儿童及青少年卵巢肿瘤187例

目的 探讨儿童及青少年卵巢肿瘤的发病特点、病理类型与发病年龄的关系,并评价其诊治手段及预后因素.方法 选取1980年1月-2005年12月在中山大学肿瘤防治中心治疗并确诊的儿童及青少年卵巢肿瘤患儿187例,并对其临床及随访资料进行回顾性分析.采用SPSS 13.0统计软件,定性变量比较采用χ2检验,生存率计算用寿命表法.采用Kaplan-Meier法进行生存分析.结果 187例卵巢肿瘤中良性56例,恶性119例,交界性12例.不同病理类型的卵巢肿瘤与患儿发病年龄有一定关系(χ2=12.396,P=0.015).187例卵巢肿瘤中,56例良性肿瘤5 a总生存率为98%,119例恶性肿瘤5 a总生存率为76%,12例交界性肿瘤5 a生存率为83%.单因素生存分析显示,影响总生存时间的危险因素为肿瘤分期、是否行规范化疗和化疗方案.结论 超声及CT是卵巢肿瘤诊断的主要方法.不同病理类型的儿童卵巢肿瘤与发病年龄有一定关系.肿瘤分期、术后辅助治疗方式均影响患儿总生存时间.     

儿童髓母细胞瘤的临床特点及预后相关因素分析

目的通过分析儿童髓母细胞瘤的临床特点、治疗方法和患者预后的关系,探索影响儿童髓母细胞瘤预后相关因素。方法收集2004年至2014年复旦大学附属儿科医院收治且术后病理证实为髓母细胞瘤患儿的病例资料,对所收集数据进行分组,采用单因素Kaplan-Meier法进行生存分析,统计各组病人的总体生存时间及累计生存率,采用Log-Rank检验分析各组病人累计生存率差异,采用Cox回归进行多因素分析,探讨儿童髓母细胞瘤临床因素与生存时间、生存结局的关联性。结果单因素分析结果认为,临床症状阳性数(P=0.039)、M分期(P=0.009)、术后放疗(P=0.001)、术后化疗(P=0.018)、肿瘤复发(P=0.04)是髓母细胞瘤患儿预后的影响因素;Cox多因素分析结果认为术后放疗(OR=0.291,95%置信区间:0.120~0.706,P=0.006)和化疗(OR=0.095,95%置信区间:0.024~0.384,P=0.001)是影响髓母细胞瘤患儿预后的保护因素;性别、年龄、肿瘤位置、T分期、肿瘤切除程度、病理分型等因素未发现与预后有关(P>0.05)。结论术后是否接受放疗、化疗对患儿预后影响较大,而临床症状的个数、M分期、肿瘤复发对患儿预后的判断价值有限,性别、年龄、肿瘤位置、T分期、肿瘤切除程度、病理分型等因素则与预后无关。     

肾母细胞瘤诊治10年回顾

目的 对10年中收治的小儿肾母细胞瘤的发病特点和诊治进行回顾分析,讨论影响肾母细胞瘤治疗和预后的因素.方法 统计10年期间69例肾母细胞瘤患儿的临床资料,包括患儿性别,发病年龄,临床表现和分期,病理分型和治疗手段,并将患儿分别以临床分期和病理分型进行分层,用Kaplan-Meier进行单因素生存分析.结果平均发病年龄(3.25±2.78)岁,发现腹部包块为主要起病症状,其中Ⅰ期18例,Ⅱ期25例,Ⅲ20例,Ⅳ期6例.治疗手段为患肾切除,NWTSG的化疗方案和有限病例的放疗.随访时间4～123个月,平均生存时间(41±30.9)个月.生存率:总体生存率78.2%,其中Ⅰ期患儿100%,Ⅱ期患儿76%,Ⅲ期患儿70%,Ⅳ期患儿50%.Kaplan-Meier生存分析表明,Ⅰ期、Ⅱ期、Ⅲ期三组患儿的生存时间差别无统计学意义,但明显高于Ⅳ期组患儿(LogRank法,P=0.04).预后良好组织类型(FH)组生存率为86.9%,其中Ⅰ期为100%,Ⅱ期93.3%,Ⅲ期72.7%,Ⅳ期50%;预后不良组织类型(UH)组总体生存率为65.2%,Kaplan-Meier生存分析生存表明,二组的生存时间具有显著性差异(Log Rank法,P=0.004).结论 临床分期,病理类型和治疗手段是影响肾母细胞瘤患儿预后的重要因素.     

