儿童原发性骨淋巴瘤11例并文献复习

目的探讨儿童原发性骨淋巴瘤的临床表现、病理类型、治疗及预后等。方法回顾性分析首都医科大学附属北京儿童医院2016年1月至2020年1月收治的11例初诊为原发性骨淋巴瘤患儿的临床资料, 包括性别、发病年龄、原发受累部位、临床分期、病理类型、是否骨折及临床转归等, 并复习相关文献。结果 11例患儿均为初治原发性骨淋巴瘤, 中位发病年龄8.6岁(2.7～12.3岁), 其中男性7例, 女性4例;弥漫大B细胞淋巴瘤(DLBCL)7例, B淋巴母细胞淋巴瘤(BLL)3例, 间变性大细胞淋巴瘤(ALCL)1例;首发症状为骨痛8例, 局部肿胀1例, 跛行1例, 发热1例;临床分期Ⅰ期1例, Ⅱ期7例, Ⅳ期3例;最常见的受累部位为股骨、胫骨。所有患儿均依据不同病理类型接受化疗, 中位随访时间45个月(7～80个月)。10例完全缓解, 1例BLL患儿化疗缓解后因骨髓复发死亡。结论儿童原发性骨淋巴瘤临床表现隐匿, 以DLBCL多见, 接受规范治疗预后良好。     

原发性睾丸非霍奇金淋巴瘤33例临床分析

目的探讨33例原发性睾丸非霍奇金淋巴瘤的临床病理特点及预后因素。方法回顾性分析新疆医科大学附属肿瘤医院收治的33例原发性睾丸非霍奇金淋巴瘤,采用单因素分析、多因素分析和Kplan-Meier生存曲线分析评价该病的临床特征及预后因素。结果33例患者中,弥漫性大B细胞淋巴瘤27例（81.8%）,T细胞淋巴瘤3例（9.1%）,Burkitt淋巴瘤1例（3.1%）,未能分型的2例（6.1%）。Ⅰ期和Ⅱ期患者28例（84.8%）,Ⅲ期和Ⅳ期患者5例（25.2%）。全组5年总生存率为39.1%,5年无进展生存率为39.3%。单因素分析显示,LDH≥240 U/L（P=0.000）,临床分期≥Ⅲ（P=0.020）,IPI预后指数≥2（P=0.020）和初次治疗未达CR（P=0.000）的患者预后不佳。多因素分析结果显示,初次治疗达CR是影响预后的独立因素。结论原发性睾丸非霍奇金淋巴瘤的主要病理类型是弥漫性大B细胞淋巴瘤,其预后较其他部位恶性淋巴瘤差,手术切除后行以蒽环类为基础的化疗联合放疗是其主要的治疗手段。     

泌尿系统淋巴瘤16例临床分析并文献复习

目的 探讨泌尿系统淋巴瘤患者的临床特征、治疗效果及预后.方法 回顾性分析同济大学附属同济医院2009年1月至2016年4月收治的16例泌尿系统淋巴瘤患者的临床资料,对其临床特点、诊治经过及预后进行分析.结果 16例患者中位发病年龄68岁,其中14例以泌尿系统症状起病,10例影像学检查提示泌尿系统肿块.淋巴瘤起病部位:肾脏4例,肾上腺5例,睾丸5例,前列腺及尿道外口各1例.12例病理类型为弥漫大B细胞淋巴瘤,以非生发中心为主;12例Ann Arbor临床分期为ⅢE~ⅣE期;10例国际预后指数(IPI)评分≥3分;7例伴有B症状;10例患者通过手术确诊;14例接受含利妥昔单抗方案的化疗.初治完全缓解5例,部分缓解3例.结论 泌尿系统淋巴瘤患者诊断时多为晚期,病变侵袭性强.患者病理类型以弥漫大B细胞淋巴瘤为主,治疗主要采取手术联合放化疗,初治完全缓解率低,应重视早期诊断及治疗.     

