Jejunal stricture: a rare complication of chemotherapy in pediatric gastrointestinal B-cell non-Hodgkin lymphoma

The use of intensive chemotherapy has led to remarkable improvements in the treatment of high-grade B-cell Non-Hodgkin lymphoma (NHL); however, it is associated with significant side effects such as myelosuppression and mucositis. Gastrointestinal NHL rarely leads to the development of aneurysmal dilatation of the bowel, as desmoplastic reaction is not a feature of NHL. Strictures and fibrosis are not a manifestation of NHL involvement. Here, we report a child with primary gastrointestinal B-cell NHL who presented with jejunal stricture developing as a sequela of severe chemotherapy-induced mucositis. The patient improved with surgical resection of stricture and end-to-end anastomosis.     

A new therapy schedule for pediatric non-Hodgkin lymphoma toxicity and preliminary results

A pilot study of the toxicity and efficacy of a new treatment schedule for childhood non-Hodgkin's lymphoma was conducted by members of the Children's Cancer Study Group (CCSG) prior to its use in a randomized phase III trial. Chemotherapeutic agents used were cyclophosphamide (CPM), vincristine (VCR), and prednisone, together with intravenous (IV) and intrathecal methotrexate (IT MTX). Radiation therapy was also employed. From September 1976 to April 1977, 27 eligible, newly diagnosed patients with non-Hodgkin's lymphoma were entered onto this pilot study. Toxicity was acceptable with minor adjustments in dosage and timing of the myelosuppressive agents. Fourteen of the 22 patients entered onto maintenance remain entirely disease-free, and all have completed the prescribed course of chemotherapy. None of the 12 patients characterized as having a “favorable” prognosis has relapsed, with a median follow-up of 27 months from on study.     

Granulomatous reaction in mediastinal B-cell non-Hodgkin lymphoma and intracardiac thrombosis

Epithelioid cell granulomas may be associated with several neoplasms. Lymphomas may mimic or are associated with epithelioid granulomas. In this article the authors report a child with granulomatous reaction in B cell non-Hodgkin lymphoma and intracardiac thrombosis. Although cancer alone is a risk factor for thromboembolism, thrombosis is a multifactorial disorder with both hereditary and acquired risk factors. This is the first reported case of intracardiac thrombosis with MTHFR A1298C and factor XIII V34L mutations together with granulomatous reaction in non-Hodgkin lymphoma.     

儿童弥漫大B细胞淋巴瘤12例临床分析

No abstract available     

MLL duplication in a pediatric patient with B-cell lymphoblastic lymphoma

Lymphoblastic lymphoma is the second most common type of non-Hodgkin lymphoma seen in children. Approximately, 90% of lymphoblastic lymphomas arise from T cells, with the remaining 10% being B-cell-lineage derived. Although T-cell lymphoblastic lymphoma most frequently occurs in the anterior mediastinum (thymus), B-cell lymphoblastic lymphoma (B-LBL) predominates in extranodal sites such as skin and bone. Here, we describe a pediatric B-LBL patient who presented with extensive abdominal involvement and whose lymphoma cells displayed segmental duplication of the mixed lineage leukemia (MLL) gene. MLL duplication/amplification has been described primarily in acute myeloid leukemia and myelodysplastic syndrome with no published reports of discrete MLL duplication/amplification events in B-LBL. The MLL gene duplication noted in this case may represent a novel mechanism for tumorigenesis in B-LBL.     

Surgical treatment of pediatric and adolescent papillary thyroid cancer: a retrospective study of 54 patients in a single center

ObjectiveIn 2015, American Thyroid Association (ATA) issued the first version of Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer. The purpose of this study is to evaluate whether the ATA pediatric guidelines recommended surgical approach for the patient can be applied to surgical treatment of pediatric PTC in China.MethodFrom April 2012 to December 2020, clinical data of children (≤18 years) with PTC consecutively admitted and treated with initial surgery in the study's department were retrospectively reviewed.ResultsThe authors found that the central lymph node metastasis (CLNM) rate was significantly higher than that in the lateral neck (83.33 % vs 62.96%, χ² = 5.704, p = 0.017) .The lymph node metastasis rate was significantly lower in cN1b (-) patients than in cN1b (+) patient (55.00% vs 100.00%, χ² = 15.263, p = 0.000); Meanwhile, the CLNM and LLNM rates of ipsilateral were significantly higher than those of contralateral central compartment (83.33?vs 57.41?%, χ² = 8.704, p = 0.003). Lymph nodes of 51 lateral lymph node dissection (LND) were analyzed, which revealed the LNM rate of cN1b (-) patients was significantly lower than that of cN1b (+) patients (55.00% vs. 100.00%, χ² = 15.263, p = 0.000).ConclusionChildren and adolescents have a higher rate of lymph node metastasis at the time of diagnosis. TT should be conducted in the majority of children with PTC. CND should be routinely performed; therapeutic LND is recommended for children with cN1b (+).     