核苷酸交叉互补基因1和B细胞特异单克隆鼠白血病毒整合位点基因1在青少年儿童脑胶质瘤中的表达与放疗疗效及预后的关系

目的 探讨儿童及青少年胶质瘤组织中核苷酸交叉互补基因1(ERCC1)、B细胞特异单克隆鼠白血病毒整合位点基因1(BMI-1)的表达与胶质瘤放疗敏感性及预后的关系.方法 收集2000年1月-2006年2月初诊接受手术、单纯放疗或同期放化疗、由中山大学肿瘤防治中心病理确诊为胶质瘤的儿童及青少年患者肿瘤石蜡标本85例.采用免疫组织化学二步法检测ERCC1、BMI-1蛋白的表达,统计分析二者与胶质瘤患者临床病理特征、放疗疗效、术后总生存率的关系.结果 ERCC1、BMI-1蛋白在85例胶质瘤组织中的阳性率分别为32.9%(28例)、37.6%(32例);BMI-1在高级别胶质瘤中的表达显著高于低级别,差异有统计学意义(P=0.027).BMI-1、ERCC1高表达患者放疗有效率明显低于低表达患者(P=0.020,0.024).Kaplan-Meier生存分析显示ERCC1、BMI-1蛋白高表达患者术后总生存时间短于低表达患者(P=0.028,0.006).多因素Cox回归分析显示病理级别、术后行为状态评分、放疗疗效、BMI-1的表达是青少年儿童脑胶质瘤患者独立的预后因素(Pa<0.05).结论 ERCC1、BMI-1蛋白表达与胶质瘤放疗疗效相关,BMI-1蛋白高表达与胶质瘤的恶性程度及预后相关,是青少年儿童胶质瘤患者的独立预后指标.     

184例儿童与成人横纹肌肉瘤的比较研究

目的 比较分析儿童与成人横纹肌肉瘤的预后差别及其临床影响因素.方法 回顾性分析天津医科大学肿瘤医院1993年1月至2009年6月间经病理确诊为横纹肌肉瘤且有完整随访资料的184例患者,其中分为儿童组93例(≤18周岁)和成人组91例(＞18周岁).结果 1年、3年、5年生存率儿童组分别为90.3％、62.0％、43.1％,成人组分别为86.8％、35.1％、20.0％.两组间1年生存率差异无统计学意义(P=0.454),但3年(P=0.001)和5年(P=0.007)生存率成人组均低于儿童组,并且Kaplan-Meier生存曲线也提示成人组(中位生存期26.7个月)低于儿童组(中位生存期47.3个月),Log-rank检验两生存曲线差异有统计学意义(P=0.003).多因素分析显示组织学类型、原发部位和术后分组是儿童RMS预后的独立影响因素,组织学类型、原发肿瘤大小和术后分组是成人RMS预后的独立影响因素.x2检验显示儿童组与成人组在组织学类型(胚胎型:77.4％、27.5％；腺泡或多形性:22.6％、72.5％；P=0.000)、原发部位(预后好的部位:40.9％、22.0％；预后不好部位:59.1％、78.0％;P=0.006)和远处转移(无转移:87.1％、73.6％；有转移:12.9％、26.4％；P=0.021)因素的差异具有统计学意义.结论 成人RMS的预后明显差于儿童,组织学类型、原发部位和远处转移的差异是导致这种差别的可能因素.     