睾丸恶性淋巴瘤26例临床分析

目的:睾丸恶性淋巴瘤临床较为少见,本文总结了该病的临床特点、诊断、治疗方法及其预后.方法:26例睾丸恶性淋巴瘤,原发22例(84.6%),继发4例(15.4%),所有患者均接受睾丸手术切除治疗,病理诊断明确.原发睾丸恶性淋巴瘤息者中可随访的为19例,其中3例仅接受手术治疗,14例接受了手术和化疗,2例患者接受了手术联合化疗和放疗.4例继发患者均接受了局部放疗和全身化疗.结果:全组原发患者的中位年龄57岁,首发症状均为无痛性睾丸肿大,主要病理类型为弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL),占84.6%(22/26),少见病理类型包括NK/T细胞淋巴瘤、淋巴母细胞淋巴瘤和B细胞不能分型者.所有原发患者的5年生存率为72%,Ann-Arbor分期Ⅰ～Ⅱ期患者的中位生存时间(median Burvival time,MST)为95.8个月,Ⅲ～Ⅳ期的MST时间为21.5个月.接受手术和化疗的患者的中位复发和MST分别为78.8和92.3个月.继发患者的中位复发时间为7.5个月,MST为25.0个月.结论:睾丸恶性淋巴瘤好发于老年男性,其预后可能与临床分期、病理类型和治疗方法有关.建议对所有患者在手术后进行放疗,并给予4～6个疗程的CHOP/CHOPlike方案化疗.     

皮下脂膜炎样T细胞淋巴瘤一例报告并文献复习

目的:报道1例皮下脂膜炎样T细胞淋巴瘤,探讨此类少见的皮肤T细胞淋巴瘤的临床和病理组织学特征以及治疗预后.方法: 分析1例皮下脂膜炎样T细胞淋巴瘤患者的临床资料并通过复习文献,总结该病的临床病理、免疫表型、治疗及预后特点.结果: 患者临床表现为发热、皮疹,皮肤活检病理示,真皮及皮下组织内大量核深染、形状不规则单个核细胞.免疫组化示,CD45RO弥漫阳性, CD3阳性,CD4阴性,CD8阳性,CD30部分阳性,TIA-1阳性, CD20阴性.CHOP方案化疗后一度好转,但很快复发,死于严重感染和多脏器功能衰竭.结论: 皮下脂膜炎样T细胞淋巴瘤是一种少见的皮肤T细胞淋巴瘤,预后差,免疫表型和T细胞受体基因重排检测在该病的诊断、治疗及预后等方面起着关键的作用.     

原发性肠道恶性淋巴瘤66例临床分析

目的探讨原发性肠道恶性淋巴瘤的发病、诊断、治疗及预后。方法临床资料回顾性分析。结果本病例占同期恶性淋巴瘤的12．9％,66例患者平均年龄35．8岁,临床上常见状是腹痛、腹块、腹泻、便血,主要井发症为肠套叠、肠梗阻。按AnnArbo会议分期：Ⅰ期13例,Ⅱ期27例（ⅡA21例,ⅡE6例）,Ⅲ期18例（ⅢA15例,ⅢE3例）,Ⅳ期1例。本组病例均经病理确诊,病理类型上均属非霍奇金淋巴瘤（NHL）,59例为B细胞淋巴瘤,5例T细胞淋巴瘤,2例组织淋巴瘤。结论肿瘤越大、病期越晚预后越差,病理类型以T细胞淋巴瘤预后最差;手术、化疗加放疗疗效最佳。     

