儿童非霍奇金淋巴瘤的临床及病理特点

目的:对73例儿童非霍奇金淋巴瘤临床资料进行回顾性分析,明确其临床特点、病理类型及与疗效的关系。方法:收集2014年10月至2018年10月上海市儿童医院73例初诊非霍奇金淋巴瘤患儿资料,其中男52例,女21例;年龄2～13岁。对所有患儿发病的起始或累积器官、病理诊断、疾病分期、手术及疗效等进行综合评估。结果:73例儿...     

儿童肝脾γδT细胞淋巴瘤的临床病理分析

目的探讨儿童肝脾γδT细胞淋巴瘤的临床病理和免疫表型特征。方法应用组织病理和免疫组化方法分析2例儿童肝脾γδT细胞淋巴瘤的骨髓和肝脏活检标本,并对相关文献进行调研。结果本文报道的2例肝脾γδT细胞淋巴瘤为9岁女孩,主要表现发热、肝脾肿大和血细胞减少,无淋巴结肿大。其中1例合并慢性活动性EB病毒感染和噬血细胞综合征,诊断时有骨髓受累。2例肝脏针刺活检病理均示中等大小非典型淋巴细胞的窦内浸润,免疫组化示CD3+CD4-CD8-Granzyme B+EBER-。结论肝脾γδT细胞淋巴瘤是一类罕见的结外侵袭性外周T细胞淋巴瘤,典型特征包括:全身症状,肝脾肿大,血细胞减少,肝脾和骨髓的窦内有CD2+CD3+CD4-CD5-CD7+CD8-TCRγδ+表型淋巴细胞的浸润,7号染色体长臂等臂畸形和8号染色体三体同时出现的细胞遗传学改变。骨髓或肝脏活检组织的免疫表型和TCR受体分析有助于此型肿瘤的诊断,目前尚无有效治疗方法。     

儿童非霍奇金淋巴瘤195例临床病理分析

目的探讨儿童非霍奇金淋巴瘤(NHL)的临床病理特点。方法收集1982年1月—2010年1月诊断的非霍奇金淋巴瘤病例195例,进行临床和病理分析。结果儿童NHL发病年龄高峰在6～8岁,男女之比为2.3∶1,Ⅲ期和Ⅳ期占49.2%。病理组织类型主要有淋巴母细胞性淋巴瘤(LBL)、Burkitt淋巴瘤(BL)、间变性大细胞性淋巴瘤(ALCL);88.8%LBL为T细胞性淋巴瘤,BL皆为B细胞性淋巴瘤,ALCL皆为T细胞性淋巴瘤。37.9%病例首发于淋巴结外组织,56.1%LBL患儿确诊时已累及1个以上部位。结论儿童NHL的临床及病理表现与成人NHL存在很大差异。免疫组化等技术对NHL的病理诊断有重要作用。     

恶性淋巴瘤65例

目的探讨小儿恶性淋巴瘤的临床和病理特点。方法根据WHO2000年淋巴造血组织肿瘤分类标准,回顾性分析65例儿童恶性淋巴瘤的临床、实验室资料及病理免疫组化结果。结果本组以非霍奇金淋巴瘤(NHL)更多见,NHL与霍奇金淋巴瘤(HL)比例为2.25∶1。发病年龄高峰在6~9岁,男女比例为3.5∶1。NHL主要以淋巴母细胞型、间变性大细胞型、弥漫性大B细胞型及Burkitt′s型多见,以周围淋巴结大、纵隔和腹部包块、骨髓受累等为主要表现,临床分期以Ⅲ~Ⅳ期为主;HL主要以混合细胞型为主,大部分以颈淋巴结无痛性大为首发症状,可伴腹腔淋巴结、脾脏及骨髓浸润。结论儿童NHL的临床特点及病理学类型与成人存在差异,而HL病理类型与成人相似。免疫组化在恶性淋巴瘤的诊断及鉴别诊断中起着重要的作用。     

