏特异性表现，术前诊断十分困难，脑活组织病理检查是确诊本病的唯一手段。发病机制不明确，预后差。手术不能根治，对延长生存期无益，其作用限于明确组织学诊断，降低颅压，结合放疗化疗行综合治疗。     

原发性中枢神经系统淋巴瘤影像学特征和疗效分析

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。     

儿童非典型畸胎瘤样/横纹肌样瘤15例诊治体会

目的总结儿童中枢神经系统非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumor,AT/RT)的临床特征及预后,以提高大家对其的认识。方法通过回顾性分析我科近两年来收治的15例原发于儿童中枢神经系统AT/RT,详细分析其临床特征、影像学表现、病理学表现、手术治疗,并对其进行有效的随访,详细分析其预后。结果 15例患者均接受手术治疗,其中颅内13例,椎管内2例,手术全切除12例,次全切除3例,术后病理均证实为AT/RT。术后都得到有效随访,除1例椎管内AT/RT患者术后完成化疗后至今7个月仍未见肿瘤复发外,其余14例均在术后1个月至9个月不等死亡。结论 AT/RT好发于婴幼儿中枢神经系统,恶性度高,确诊有赖于病理,争取手术全切除结合术后放化疗是目前主要治疗方法,总体预后差。     

中枢神经系统原发性淋巴瘤32例临床分析

目的：探讨中枢神经系统原发性淋巴瘤的诊断、治疗方法选择及预后。方法：采用手术、病理及免疫组织化学方法证实32例淋巴瘤均来源于中构神经系统，并进行临床分析。结果：中枢神经系统原发性淋巴瘤多位于颅内幕上，也可发生于椎管内，多呈浸润性生长，边界较清楚；影像学检查无特异性表现，确诊要靠病理检查；单纯手术不能延长患的生存期，放疗和化疗有一定疗效，多数预后差。结论：中枢神经系统原发性淋巴瘤恶性程度高，发展快，早期手术、病理证实后采用放疗和化疗是延长患生存期的关键。     

颅内原发性淋巴瘤13例分析

目的探讨原发性中枢神经系统淋巴瘤的临床诊断和治疗。方法回顾性分析我院1997—02-2008—12经病理证实的13例原发性中枢神经系统淋巴瘤的临床特点及治疗结果。结果13例病人中8例手术切除,5例立体定向活检。本组13例病人共22个肿瘤,其中7例（53．8％）为单发肿瘤,6例（46．2％）为多发性肿瘤。肿瘤多位于额叶、颞叶部及基底节区,12例（92．3％）肿瘤位于小脑幕上。病理学检查发现病人均为B细胞来源。13例患者均行化疗及放疗,生存时间为6～51个月,平均23．5个月。结论原发性中枢神经系统淋巴瘤术前诊断困难,预后差,诊断主要依靠病理。该病多采用综合治疗,手术治疗,辅以化疗及放疗。     

颅骨海绵状血管瘤手术治疗临床分析

目的探讨原发性颅骨海绵状血管瘤的临床表现、影像学特征、病理学特点及治疗措施。方法回顾分析6例原发性颅骨海绵状血管瘤患者的临床资料;并结合文献对其临床表现、影像学特点、治疗方法进行分析。结果本组患者的原发性颅骨海绵状血管瘤为单发,临床症状各异。头颅CT表现为肿瘤呈"日光放射征"改变,周边骨质硬化。头颅MRI示,肿瘤呈T_1WI不均匀中-高信号,T_2WI不均匀高信号,增强扫描不均匀强化。手术切除包括肿瘤在内的颅骨及周边硬化颅骨后,患者的临床症状均消失,预后良好。术后病理检查示,病变表现为蜂窝状肿块,由充填有多量血细胞的囊状血管组成。结论原发性颅骨海绵状血管瘤较为罕见,手术治疗效果确实可靠;术后病理检查为其最终确诊的有效手段。     

原发性中枢神经系统淋巴瘤的影像学变化及临床(附1例报告及文献复习)

目的分析原发性中枢神经系统淋巴瘤MRI的动态变化及临床特点。方法对1例原发性中枢神经系统淋巴瘤患者的MRI变化、临床表现、病检结果,结合相关文献进行讨论分析。结果患者临床上反复发作,影像学上表现不典型,多在基底节、胼胝体和脑室周围,常误诊为多发性脑梗死或脱髓鞘疾病。结论原发中枢神经系统淋巴瘤临床上和影像学上酷似脱髓鞘病,行CT引导下立体定向穿刺或病灶组织活检有助于确诊。     

Lymphoma of the central nervous system in AIDS

Central nervous system (CNS) lymphoma is a common complication of patients with HIV infection occurring in as many as 20% of patients with AIDS. This article reviews current observations on primary CNS lymphoma and systemic AIDS-related lymphoma with CNS involvement. Clinical features, diagnosis, differential diagnosis, clinical course, and therapeutic options are herein reviewed.     