原发鼻腔B细胞淋巴瘤临床特征及治疗分析

目的 探讨原发鼻腔B细胞淋巴瘤(PNBCL)的临床特点及治疗方法.方法 回顾分析2009年1月至2015年6月经病理证实的18例PNBCL的临床资料,对其临床表现、影像学特征、病理类型及治疗进行分析.结果 PNBCL临床多表现为鼻塞、流涕.18例患者年龄12~76岁,中位年龄51岁,男女比例为11:7;Ann Arbor分期ⅠE~ⅡE期15例,ⅢE~Ⅳ期3例;弥漫大B细胞淋巴瘤13例,套细胞淋巴瘤3例,伯基特淋巴瘤2例;血清乳酸脱氢酶增高4例;有B症状1例.CT检查示鼻腔异常软组织影,多位于单侧,可呈轻-中度均匀强化.18例患者中1例放弃治疗,14例接受单纯化疗,3例接受放化疗,治疗总有效率为82.3%(14/17).随访结束死亡5例,3年总生存率为54.5%(6/11).结论 PNBCL患者ⅠE~ⅡE期多见,B症状少见,病理分型中弥漫大B细胞淋巴瘤最多见.目前PNBCL的治疗首选化疗,可辅助放疗,但预后欠佳,化疗方案仍需进一步改进.     

原发扁桃体非霍奇金淋巴瘤89例临床分析

背景与目的：扁桃体是原发头颈部非霍奇金淋巴瘤（non-Hodgkin’s lymphoma,NHL）的最常见部位。本研究主要分析和总结原发扁桃体NHL的临床特点及治疗预后,探讨进一步改善预后和治疗后生存质量的可能途径。方法：回顾性分析1990年5月至2003年1月,89例初治原发扁桃体NHL患者的临床资料。按照改良的欧洲．美国淋巴瘤和WHO淋巴瘤分类原则进行病理分类。根据AnnArbor标准进行分期。Ⅰ期和Ⅱ期患者主要接受放化疗联合治疗,Ⅲ期和Ⅳ期患者以化疗为主。结果：89例患者中弥漫大B细胞型60例（67％）,外周T细胞型11例（12％）,惰性淋巴瘤5例（6％）,间变大T细胞型和T淋巴母细胞型各1例,未明确分类11例（12％）。Ⅰ期和Ⅱ期患者8l例（91％）,其中58例（72％）为放化疗联合治疗,19例（23％）为单纯放疗,3例（4％）为单纯化疗,1例（1％）为放化疗联合美罗华治疗。全组患者5年总生存率和无病生存率均为80％,Ⅰ期和Ⅱ期患者的5年生存率为84％。早期患者的生存率与原发耐药、复发和国际预后指数（internationalprognostic index,IPI）评分有显著性相关;而性别、年龄、病理类型、B症状、是否有巨块等对生存率均无显著性影响。结论：原发于扁桃体的NHL绝大部分为Ⅰ期和Ⅱ期的早期患者．因此预后较好。病理类型以弥漫大B细胞型最为多见。Cox回归分析显示在早期患者中原发耐药、复发和IPI〉1是影响预后的主要因素。     

原发性卵巢非霍奇金淋巴瘤1例及相关文献复习

目的:探讨原发性卵巢弥漫性大B细胞淋巴瘤的病因、临床表现、诊断、治疗及预后。方法:报道1例原发性卵巢弥漫性大B细胞淋巴瘤的临床病理资料及术后免疫组化结果,并阅读及复习国内外相关文献。结果:该例患者为（右）卵巢弥漫性大B细胞淋巴瘤,积极治疗后现考虑肿瘤复发。结论:原发性卵巢弥漫性大B细胞淋巴瘤罕见,确诊依赖于术后病理及免疫组化,预后不佳,常采用以手术为主,辅以化疗和放疗的综合治疗。     