儿童胚芽细胞肿瘤预后相关临床因素分析

目的了解儿童胚芽细胞肿瘤的预后与临床因素的相关性。方法调查新华医院1985-1996年和上海儿童医学中心2000-2006年二个阶段收治的208例儿童胚芽细胞肿瘤病例，分析其发病年龄、部位、病理类型及临床分期，并作临床随访。结果208例不同部位肿瘤中，骶尾部肿瘤获诊年龄最早，其他依次为：腹膜后、睾丸、纵隔、卵巢。恶性肿瘤的发生率随年龄递增。200例获得病理诊断的肿瘤中，畸胎瘤占绝大多数，为81．5％，其他依次为：卵黄囊瘤15．0％、胚胎癌3．0％、精原细胞瘤0．5％。在各部位肿瘤中，睾丸肿瘤的病理类型最多。208例中106例（51．0％）获得长期随访，其中83例长期存活，23例复发死亡。结论病理类型、肿瘤部位及临床分期是影响儿童胚芽细胞肿瘤预后的重要因素，在过去10年中新生儿及低龄儿胚芽细胞肿瘤的生存率有了明显提高。     

C5V作为首选化疗方案治疗肝母细胞瘤效果观察

目的 总结C5V方案(顺铂+5-氟尿嘧啶+长春新碱)治疗肝母细胞瘤的经验,并探讨影响治疗效果的相关因素.方法 回顾分析2006年1月至2014年4月在复旦大学附属儿科医院治疗的肝母细胞瘤病例资料,共63例采用C5V作为首选化疗方案,其中男37例,女26例,初诊年龄6d～ 124个月,中位年龄15个月.按照PRETEXT分期系统,其中Ⅰ期4例、Ⅱ期16例,Ⅲ期28例,Ⅳ期15例.其中有9例(Ⅰ期4例、Ⅱ期5例)行一期肿瘤切除术,剩余54例需术前辅助化疗.中位随访时间30个月.结果 40例患儿经辅助化疗后行延期肿瘤切除术,其中C5V方案单独化疗35例,C5V配合CITA方案(顺铂+阿霉素)化疗5例,术前平均化疗时间为(3.4±1.7)个疗程.49例行肿瘤切除术患儿术后平均化疗(5.3±2.0)个疗程.术后复发12例(24.5％),切缘＜0.5 cm、静脉瘤栓、Ⅲ或Ⅳ期肿瘤是术后复发的危险因素(P =0.049、0.001、0.022).本组病例2年和5年总体生存率分别为61.1％、58.7％,其中Ⅰ～Ⅲ期分别为75.0％和75.0％,100.0％和100.0％、65.8％和61.4％.Ⅳ期的1年和3年的总体生存率为20.0％和13.3％.单因素分析结果显示,初诊年龄＜60个月、静脉瘤栓、初诊时伴有血小板增多症、Ⅲ或Ⅳ期肿瘤、肿瘤完整切除是生存时间的影响因素(P=0.019、＜0.001、0.011、0.001、＜0.001).多因素分析提示肿瘤完整切除(P＜0.001),初诊年龄＜ 60个月(P =0.004)是重要的影响因素.结论 C5V是肝母细胞瘤有效的治疗方案,肿瘤完整切除仍是治疗的关键.生存时间的影响因素包括年龄、分期、伴随症状等.     