Salvage therapy for primary CNS lymphoma with a combination of rituximab and temozolomide

The authors evaluated the efficacy of a combination of rituximab and temozolomide for recurrent or refractory primary CNS lymphoma (PCNSL). Fifteen patients with a median age of 69 years had a 53% objective response rate with acceptable toxicity. Median overall survival is 14 months and median progression free survival of responding patients is 7.7 months. This combination merits further study and provides a reasonable therapeutic alternative for older patients with progressive PCNSL.     

原发性脑内恶性淋巴瘤15例临床分析

目的探讨原发性脑内恶性淋巴瘤的临床特点、诊断及治疗方法.方法回顾性分析15例原发性脑内恶性淋巴瘤的影像学表现、治疗结果及病理学特征.结果病变位于幕上12例,幕下3例;多发者8例.CT通常示低密度,瘤周中度水肿,均匀增强.MR通常示长T1长T2信号,均匀增强.术前无一例确诊.病理示大B细胞淋巴瘤弥漫型11例,局限型2例,小B细胞淋巴瘤1例,无裂细胞型1例;免疫组化:LCA阳性率为100%.中位生存期:肿瘤全切除后化疗加放疗16个月,肿瘤部分切除后化疗加放疗12个月,部分切除加化疗11个月.结论原发性脑内恶性淋巴瘤常发生于幕上大脑半球,术前误诊率高,预后差,综合治疗可提高生存期.     

Primary central nervous system lymphomas and related diseases: Pathological characteristics and discussion of the differential diagnosis

Although primary diffuse large B‐cell lymphomas of the CNS are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue in 2008, a variety of other lymphomas (Burkitt lymphomas, EBV‐positive diffuse large B‐cell lymphoma of the elderly) and related diseases (lymphomatoid granulomatosis) that are also found in the CNS have been spotlighted in recent years. The histopathology of primary CNS Burkitt lymphomas mimics that of primary diffuse large B‐cell lymphomas of the CNS after steroid administration. Therefore, for correct diagnosis of the involved lymphoma, comprehensive fluorescent in situ hybridization analysis for c‐MYC and BCL2 is recommended in all primary CNS lymphoma cases with aggressive clinical course, multifocal involvement of the CNS, and a high proliferation index. The pathological characteristics of primary CNS EBV‐positive diffuse large B‐cell lymphoma of the elderly have similarities with those of the latency phenotype III, EBV lymphoproliferative disorders that arise in the setting of immunodeficiency. These age‐related lymphomas usually occur in elderly immunocompetent patients, and the incidence of this disease was estimated to range from 4.0% to 13.6% of all primary CNS lymphomas. Shorter overall survival has been reported for patients with this disease. Lymphomatoid granulomatosis (LYG) is a systemic, EBV‐driven, angiocentric and angiodestructive lymphoproliferative disorder. Primary LYG that shows distinct clinicopathological features compared with systemic LYG was recently reported. Finally, this review focuses on the relationship between primary CNS lymphomas and demyelinating diseases, and the concomitant use of intraoperative cytology and frozen sections that are helpful in rapid intraoperative diagnosis.     

脑结核瘤的临床分析及文献回顾

目的 :分析脑结核瘤的临床特点 ,提高临床诊断准确率及治疗效果。方法 :结合文献对 1例多发性脑结核瘤患者的临床特点、影像学表现及治疗效果进行回顾性分析。结果 :该例脑内结核瘤粟粒样分布 ,病灶多达 15个以上 ,同时累及大脑、小脑及脑干 ,抗结核治疗后病灶缩小 ,数目明显减少。结论 :脑结核瘤的诊断需结合临床特点、影像学表现及抗痨效果综合判定。药物治疗是有效的 ,但需长程、规则。     

原发性中枢神经系统恶性淋巴瘤

目的 分析原发性中枢神经系统恶性淋巴瘤（ＰＭＬ－ＣＮＳ）的临床及影像学表现与病理所见,加深对本病的认识。方法 回顾分析１９８９年１月至１９９９年７月经病理结果证实的１５例ＰＭＬ－ＣＮＳ病例的临床、影像学及病理检查结果。结果 ＰＭＬ－ＣＮＳ临床表现多变,但其主要临床表现为颅内压增高和神经功能缺损（肢体无力、言语障碍和癫痫发作等）,半数以上的病人有头痛、视乳头水肿等颅内压增高的表现,易误诊为其他脑瘤;约２０％的病人表现类似脱髓鞘疾病,易误诊为多发性硬化。结论 综合分析临床及影像学检查结果,有助于提高本病的诊断率;活检是确诊的必要手段。     