原发性肾淋巴瘤的临床病理分析

  目的   探讨原发性肾淋巴瘤（primary renal lymphoma,PRL）的临床病理、诊断及预后特征。  方法   回顾性分析2011年1月至2016年7月就诊于郑州大学第一附属医院22例PRL患者的临床特征、病理学特征、免疫表型、诊断、治疗及预后。  结果  22例PRL患者的年龄2~72岁,平均年龄54.3岁,≥50岁为13例（59.1%）。病理学检查以非霍奇金淋巴瘤常见,其中B细胞淋巴瘤20例,T细胞淋巴瘤2例。PRL:7例生存,生存时间6~50个月;15例死亡,生存时间5~35个月。  结论   PRL临床比较罕见,临床表现及影像学表现特异性小,容易漏诊或误诊,组织病理学仍是确诊该病的金标准。组织学类型以B细胞性为主,弥漫性大B细胞性淋巴瘤（diffuse large B-cell lymphoma,DLBCL）最常见。根据随访结果得出生存时间与发病年龄、性别、组织学类型等有关。PRL暂无成熟的治疗经验,治疗方案亟待临床病例数的扩大,以及临床诊治经验的进一步积累。目前临床针对该病主要以行手术为主,辅助以化疗的综合治疗方式;对于局部晚期或高恶性度的肿瘤,单纯化疗通常疗效满意。      

原发性骨淋巴瘤3例并文献复习

目的：探讨原发性骨淋巴瘤的临床特征、治疗方法及预后.方法：对南京八一医院确诊为原发性骨淋巴瘤的3例患者的临床资料进行回顾性分析.结果：随访8月-11年,采用化疗联合放疗2例,均存活.单纯化疗1例,因病情进展已死亡.结论：原发性骨淋巴瘤为临床少见的结外淋巴瘤,以弥漫大B细胞性非霍奇金淋巴瘤多见,预后较好.通常见为溶骨性破坏,局部症状重而全身症状轻,影像学检查无特异性,造成早期准确诊断非常困难,容易发生误诊.确诊有赖于病理及免疫组化证实.治疗以放化疗综合治疗为宜.     

原发骨非霍奇金淋巴瘤26例的特点与治疗

 目的　回顾分析原发骨非霍奇金淋巴瘤（PLB）的临床特点和治疗疗效,结合文献探讨其合理的治疗方法。方法　收集并分析经病理证实的PLB 26例的临床资料,其中放化疗综合治疗23例（88.5 ％）,单纯化疗3例（11.5 ％）,3例还接受过手术治疗。结果　26例患者中,男性16例,女性10例,年龄8～70岁,中位年龄49岁。病理类型中,弥漫大B细胞型15例（57.7 ％）,小淋巴细胞型1例（3.8 ％）,B细胞来源未具体分型4例（15.5 ％）,T细胞型5例（19.3 %,其中间变大细胞型3例）,未分型1例（3.8 ％）。Ann-Arbor临床分期ⅠE期15例（57.7 %）,ⅡE期和ⅢE期各3例（11.5 %,11.5 %）,ⅣE期5例（19.3 %）。全组3年和5年生存率分别为59.16 ％和31.37 ％,11例患者死亡,其中3例为接受手术切除后出现原发灶复发,9例发生其他部位侵犯。中位剂量50 Gy的放射治疗未观察到放疗后骨折发生。结论　PLB以骨盆较常见,病理类型以弥漫大B细胞淋巴瘤居多,临床分期Ⅰ、Ⅱ期为主,多不伴有B症状。放化疗综合治疗仍是目前主要治疗手段,放疗中位剂量50 Gy较为安全可行。     

原发性骨淋巴瘤九例诊治分析

目的 分析原发性骨淋巴瘤(PBL)患者的临床特点、治疗策略及预后.方法 回顾性分析9例PBL患者的临床资料,所有患者原发病变均位于骨骼,无其他组织起病证据.患者均经病理及免疫组织化学确诊为PBL,临床及影像学检查均无特殊表现.结果 9例患者中,男6例,女3例,中位年龄34岁(15～71岁).全部患者均以局部疼痛或肿胀为首发症状,单发骨病灶7例,多发骨病灶2例.原发病灶部位:骨盆4例(髂骨3例、骶骨1例),股骨1例,肱骨1例,肩胛骨1例,椎体2例.其中2例接受放化疗联合治疗,3例进行化疗联合造血干细胞移植治疗,1例行化疗联合手术治疗,3例行单纯化疗.中位随访8个月,总体有效6例(完全缓解5例,部分缓解1例).结论 PBL临床少见,诊断标准和治疗方式仍在探索中,早期诊断和治疗预后较好.对于单发骨痛、软组织肿胀或不明原因骨折的患者,应高度警惕PBL的可能,尽早进行病理检查.     