儿童乙型肝炎病毒相关性肾炎74例临床病理分析

目的探讨儿童乙型肝炎病毒相关性肾炎（HBV-GN）的临床和病理特点。方法收集经肾活检确诊为HBV—GN的74例患儿入院时临床表现（水肿、血压）及实验室检查结果（尿常规、24h尿蛋白定量、乙肝5项、血清清蛋白、血HBVDNA等），并对其肾组织进行光镜（常规染色及特殊染色）及免疫组织化学检查（IgG、IgA、IgM、C3e、C4e、HBsAg、HBcAg）。根据血清HBVDNA水平不同将74例HBV—GN患儿分为高病毒载量组（HBVDNA〉1．0×10^8copies／L）58例和低病毒载量组（HBVDNA〈1．0×10^8copies／L）16例，并比较分析其临床病理特点。结果临床表现：肾病综合征（NS）52例（70．3％）；血尿和蛋白尿13例（17．6％）；单纯性蛋白尿6例（8．1％）；单纯性血尿3例（4．1％）。病理分型：膜性肾病（MN）56例（75．7％）；系膜增生性肾小球肾炎（MsPGN）12例（16．2％）；膜增生性肾小球肾炎（MPGN）5例（6．6％）；毛细血管内增生性肾小球肾炎（EnPGN）1例。高病毒载量组与低病毒载量组入院时血清清蛋白[（22．04±7．69）g／L vs（31．95±5．83）g／L]，C3[（0．759±0．252）g/L vs （0．986±0．330）g／L]比较均有统计学差异（Pa〈0．05）。高病毒载量组病理类型多样，而低病毒载量组相对单一，以MsPGN、MN为主。结论HBV—GN患儿学龄期多见，且男多于女，临床表现以NS为主，病理类型以MN多见，血清HBVDNA水平与HBV—GN病变密切相关。     

儿童胰母细胞瘤的临床病理分析

目的通过分析儿童胰母细胞瘤（PB ）的临床表现、病理特点及预后等因素,提高对PB的认识。方法回顾性分析近5年我院PB患儿的临床资料。结果 PB患儿共9例,男5例,女4例,平均年龄5岁（2个月～12岁）;8例因偶然发现腹部包块就诊,1例产前（32周）超声检查提示胰腺占位就诊;腹部超声（n＝6）及CT（n＝7）检查均提示胰腺占位;2例外周血白细胞升高,3例血清甲胎蛋白（AFP）升高;8例行肿瘤切除,1例取瘤组织活检;1例术后第2年和第3年分别发生肝转移;肿瘤累及胰头部6例,体尾部2例,整个胰腺1例;肿瘤最大直径3～18 cm,平均10 cm,结节状,切面灰白色,实性,细腻,部分区域呈囊性,可见明显的出血及坏死;镜下：肿瘤富含细胞,由形态一致的上皮细胞构成,瘤细胞呈实性片巢状排列,混有分化好的腺泡样结构,可见特征性“鳞状上皮小体”;免疫组织化学染色显示有腺泡、内分泌和导管分化的证据,表现为胰酶、内分泌标记和癌胚抗原（CEA）的阳性表达。结论胰母细胞瘤是儿童胰腺最常见的恶性肿瘤,镜下显示有腺泡、内分泌和导管的分化及特征性鳞状小体结构,确诊主要依据病理检查结果,应注意与胰腺其它肿瘤鉴别。首选的治疗方案是肿瘤的完整切除。预后较成人好。     

儿童畸胎瘤80例临床病理分析

目的　分析儿童畸胎瘤的临床病理特点。方法　回顾 80例儿童畸胎瘤的光镜检查、免疫组化甲胎蛋白 (AFP)染色以及临床资料。结果　成熟畸胎瘤 6 0例 (75 0 % ) ,术前血AFP阳性 7例 ,组织免疫组化AFP弱阳性 1例 ;未成熟畸胎瘤 11例 (13 8% ) ,术前血清及组织免疫组化AFP均为阳性 10例 ;恶性畸胎瘤 9例 (11 2 % ) ,术前血清及组织AFP均为阳性 8例。结论　儿童畸胎瘤的生物学良恶性应在病理组织形态的基础上 ,结合患儿年龄、AFP值、肿瘤部位进行综合分析。     

200例儿童恶性淋巴瘤临床病理分析

目的 探讨儿童恶性淋巴瘤的临床和病理特点。方法 收集1982年～2002年6月12岁以下200例儿童恶性淋巴瘤病理资料，进行光镜观察、免疫组化分析，并进行统计学分析。结果 小儿非霍奇金淋巴瘤主要以淋巴母细胞性淋巴瘤(LB)、伯基特淋巴瘤及间变性大细胞性淋巴瘤(ALCL)为主；霍奇金淋巴瘤(HL)主要表现为混合细胞型、淋巴细胞为主型和结节硬化型3种亚型。未见淋巴细胞消减型病例。结外淋巴瘤主要发生在胃肠道，大部分为B细胞型。小儿恶性淋巴瘤的发病年龄高峰在6～7岁，发病时小于2岁者大部分为LB。除ALCL男女比为1：1外，其他各型男女比在1：0．14～1：0．43间。临床分期HL与ALCL以I～Ⅱ期为主，LB以Ⅲ期最多。大部分淋巴瘤表现为颈部、腋窝、腹股沟等外周淋巴结肿大。LB者53．73％发生纵隔淋巴结肿大。结论 小儿恶性淋巴瘤组织病理学特点与成人存在一定程度的差异。除ALCL外，其他各型淋巴瘤男性均明显高于女性，T细胞来源的淋巴瘤远多于B细胞淋巴瘤，LB预后最差，ALCL预后相对较好。HL与成人相似。免疫组化在小儿恶性淋巴瘤的诊断及鉴别诊断中起着十分重要的作用。     