Neurological complications of liver transplantation in pediatric patients: a single center experience

Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 +/- 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilson's disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilson's disease had a higher incidence of NCs (60%) than did patients without Wilson's disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.     

Treatment of children with B-cell non-Hodgkin's lymphoma in developing countries: the experience of a single center in Brazil

PURPOSE: To treat non-Hodgkin's B-cell lymphoma (B-NHL) in children with manageable toxicity-related morbidity and without any decrease in survival. PATIENTS AND METHODS: Between January 1998 and April 2003, 53 consecutive patients (age 16 years or less) from a single institution were enrolled. The patients were stratified by risk factors (stage and LDH level) and treated with a BFM 86/90 (Berlin-Frankfurt-Münster)-based protocol with reduction of the methotrexate dose from 5 mg/m to 2 mg/m. RESULTS: The mean age of the patients was 6 years (range 1-16 years). Seventy-two percent of the patients had lymphomas classified as Burkitt type, 11% as diffuse large cell lymphoma, and 6% as Burkitt-like lymphoma, and 11% were not classified. At a median follow-up of 35 months, 44 patients (83%) survived in complete remission. The event-free survival rate for all patients was 78% (SE = 0.07): 100% (SE = 0.0) for stage I/II patients and 74% (SE = 0.08) for stage III/IV patients. Six patients suffered initial treatment failure and one patient relapsed, all of whom died. There was only one death from sepsis related to treatment. CONCLUSIONS: This strategy was very effective for treating B-NHL in a developing country. The results were comparable to those of the BFM 90 study and other contemporary groups and represented an increase in the cure rates in childhood B-NHL in Brazil.     

单中心116例婴幼儿神经母细胞瘤临床特征及预后分析

目的分析≤18个月龄神经母细胞瘤(NB)婴幼儿的临床特征及影响预后因素。方法回顾性分析2007年1月-2017年6月在北京儿童医院血液肿瘤中心诊断并治疗的年龄≤18个月龄婴幼儿NB患儿的临床资料,总结临床特征、评估预后。应用SPSS 19.0软件进行数据分析。结果2007年1月-2017年6月共治疗116例≤18个月龄NB婴幼儿,男64例,女52例,中位年龄10(1～18)个月,随访中位时间19. 5(1～88)个月。单因素分析显示,INSS分期4期、高危、伴有骨骼或骨髓转移、MYCN基因扩增、初诊时血清乳酸脱氢酶(LDH)>500 IU/L的患儿预后不良(P <0. 05)。存在MYCN基因扩增10例,INSS分期均为3期或4期,LDH、神经元特异性烯醇化酶(NSE)明显高于无MYCN扩增病例,差异均有显著性(P=0. 028,P=0. 002,P=0. 001)。死亡病例13例,其中9例因肿瘤复发或进展死亡。116例NB婴幼儿3年无事件生存率(EFS) 83.6%,总生存率(OS) 86. 7%;5年EFS 76. 7%,OS 86. 7%。年龄≤12个月与年龄12～18个月患儿3年OS分别为90%、80. 7%,差异无显著性(P=0. 101)。INSS分期4期患儿3年OS 74. 4%、高危患儿3年OS 57. 1%、伴有MYCN基因扩增患儿NB 3年OS 26.7%,明显低于中早期、低中危、无MYCN基因扩增患儿(P=0.013,P=0.000,P=0.000)。结论≤18个月NB患儿预后良好,MYCN基因扩增、晚期病例预后差,主要死亡原因是疾病复发或进展。     

Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center

Background  

Juvenile systemic sclerosis (JSS) is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. It has a low prevalence, even in a tertiary facility setting. The purpose of the present study is to describe and analyze the clinical and laboratory characteristics of a group of children with JSS followed in a single center.     