Primary lymphoma of bone: a case report and review of the literature

The purpose of this study was to investigate the clinical signs and therapy of primary lymphoma of bone (PLB). One primary lymphoma of bone is described, and review of the pertinent literature is introduced. The patient achieved complete remission with no evidence of local recurrence. PLB are uncommon malignancies. Patients with PLB commonly present with local bone pain, soft tissue swelling and a mass or a pathological fracture. Most cases of PLB are classified as diffuse large B-cell lymphomas in the WHO classification of hematological malignancies. Comprehensive immunohistochemical studies are required to establish an accurate histological diagnosis of PLB. Early diagnosis and active treatment can improve the prognosis.     

Primary lymphoma of bone: long term results in patients treated with vincristine--adriamycin--cyclophosphamide and local radiation

Primary lymphoma of bone (PLB) is an unusual but well-recognized extranodal presentation of non-Hodgkin's lymphoma. In most series treatment with radiotherapy alone results in a cure rate of approximately 40%. Between January 1972 and December 1982, 26 patients with biopsy proven PLB were treated at the Rizzoli Institute with radiotherapy (4000-5,500 rad) and chemotherapy (vincristine, cyclophosphamide and adriamycin). The minimal clinical staging included complete blood cell counts and serum chemistries, bone radiography, full lung tomography, isotope bone scan, bone marrow biopsy and bipedal lymphoangiograms. At a median follow-up of 13 years (8.5-18.5) there were only three relapses (2 cases of meningeal involvement and 1 recurrence in another bone). No local recurrences nor second tumor in the irradiated field were observed. We conclude that the addition of chemotherapy to radiotherapy results in an excellent cure rate for patients with PLB.     

Primary Lymphoma of Bone: Long Term Results in Patients Treated with Vincristine - Adriamycin - Cyclophosphamide and Local Radiation

Summary

Primary lymphoma of bone (PLB) is an unusual but well-recognized extranodal presentation of non-Hodgkin's lymphoma. In most series treatment with radiotherapy alone results in a cure rate of approximately 40%.

Between January 1972 and December 1982, 26 patients with biopsy proven PLB were treated at the Rizzoli Institute with radiotherapy (4000-5,500 rad) and chemotherapy (vincristine, cyclophosphamide and adriamycin). The minimal clinical staging included complete blood cell counts and serum chemistries, bone radiography, full lung tomography, isotope bone scan, bone marrow biopsy and bipedal lymphoangiograms. At a median follow-up of 13 years (8.5-18.5) there were only three relapses (2 cases of meningeal involvement and 1 recurrence in another bone). No local recurrences nor second tumor in the irradiated field were observed. We conclude that the addition of chemotherapy to radiotherapy results in an excellent cure rate for patients with PLB.     

原发性骨淋巴瘤的诊疗进展

原发性骨淋巴瘤（PLB）是一类少见的结外淋巴瘤,占骨恶性肿瘤的3%~7%,占结外淋巴瘤约5%,占成年人所有淋巴瘤的2%。PLB可发生于任何年龄阶段,但好发于中老年人,平均发病年龄为45~60岁,男性略多于女性。PLB通常以病变部位疼痛或局部软组织肿胀起病,影像学表现无特异性,诊断必须通过手术取样行病理活组织检查来确诊。目前PLB以全身化疗联合受累骨放疗为主。文章对PLB的诊疗现状及进展进行综述。     

Treatment of localized primary non-Hodgkin's lymphoma of bone in children: a Pediatric Oncology Group study.