115例儿童髓母细胞瘤综合治疗及疗效分析

目的 分析研究儿童髓母细胞瘤综合治疗疗效;探讨危险因素与治疗效果及预后的关系.方法 对2010年1月至2015年6月期间经脑肿瘤手术后病理组织确诊的115例儿童髓母细胞瘤患儿进行分析,男77例,女38例,男∶女=2.02∶1;年龄:≤3岁8例(最小15个月),＞3岁107例(最大15岁),中位年龄6.75岁.依据危险因素:年龄、Chang et al's肿瘤转移分期(无肿瘤转移M0,有肿瘤转移M+)、手术肿瘤全切/近全切及肿瘤残留(肿瘤残留≥1.5 cm)、病理组织分型(经典型CMB,促纤维结节型DMB/广泛结节型MBEN,大细胞型LCMB和间变细胞型AMB)进行低危险因素/高危险因素诊断和分层的综合治疗.对低危险因素(年龄＞3岁、M0期、手术肿瘤全切/近全切、组织病理分型CMB,DMB/MBEN)的患儿,手术后给予24～30 GY全中枢及54～56 GY后颅凹病灶部位的放射治疗,放射治疗结束后进行8个周期的维持化疗.对高危险因素(年龄≤3岁、M+期、手术后有肿瘤残留≥1.5 cm,组织病理分型AMB,LOMB)患儿,年龄＞3岁,手术后先给予2个周期的诱导化疗,诱导化疗结束后进行36 GY全中枢及54～56 GY后颅凹病灶部位的放射治疗,放射治疗结束后再进行4个周期的维持化疗,年龄≤3岁,手术后给予3个周期诱导化疗后再给予4个周期维持化疗.结果 低危险因素60例(52.2％),高危险因素55例(47.8％).随访时间最短6个月,最长60个月,中位随访时间25个月.3～5年总体生存(OS),低危险因素85.5％,高危险因素67.7％:两组之间差异有统计学意义(P＜0.05);3-5年无事件生存(DFS),低危险因素76.8％,高危险因素47.3％,两组之间差异有统计学意义(P＜0.05);复发病例31例(27.0％)其中≤3岁2例,术后复发时间:最短4周,最长48个月,中位复发时间12个月,治疗后完全缓解2例,部分缓解13例,死亡16例,其中≤3岁1例,继发颅内高级别胶质瘤1例.结论 依据危险因素分层治疗儿童髓母细胞瘤有益于儿童髓母细胞瘤患儿治疗,提高生存率及生存质量;儿童髓母细胞瘤长期生存与危险因素有关系.     

骶尾部畸胎瘤术后复发高危因素分析

目的 探讨和分析引起骶尾部畸胎瘤术后复发的高危因素.方法 回顾性分析2003年1月至2012年12月收治的107例骶尾部畸胎瘤患儿的临床资料,使用单因素和多因素危险度Lo-gistic回归法,从Altman分类、手术年龄、肿瘤大小、肿瘤是否完整切除、术中肿瘤是否溃破以及破溃成分、病理类型等方面分析肿瘤复发的高危因素.结果 肿瘤复发共16例,复发时间为6～59个月,平均(16.25±14.01)个月.恶性肿瘤复发均为术后常规随访发现,良性肿瘤仅2例常规随访发现,其余因出现排便困难、骶尾部出现肿块等临床症状就诊.根据Altman分类,Ⅰ～Ⅳ型的复发病例(复发率)分别为8例(15.4％)、2例(10.5％)、1例(10.0％)和5例(31.3％).病理类型中,成熟性畸胎74例中复发9例,其中5例病理性质复发后转变为未成熟或恶性畸胎瘤;未成熟畸胎瘤6例中复发2例;恶性畸胎瘤17例中复发5例.肿瘤复发后的5年生存率(64.8％)与未复发病例(95.0％)相比,差异有统计学意义(P=0.002).单因素及多因素危险度分析提示术中肿瘤实质成分溃破(P=0.028)、肿瘤残留(P=0.000)、未成熟(P=0.029)及恶性畸胎瘤(P=0.026)是骶尾部畸胎瘤复发的危险因素,而Altman分类、手术年龄、肿瘤大小以及囊液破裂并不是肿瘤复发的危险因素.结论 肿瘤复发是影响骶尾部畸胎瘤预后的重要因素.术中避免肿瘤实质成分溃破,尽可能完整切除肿瘤可减少肿瘤复发.重视骶尾部畸胎瘤,特别是成熟性畸胎瘤的术后随访,有利于尽早发现肿瘤复发,提高治愈率.     