小儿非霍奇金淋巴瘤的分型及治疗

目的 总结24例小儿非霍奇金淋巴瘤（NHL）的诊断及治疗。方法 采用组织病理及免疫组化诊断，并行临床分期，依据类型，分期选择治疗方案。结果 23/24例为晚期NHL，3例小无裂细胞型均为B细胞型，5例淋巴母细胞型均为T细胞型，大细胞型8例显示不均一的表现，治疗的完全缓解率为94.4%(17/18例）。10例长期治疗患儿目前无病存活7例。结论 依据病理，免疫类型及分期采用强化多药联合的大剂量化疗治疗小儿晚期NHL，可以达到较高的长期无病存活率。     

399例儿童畸胎瘤的单中心临床诊疗分析

目的回顾性分析小儿畸胎瘤的治疗及预后特点。方法收集重庆医科大学附属儿童医院1994年10月至2015年6月收治的畸胎瘤患儿,共399例,按照性别、年龄、部位、病理类型、血清AFP水平、治疗及预后等因素进行回顾性分析。结果①成熟型畸胎瘤301例;未成熟畸胎瘤17例,其中组织学Ⅰ级9例,Ⅱ级5例,Ⅲ级3例;恶性畸胎瘤81例,按COG/CCG肿瘤分期标准,Ⅰ期5例,Ⅱ期14例,Ⅲ期44例,Ⅳ期18例。②成熟型畸胎瘤及未成熟型畸胎瘤共318例一期接受手术治疗,其中未成熟畸胎瘤Ⅲ级及部分Ⅱ级病例术后辅以化疗;81例恶性肿瘤患儿中,Ⅰ、Ⅱ期均手术完全或完整切除肿瘤,部分Ⅱ期病例术后辅以化疗;40例接受术前新辅助化疗、手术治疗、术后化疗的个体化综合治疗;另外22例中断治疗并放弃;③术后随访1~10年,3例成熟型畸胎瘤患儿术后良性复发,再次予以完整切除后,截止随访时间,301例均无瘤存活;1例未成熟畸胎瘤Ⅲ级术后恶性复发,放弃治疗后死亡;恶性畸胎瘤完成治疗的患儿仅1例恶性复发后死亡,未完成治疗患儿中10例死亡,其余患儿各有不同程度的病情进展。结论在儿童畸胎瘤治疗中,一期完整切除肿瘤,避免肿瘤破溃是预防复发及恶变的关键,未成熟畸胎瘤依据AFP、影像学、肿瘤组织分级、病理免疫组化、术中情况等选择是否化疗;对于恶性畸胎瘤,彻底手术切除的同时结合化疗,术后密切随访,可获得较高的生存率。     

Clinical and epidemiological characteristics of retinoblastoma: correlation with prognosis in a Turkish pediatric oncology center

Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors' pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p < .01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p < .05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p < .001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p < .01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p < .01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.     

儿童闭塞性细支气管炎26例临床研究

目的 分析儿童闭塞性细支气管炎(bronchiolitis obliterans,BO)的临床特点、影像学特点、实验室检查、治疗方法与临床转归.方法 2009年6月-2011年4月期间广州医学院第一附属医院儿科病房诊断为BO的住院患儿26例.分析BO患儿病原学及临床特点、危险因素、影像学特点、实验室检查、治疗方法及治疗反应.结果 26例中男18例,女8例,发病年龄4.5个月～8岁;病程(6.2±3.5)个月,随诊时间在2个月～2年.临床表现为不同程度的持续喘息(26例,100％),反复咳嗽(24例,92％)、运动不耐受(22例,85％)、气促(21例,81％)、三凹征(20例,77％)、痰鸣(16例,62％)、肺部反复湿哕音(10例,38％)、唇周紫绀(3例,12％).未见杵状指(趾).18例(69％)病原学检测阳性,支原体11例(42％),呼吸道合胞病毒4例(15％),副流感病毒、流感病毒甲型、乙型各2例(8％),博卡病毒1例(4％),其中混合感染阳性者占8例(31％).胸部X线平片检查未见异常者16例(62％),肺炎样改变10例(38％),其中仅1例疑似肺间质改变.所有病例的胸部高分辨CT(HRCT)均有肺部空气潴留、肺灌注不良的马赛克征.19例患儿检测了抗中性粒细胞胞浆抗体(ANCA),其中PANCA阳性10例(53％),CANCA阳性8例(42％).所有患儿均使用口服、全身激素与低剂量阿奇霉素治疗,其中13例(50％)治疗后咳嗽、喘息的严重程度及发作频率有明显改善,喘息、三凹征改善的天数为(7.1±4.8)d;13例(50％)激素效果不理想,或HRCT无改变者加用甲氨蝶呤后喘息、三凹征改善,时间为(16.4±11.0)d.HRCT下病灶修复时间滞后于症状改善时间,HRCT最长随访时间是1.5年,大部分病例肺部病灶仅部分吸收好转,尚无一例病灶完全吸收.结论 呼吸道感染是儿童BO发生的重要病因,临床以长期持续的喘息、咳嗽、活动不耐受、气促、三凹征为主要表现,但缺乏特异性,胸部X线平片不能提供诊断的信息,HRCT提示的典型马赛克征是诊断BO的重要征象.约50％患儿ANCA阳性,提示BO患儿可能存在免疫损伤.口服全身激素及甲氨蝶呤可改善临床症状,但整体治疗效果不理想.     