霍奇金病67例病理形态与临床分析

目的分析霍奇金病(HL)的发生、病理及免疫分型。方法收集我院及南阳中心医院1998~2004年诊断HL标本,常规石蜡切片,HE染色,标准链菌素生物素过氧化物酶标记物法(SABC法)免疫分型,对其病理和临床相关资料进行分析。结果67例中非霍奇金病(NHL)50例(74.63%),HL12例(17.91%),未能分型5例(7.46%),Ⅰ~Ⅱ期19例(28.36%),Ⅲ~Ⅳ期48例(71.64%)。结论NHL和HL二者发病年龄、临床表现、部位和免疫均有所不同,免疫组织化学技术是提高本病不可缺少的检测手段。     

髓母细胞瘤组织bcl-XL mRNA和蛋白表达及临床意义

目的 研究bcl-XL基因在儿童髓母细胞瘤组织中的表达水平及意义。方法 应用免疫组织化学染色和原位杂交方法对41例儿童髓母细胞瘤（其中复发性肿瘤11例）和20例正常人脑组织中Bcl-XL蛋白和bcl-XL mRNA的表达水平进行检测，并与临床资料和预后进行比较。结果 儿童髓母细胞瘤组织免疫组织化学染色90．2％（37／41）表达Bcl-XL蛋白；原位杂交显示95．1％（39／41）表达bcl-XL mRNA。两者呈正相关。正常人脑组织Bcl-XL表达水平和强度显著低于髓母细胞瘤（P〈0．01）。复发性肿瘤Bel-XL表达水平和强度显著高于非复发肿瘤（P〈0．01），且98％复发性肿瘤在首次治疗时均有肿瘤在第四脑室底浸润。结论 凋亡抑制基因bcl-XL在儿童髓母细胞瘤的恶性进展中发挥重要作用。bcl-XL表达水平和强度和首次发病时肿瘤在第四脑室底的浸润是儿童髓母细胞瘤的重要预后因素。     

Neutrophil-rich Anaplastic Large Cell Lymphoma of the Skull Presenting after Head Trauma

The presentation of anaplastic large cell lymphoma in bone is uncommon. We report a case of anaplastic large cell lymphoma of the skull that was diagnosed after head trauma. Biopsy revealed significant destruction of the outer table of the frontal bone. Histopathologically, the initial evaluation suggested osteomyelitis because of a mixed inflammatory infiltrate with large numbers of neutrophils. However, several clusters and individual mononuclear cells were atypical. The tumor cells had large, pleomorphic nuclei; these cells stained positively with antibodies to Ki-1 (CD 30), ALK-1, and EMA. Fluorescence in situ hybridization (FISH) showed rearrangement of the ALK gene, which usually results from the t(2;5) translocation, present in most anaplastic large cell lymphomas. There was no evidence of systemic disease. The patient has tolerated chemotherapy and is free of disease 12 months later. Received April 24, 2000; accepted October 25, 2000.     

儿童NK/T细胞淋巴瘤的免疫表型及其与EB病毒的关系

目的探讨儿童NK/T细胞淋巴瘤的免疫表型特征及其与EB病毒（EBV）感染的关系。方法NK/T细胞淋巴瘤5例标本,采用免疫组织化学链霉素抗生物素-过氧化酶连接法（SP法）检测CD45RO、CD3ε、CD56、CD20、T细胞内抗原（TIA-1）、丝氨酸蛋白酶-粒酶B（Granzyme B）确定肿瘤细胞免疫表型及EBV潜伏膜蛋白（LMP-1）,原位杂交技术检测EBV编码的RNA（EBER1/2）。结果NK/T细胞淋巴瘤5例CD45RO、CD3ε、TIA-1和Granzyme B全部阳性,CD56阳性2例,CD20全部阴性。EBER1/2阳性4例,LMP-1阳性3例。结论EBV感染与儿童NK/T细胞淋巴瘤关系密切,EBV感染在儿童NK/T细胞淋巴瘤的发生发展中可能起重要作用。     

Cytogenetic Analysis of Childhood Endodermal Sinus Tumors: A Pediatric Oncology Group Study