肾母细胞瘤复发和转移后的再手术治疗

目的 探讨肾母细胞瘤复发和转移后的再手术治疗.方法 回顾分析2000至2008年期治疗后的15例肾母细胞瘤复发和转移病例,记录其相关的病例资料及肿瘤复发的时间、复发部位、肿瘤复发后的手术治疗方案,并统计再治疗后的随访结果.结果 肿瘤复发和转移时间3个月～5年(中位时间7个月).病理类型FH 13例,UH 2例,NWTSG分期Ⅱ期4例,Ⅲ期5例,Ⅳ期3例,另有3例资料不完整.5例有术前或术中肿瘤破溃史(33%),有6例术后未按照规范化治疗(40%).9例复发肿瘤行再次手术,术式为单纯肿瘤切除,术后再行化疗和放疗,再次手术后1年总体生存率为67.7%,2年总体生存率为57.1%,2年无瘤生存率42.9%,生存时间较非手术病例明显延长(P<0.05).结论 肿瘤破溃与不规范治疗是肾母细胞瘤复发的重要因素.对复发肿瘤行积极的手术切除具有重要意义,结合综合治疗有助于提高复发肿瘤治愈率.     

小儿原发性纵隔肿瘤和囊肿的诊治

目的 探讨小儿原发性纵隔肿瘤和囊肿的诊断及治疗.方法 回顾性分析79例小儿原发性纵隔肿瘤和囊肿的临床资料.所有病例均经手术和病理证实.结论 本组病例中,小儿原发性纵隔肿瘤和囊肿占同期我科收治的原发性纵隔肿瘤及囊肿的13.96%(79/566).其中神经源性肿瘤21.5%(18/79)、肠源性囊肿15.2%(12/79)、畸胎瘤17.7%(14/79),其他肿瘤和囊肿44.30%(35/79).无临床症状而于查体时发现12例,占15.19%.手术切除74例,手术切除率93.7%(74/79),经治疗后康复出院76例,死亡3例.结论 小儿纵隔肿瘤和囊肿的术前诊断依据胸部X线、CT扫描及超声检查,必要时可以结合MRI扫描判断病变范围,而确诊则依赖于术中所见及术后病理检查.其病情复杂,变化快,可危及患儿生命,除淋巴瘤外宜选手术治疗.

Abstract：

Objective To investigate the diagnosis and treatment of primary mediastinal tumors and cysts in children.Methods The data of 79 patients admitted from 1966 to 2009 with primary mediastinal tumors and cysts were retrospectively reviewed.All patients underwent surgery and pathological examination.Results The primary mediastinal tumors and cysts in children accounted for 13.96% of primary mediastinal tumors and cysts at the same period.In these 79 patients,neurogenic tumors were diagnosed in 18 (21.5%)patients,gastrointestinal cyst was 15.2%(12/79),teratoma was 17.7%(14/79),and the others were 44.30%(35/79).Twenty-two patients without clinical symptoms were diagnosed by health checkup,accounting for 15.2% in all cases.Tumors in 74 patients(93.7%)were resected,and the mortality rate was 3.8%(3/79).Conclusions The preoperative diagnosis of mediastinal tumors and cysts in children primarily bases on chest X ray,CT scan,ultrasonography,and MRI scan in some cases,but the final diagnosis depends on the intraoperative findings and pathologic examination.Except of lymphoma,primary mediastinal tumors and cysts should be treated by surgery appropriately.     