肿瘤患儿疼痛管理现状的单中心调查

目的了解目前血液肿瘤患儿疼痛管理现状及存在的阻碍。方法以2018年1月8日—2018年1月26日期间血液肿瘤科实体瘤及淋巴瘤病房住院患儿、家长及医生为研究对象,对年龄大于4岁的住院患儿进行连续地疼痛评估,对23名医生及28名患儿家长进行问卷调查。结果有97名患儿接受了多次疼痛评估,其中20人发生中重度疼痛(20.6%)。共计评估367人次,67人次为中重度疼痛(占18.3%),其中70.2%的疼痛未向医生报告,予以镇痛治疗的仅占17.9%,其中阿片类药物占8.3%。针对家长和医师的问卷调查显示:有5/23的医生选择偶尔或从不对患儿评估疼痛,21/28的家长选择医生偶尔或从不评估患儿疼痛,差异具有显著性。当被问及开具镇痛药物依据时,有11/23的医生选择依据疼痛评分;当被问及最有助于缓解晚期癌症患儿中到重度疼痛方法时,有17/23的医生选择阿片类药物;而当被问及实际推荐首选药物时仅6/23的选择阿片类药物,差异具有显著性。结论肿瘤患儿的镇痛治疗尚未被充分重视,甚至中重度疼痛都仍被忽视。疼痛评估不足、家长及医生缺失疼痛相关知识是目前疼痛管理关键阻碍。完善疼痛评估是开展儿童癌痛管理的关键。     

儿童纵隔神经源性肿瘤单中心外科治疗经验CSCD

目的 分析儿童纵隔神经源性肿瘤行手术治疗患儿的临床特征、治疗方法及预后。方法 收集中国医科大学附属盛京医院小儿外科2012年1月至2021年12月间收治的30例纵隔神经源性肿瘤患儿临床资料,对其临床症状、影像学表现、治疗方法、病理类型及预后资料进行回顾性分析。结果 30例患儿平均年龄4.7岁(1~13岁),男20例,女10例。8例患儿未出现特异性症状(8/30,26.7%),7例为检查过程中意外发现,1例为肾上腺节细胞神经母细胞瘤术后随诊过程中发现;18例患儿因呼吸道症状就诊(18/30,60.0%),表现为咳嗽、咳痰、喘息等;2例胸背痛(2/30,6.7%);2例锁骨上淋巴结肿大(2/30,6.7%);12例伴发热(12/30,40.0%)。行胸腔镜手术16例(53.3%),胸腔镜辅助开胸手术9例(30.0%),开胸手术5例(16.7%)。术后病理检查结果:神经母细胞瘤4例(13.3%),神经节母细胞瘤8例(26.7%),节细胞神经瘤16例(53.3%),神经鞘瘤1例(3.3%),原始神经外胚层肿瘤2例(6.7%)。30例中良性16例(53.3%),恶性14例(46.7%)。结论 小儿纵隔肿瘤症状不典型,难以早期发现,胸部CT及MRI是诊断的主要手段,经手术治疗后预后相对较好。