PURPOSE: The treatment of primary lymphoma of bone (PLB) in children has traditionally included radiotherapy to the primary site; more recently, it has included systemic chemotherapy. Because of concern about the untoward effects of treatment in a disease that is curable, we attempted to determine whether radiotherapy can be safely excluded from treatment. PATIENTS AND METHODS: The results of three consecutive Pediatric Oncology Group (POG) studies were examined to determine the impact on outcome of radiotherapy as adjunctive treatment in children and adolescents receiving chemotherapy for early-stage primary lymphoma of bone. RESULTS: From 1983 to 1997, 31 patients with localized PLB were entered onto POG studies of early-stage non-Hodgkin's lymphoma (NHL). Between 1983 and 1986, seven patients were treated with 8 months of chemotherapy with irradiation (XRT) of the primary site. After 1986, patients were treated without XRT; four received 8 months of chemotherapy, and 20 received 9 weeks of chemotherapy. Primary sites were the femur (nine), tibia (eight), mandible (five), mastoid (one), maxilla (one), zygomatic arch (one), rib (one), clavicle (one), scapula (one), ulna (one), talus (one), and calcaneous (one). Histologic classification revealed 21 cases of large cell lymphoma, five cases of lymphoblastic lymphoma, two cases of small, noncleaved-cell lymphoma, and three cases of NHL that could not be classified further. One patient relapsed at a distant site 22 months after completion of therapy. There have been no deaths. CONCLUSION: Localized PLB is curable in most children and adolescents with a 9-week chemotherapy regimen of modest intensity, and radiotherapy is an unnecessary adjunct.     

Primary non-Hodgkin's lymphoma of bone: a rare disorder with high frequency of T-cell phenotype in southern Taiwan

Primary non-Hodgkin's lymphoma of bone (PLB) is a rare disorder representing less than 1% of all non-Hodgkin's lymphomas and has rarely been reported in Taiwan. A retrospective clinicopathological study was performed according to the 2002 World Health Organization criteria and identified 14 cases during a 13-year period in 2 medical centers in southern Taiwan. There was male predominance (M:F = 6:1) with a median age of 42 and bone pain (6 patients, 43%) as the most common symptom. Half of the patients had monostotic and the other half polyostotic lesions. Axial skeletons (10 cases, 71%) were the most frequent sites of involvement. The staging results were stage I (9 patients, 64%), stage II (2, 14%) and stage IV (3, 21%). Eight cases (57%) were of B-cell phenotype and the remaining 6 (43%), T-cell. Histologically, 7 (50%) were diffuse large B-cell lymphomas (DLBCLs) and 5 (36%) anaplastic large cell lymphomas. Seven patients received chemotherapy and radiotherapy; 4 chemotherapy and 3 radiotherapy alone. Of the 11 patients with follow-up information, 6 (55%) died of disease within 1 year including 5 with T-cell lymphomas, while all the 5 patients surviving over 1 year were of B-cell phenotype. The overall 1-year survival rate was 45%. The survival of B-cell lymphomas was significantly better than T-cell tumors (p = 0.016, log-rank test). In summary, this study reported the largest series of PBL in Taiwan and confirmed that the majority was DLBCL and B-cell tumors had more favorable prognosis. As compared to the Western series, the cases showed a striking male predominance, higher percentage of axial skeleton involvement, higher relative frequency of T-lineage tumors and poorer prognosis.     

嗅神经母细胞瘤7例诊疗分析

目的:探讨嗅神经母细胞瘤的临床特点及诊断治疗方法,提高本病的诊疗水平.方法:回顾分析我科1 993年4月-2010年10月收治经手术和病理证实的7例嗅神经母细胞瘤的临床资料,综合文献,对其临床表现、诊断和治疗进行探讨0 7例中A期1例,B期4例,C期2例.结果:本组单纯放疗1例,手术+术后放疗4例,手术+术后放疗+化疗2例;本组病例1、3、5年生存率分别为100％,71.43％,57.14％.随访中发现颈部淋巴结转移由初次确诊的14.28％上升至42.85％.结论:嗅神经母细胞瘤的预后与Kadish分期、病理分化程度、治疗方式有关,病变位于鼻腔顶部且前颅窝骨质破坏提示本病的可能,早期诊断,手术+放疗等的综合治疗,能提高本病的生存率,晚期患者的辅助化疗是必要的.