Clinical, radiological, and pathological findings in four children with gastrointestinal stromal tumors of the stomach

The incidence of gastrointestinal stromal tumors (GISTs) in children is exceptionally low. However, during the last decade these tumors attracted increasing attention, because they were found to express the cell surface transmembrane receptor kit (CD117) that has tyrosine kinase activity. This tyrosine kinase can be semi-selectively inhibited by signal transduction inhibitors such as imatinib mesylate (Glivec), which is a competitive inhibitor of c-kit, c-abl, platelet-derived growth factor receptor-alpha (PDGFR-alpha) and PDGFR-beta, and abl-related gene (arg). The authors present the clinical, radiographic, and pathological findings of 4 children who were diagnosed with gastric GIST. One of them had an incomplete Carney triad including GIST and mediastinal paraganglioma. All 4 patients presented with anemia and anemia-related symptoms and underwent total resection of the tumor. One patient received additional chemotherapy (in the pre-imatinib era) and 2 patients received a short course of imatinib mesylate. With a follow-up of 116, 55, 23, and 10 months all patients are alive in first complete continuous remission. In children and adolescents, particularly in female patients, GISTs should be included in the differential diagnosis of anemia secondary to gastrointestinal hemorrhage. Complete surgical resection is the mainstay of treatment for this tumor, with imatinib mesylate restricted to patients with advanced or metastatic tumors. Since late recurrences (up to 30 years following initial diagnosis) are reported, a life-long follow-up is mandatory in these patients.     

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。     

Mediastinal masses in children: experience with 120 cases

Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.     

Childhood mediastinal masses in infants and children

We reviewed all cases of primary pediatric mediastinal masses diagnosed and treated over a 24-year period. In this study, out of 187 primary mediastinal mass cases diagnosed between 1980 and 2004 in Istanbul University Istanbul Faculty of Medicine, Cardiovascular Surgery Department, 37 pediatric primary mediastinal mass cases were retrospectively evaluated according to age, sex, symptoms, diagnostic procedure, anatomical location, surgical treatment, histopathological evaluation and postoperative adjuvant therapy. The patients ranged in age from 2 months to 15 years at the time of diagnosis, with a mean age of 8 years. There were 24 benign (64.8%) and 13 malignant (35.2%) tumors. The cases were lymphoma (27%), neurogenic tumors (21.6%), cystic lesions (18.9%), germ cell tumors (13.5%), thymic lesions (10.8%) and cardiac tumors (8.1%). Complete and partial resections of the tumor were the surgical procedures performed in 24 patients (64.8%) and 3 patients (8.1%), respectively. The three patients with a malignant tumor, in whom the entire mass could not be removed, received chemotherapy and radiation after surgery. In 10 patients with lymphoma, surgery was not a part of treatment and they received medical and radiation therapy after the establishment of the definitive diagnosis. All patients survived and were discharged from the hospital. Except for the cases with lyphoma, all patients are now free of recurrent disease. Compared to adults, children had more lymphomas and neurogenic tumors. Primary pediatric mediastinal malignancies are relatively common in infants and children. Lymphoma, neurogenic tumors and cystic lesions predominated. These differences between the age groups should also be considered when dealing with a mediastinal mass.     

Clinical stage I nonseminomatous germ cell tumors of the testis in childhood and adolescence: an analysis of 31 cases

A 20-year single-institution experience of clinical stage I nonseminomatous germ cell tumors of the testis (NSGCTT) in childhood and adolescents was reviewed in relation to clinical characteristics, treatment modalities, and survival. Thirty-one patients with clinical stage I NSGCTT were seen between 1980 and 2000: 14 children and 17 adolescents. Yolk sac tumors and/or teratomas occurred in the children, whereas mixed histologies, including embryonal carcinoma, were predominant in the adolescents. After orchiectomy, the children were assigned to surveillance and the adolescents to active treatment: 16 underwent retroperitoneal lymph node dissection (RPLND) and 1 had adjuvant cisplatin-based chemotherapy because of a high-risk histology. Three of the 14 children (21.4%) relapsed 3, 7, and 8 months after orchiectomy: all 3 had yolk sac tumors and presented with increased alpha-fetoprotein levels. No patients had retroperitoneal relapse; two recurred locally and one in the lung. All three children were treated with cisplatin-based chemotherapy with or without surgery. Among the 16 adolescents undergoing RPLND, 4 (25%) had nodal metastases. Three of the 12 patients (25%) who had negative nodes at RPLND relapsed in the lung 3, 7, and 8 months after RPLND. All were treated with cisplatin-based chemotherapy with or without surgery. Five-year relapse-free and overall survival rates for the whole series were 80.6% and 100%, respectively. This series enabled the authors to pinpoint several important aspects of stage I NSGCTT in children and adolescents. In particular, almost all the childhood cases had the same yolk sac tumor histology, the children tended to have localized disease, and an increased alpha-fetoprotein level had a very high predictive value, suggesting that follow-up should include AFP measurements. A conservative approach is the best option in children, while adolescent NSGCTT behaves like the adult disease and management must include similar treatment strategies.     