Most adult germ cell tumors have a consistent cytogenetic abnormality, i(12p), and are aneuploid. Many pediatric germ cell tumors are biologically distinct from their adult counterparts, particularly endodermal sinus tumors (ESTs) of young children. We report cytogenetic and ploidy analysis of nine ESTs involving children under 3 years of age (four extragonadal and five testicular). Structural abnormalities were present in seven tumors and were identifiable in six: 5/6 had a structural abnormality of chromosome 1, usually terminal deletion of 1p; 5/6 showed 6q deletion; 3/6 had structural abnormalities of 3p; 2/6 showed abnormalities of chromosome 2. None showed an i(12p) or abnormality of chromosome 12. Ploidy analysis of the tumors correlated with the cytogenetic analysis; in particular, the tumor that was cytogenetically normal showed no aneuploid peaks. To determine if a marker chromosome was derived from chromosome 12 or if karyotypically normal cases included nondividing tumor cells, interphase fluorescence in situ hybridization using an alpha satellite probe for chromosome 12 was performed. These studies showed no evidence of an i(12p). We conclude that ESTs in young children show cytogenetic differences from their adult counterparts and that loci on 1p, 6q, and 3q need to be further studied.     

原发性回盲肠恶性淋巴瘤8例

目的探讨原发性回盲肠恶性淋巴瘤的临床特点、诊断及治疗。方法对小儿原发性回盲肠恶性淋巴瘤8例的临床特征、影像学表现、术中发现及病理资料进行回顾性分析。结果患儿8例均行根治性手术,术后辅以化疗和放疗2例,1例生存4年,1例生存1年失访;余6例患儿,2例生存2年仍在随访中,生存1、0.5年死亡各1例,另外2例失访。病理及免疫组织化学检查结果:CK(-),CD20( ),CD45RO(-),MPO(-),Ki67>60%、Ki67>75%各4例。结论原发性回盲肠恶性淋巴瘤临床表现复杂多样而缺乏特异性,组织病理学检查是确诊本病的主要手段,早期根治手术辅以化疗和放疗是主要治疗方法。     

Demonstration of Epstein-Barr Viral DNA in Paraffin-Embedded Tissues of Burkitt's Lymphoma from Argentina Using the Polymerase Chain Reaction and in Situ Hybridization

Burkitt's lymphoma (BL) is the most frequent non-Hodgkin's lymphoma in children in Argentina. Although epidemiologic studies have linked Epstein-Barr virus (EBV) to more than 90% of African BL cases but to only 10-20% of American and French cases, increased EBV-specific antibody titers were demonstrated in 73% of Argentinean patients with BL (1). To characterize the relationship between EBV and BL in Argentina, we analyzed paraffin-embedded tissues from 16 cases of BL for the presence of EBVDNA using the polymerase chain reaction (PCR) and in situ hybridization (ISH). PCR analysis showed that only 4 of 16 specimens contained the EBV BamW fragment, and these specimens were all from cases diagnosed in 1984. Results of ISH performed with a specific biotinylated DNA probe against the NotI/PstI fragment of EBV correlated with the PCR findings. EBV sequences were detected with ISH in 70-90% of the tumor cells from the 4 positive cases. These data may suggest an epidemic outbreak of EBV-related BL in 1984 superimposed on sporadic cases of BL, for which EBV may not have been an essential factor. This study also demonstrates the value of using molecular techniques on archival tissue to track epidemiologic trends.     

Lymphoblastic Lymphoma in Childhood and Adolescence

Lymphoblastic lymphoma (LBL) are thought to derive from immature precursor T-cells or B-cells. LBL are the second most common subtype of Non-Hodgkin Lymphoma (NHL) in children and adolescents. LBL are closely related to acute lymphoblastic leukemia (ALL), the most common type of cancer in children. Using ALL-type treatment regimen to treat children with LBL was an important development in the treatment of LBL. During the last decades, several systematic clinical trials contributed to the controlled optimization of treatment. Today event-free survival (EFS) can be achieved for 75–90% of patients. However, acute and long-term toxicity, the lack of prognostic parameters and the poor outcome for patients who suffer from refractory or relapsed LBL remain highly relevant subjects for improvement. To date, the pathogenesis of LBL is poorly understood. Learning more about the biology and pathogenesis of LBL might pave the way for targeted treatment to improve survival especially in relapsed and refractory patients.     

Follicular Center Cell Lymphoma of Childhood in Japan

Following a histologic review of 108 patients with childhood lymphoma from 20 hospitals in the Osaka, Hyogo, Nara, and Kyoto prefectures of Japan during the period 1964-1989, 16 cases (15%) were classified as follicular center cell (FCC) lymphomas. None had a follicular pattern of proliferation. The age range was from 2 to 15 years (median 10) with no peak incidence. The male/female ratio was 1.3:1. Presentation was nodal in 10 patients and extranodal in 6. Chronic infection and other predisposing factors for the development of FCC lymphomas in childhood are discussed, with a review of the literature.