中枢神经系统原发性黑色素肿瘤的临床特征及病理特征分析

目的探讨中枢神经系统原发性黑色素肿瘤的临床表现、病理特征及手术效果。方法回顾性分析2003年3月至2017年2月手术治疗的3例中枢神经系统原发性黑色素性肿瘤的临床资料,1例位于颞叶,1例位于小脑,1例位于右侧桥小脑角区。结果 2例全切,1例次全切。术后随访1~3个月,3例一般情况良好。结论中枢神经系统原发性黑色素肿瘤确诊依赖病理检查,临床分型对判断预后有重要意义。     

HIV-associated primary CNS lymorbidity and utility of brain biopsy

INTRODUCTION: Human immunodeficiency virus (HIV) infection is associated with several central nervous system (CNS) infections and neoplasms. These opportunistic processes generally occur with advanced immunosuppression, but if an accurate diagnosis is made, effective treatment can frequently be initiated. METHODS: In an attempt to assess the safety, diagnostic yield, and utility of stereotactic brain biopsy in the clinical management of suspected HIV-associated primary CNS lymphoma, we retrospectively studied the performance of biopsy in HIV-seropositive patients presenting with focal intracranial lesions. This analysis included 435 patients undergoing brain biopsy, identified through a local case series (n=47) combined with all published cases (n=388). The years of analysis for this study were 1984 and 1997. We also assessed the survival of HIV-associated intracranial mass lesions and of PCNSL patients treated at JHU. RESULTS: Definitive histopathological diagnoses were established in eighty-eight percent of biopsied cases: primary CNS lymphoma (PCNSL) (30%), CNS toxoplasmosis (CNS TOXO) (16%), progressive multifocal leukoencephalopathy (PML) (25%), and other specific diagnoses (17%). Post-biopsy morbidity within thirty days was 8.4% and mortality was 2.9%. PCNSL was the most common diagnosis among cases biopsied after failure of anti-toxoplasmosis therapy, 134/205 (65%). In the local case series, biopsy-related morbidity was associated with poor functional status, decreased platelet count, and number of lesions at presentation. The median survival of irradiated PCNSL cases was 29 days longer than untreated cases (median survival 50 days versus 21 days, respectively, Chi-square=6.73, P<0.01). DISCUSSION: Stereotactic brain biopsy had a high diagnostic yield for HIV-associated focal intracranial lesions, however, the biopsy complication rate in this patient population was relatively high. PCNSL was diagnosed in the majority of patients failing anti-toxoplasmosis therapy. Survival after irradiation for PCNSL remains very poor.     

Leukoencephalopathy and Disseminated Necrotizing Leukoencephalopathy Following Intrathecal Methotrexate Chemotherapy and Radiation Therapy for Central Nerve System Lymphoma or Leukemia

Objective

Intrathecal methotrexate (MTX) therapy combined with whole brain radiotherapy (WBRT) is one of the major treatment modalities for leukemia and lymphoma involving the central nervous system (CNS). The purpose of this study was to retrospectively determine the incidences of leukoencephalopathy and disseminated necrotizing leukoencephalopathy (DNL) following intrathecal MTX therapy for CNS lymphoma or leukemia and to assess the potential risk factors.

Methods

Between January 2000 and August 2009, 143 patients with CNS lymphoma or leukemia received intrathecal MTX therapy alone or in combination with WBRT at a single institution. Patients were followed up clinically and radiologically at regular two- or three-month intervals. Medical records were reviewed to obtain information regarding the patients'' demographics, medical histories, radiologic characteristics, treatments, and clinical courses.

Results

On follow-up MR images, leukoencephalopathy was found in 95 of 143 patients (66.4%). The median time to develop leukoencephalopathy was 6.6 months. Among those with leukoencephalopathy, four patients showed seven extensive white-matter changes with strongly enhancing lesions demonstrating DNL. Histological confirmation was done in six lesions of three patients and radiological diagnosis alone in one patient. Four lesions spontaneously disappeared on MR images without any treatment, with a mean duration of 14 months before disappearance of DNL.

Conclusion

Leukoencephalopathy is a common phenomenon that occurs following intrathecal MTX therapy; however, DNL occurs at a very low incidence. For newly developed enhancing lesions, consideration for the occurrence of DNL should be taken to avoid unnecessary invasive procedures or therapies.