Long-term outcomes of pediatric and adolescent mediastinal germ cell tumors: a single pediatric oncology institutional experience

Purpose

To evaluate the epidemiologic, demographic, and clinical characteristics, as well as prognostic factors and long-term outcomes of mediastinal germ cell tumors (MGCT) in children.

Patients and methods

A retrospective study of pediatric patients diagnosed with a primary MGCT between January 1963 and August of 2014 was performed.

Results

Twenty-five patients were identified. Six children with teratomas were treated with resection alone (median age 7.8 years, range newborn to 15 years) and were cured without recurrence or progression. Nineteen children were treated for a malignant MGCT (median age 11.7 years, range 7 months–18 years); 5 year overall survival (OS) was 0.39 ± 0.12. For malignant non-seminomatous mediastinal germ cell tumors, platinum-based chemotherapy regimen (OS 0.56 vs 0.14, p = 0.03), complete surgical resection with negative margins (OS 0.73 vs 0.11, p = 0.03); and localized disease (OS 0.76 vs 0.0, p = 0.004) demonstrated a survival advantage.

Conclusions

Initial surgical resection is appropriate for teratomas. Localized disease, complete resection, and platinum-based chemotherapy are associated with improved survival in malignant non-seminomatous mediastinal germ cell tumors. Neoadjuvant, platinum-based three drug regimens followed by delayed surgical resection is the appropriate treatment modality for malignant mediastinal germ cell tumors.

     

1000例肾活检儿童临床与病理分析

目的探讨肾活检儿童的临床、病理特点及两者之间的关系。方法回顾性分析1 000例肾脏病患儿临床与肾脏病理资料。结果 1 000例患儿肾活检取材成功962例,成功率96.2%。患儿术后出现轻度并发症170例,并发症发生率为17%。临床表现与病理大部分符合,但有20例临床与病理诊断不符。结论儿童肾脏病的临床表现轻重与病理改变轻重并不平行,仅凭临床表现进行诊治,有误诊、过度治疗、延误治疗的可能,病理诊断对临床治疗及预后评价具有重要的指导意义。     

小儿新月体肾炎临床研究

目的了解小儿新月体肾炎的临床特点及诊断和治疗。方法对我科近3年来经肾脏病理确诊的16例新月体肾炎患儿发病年龄、病因、临床特点,实验室检查特点、肾脏病理改变、治疗和预后进行分析。结果小儿新月体肾炎以>10岁的儿童多见,最常见的原因为IgA肾病,其次为紫癜性肾炎和狼疮性肾炎,以急进性肾炎表现的较少。本组患儿均有肉眼血尿,大量蛋白尿、血清肌酐清除率(Ccr)逐渐下降、血尿素氮(BUN)逐渐上升的发生率分别为87.5%、75%、62.5%,病理有广泛的细胞性及细胞纤维性新月体形成,治疗主要是激素加免疫抑制剂,联合抗凝,抗血小板治疗及对症治疗。结论(1)小儿新月体肾炎以IgA肾病为最常见的病因,对肉眼血尿并大量蛋白尿持续存在的患儿建议早期肾病理检查,早期诊断,及时治疗,坚持随访尤为重要;(2)治疗采用激素加用免疫抑制剂,先强化后维持的治疗方案。