儿童后肾腺瘤五例报告

目的 探讨儿童后肾腺瘤临床病例特点,提高对儿童后肾腺瘤的认识.方法 回顾性分析2008年5月至2016年1月收治的5例儿童后肾腺瘤的临床资料,并进行文献复习.5例患儿中,男4例,女1例;年龄小于2岁2例,8～10岁3例;肿瘤位于左侧2例,右侧3例.以腹部包块就诊1例,超声偶然发现4例.术前均行超声和增强CT,2例考虑肾母细胞瘤,2例考虑肾细胞癌,1例考虑肾囊肿合并出血.结果 肿瘤直径小于5 cm者4例.行保留肾单位的肿瘤剜除术3例,行瘤肾切除术2例.病理检查报告:后肾腺瘤4例,后肾腺瘤合并乳头状肾细胞癌1例.术后平均随访4年5个月,均无复发和转移.结论 后肾腺瘤罕见,临床表现及影像缺乏特异性,术前诊断困难,确诊依靠病理及免疫组织化学.后肾腺瘤为良性病变,手术完整切除为治疗原则.有合并恶性成分的病例报道,术后需长期随诊复查.     

儿童Xp11.2易位/TFE3基因融合相关性肾癌单中心诊疗分析CSCD

目的探讨儿童Xp11.2易位/TFE3基因融合相关性肾癌的诊疗经验。方法回顾性分析2017年1月至2021年6月上海交通大学医学院附属新华医院儿普外科收治的11例肾Xp11.2易位/TFE3基因融合相关性肾癌患儿临床资料。其中男3例, 女8例;肿瘤位于右侧6例, 左侧5例。患儿诊断时年龄1~12岁, 中位年龄6岁。首诊原因包括血尿6例, 腹痛2例, 尿频1例, 肿块1例, 疑似性早熟1例。所有患儿未经化疗直接行手术切除。手术方式均为根治性肾切除术。结果患儿均顺利完成手术, 手术时间100~135 min。术中出血量5~30 mL。3例出现局部淋巴结转移, 1例肿瘤侵犯肾周脂肪。肿瘤标本病理检测符合Xp11.2易位/TFE3基因易位相关肾细胞癌, 含Xp11.2易位/TFE3基因融合。肾癌Robson分期Ⅰ期7例, Ⅱ期1例, Ⅲb期3例。患儿术后均未接受化疗及放疗, 术后随访6~38个月, 随访中1例Ⅲb期患儿出现肿瘤复发转移, 给予舒尼替尼治疗3个月后死亡;其余患儿均无事件生存。结论 Xp11.2易位/TFE3基因融合相关性肾癌是儿童、青少年易发的肾癌类型。发病年龄与肾母细胞瘤发病年龄有重叠。早期发现、早期行根治性手术可获得良好预后。临床应重视淋巴结切除在儿童肾细胞癌治疗中的作用。未来需要寻找有效的辅助治疗手段以改善复发肿瘤的预后。     

儿童非肾母细胞瘤肾脏肿瘤的临床治疗探讨

目的总结和探讨儿童非。肾母细胞瘤肾脏肿瘤的治疗方法。方法1998年7月至2010年7月我们共收治非肾母细胞瘤肾脏肿瘤患儿18例,其中男性14例,女性4例,年龄最大9岁,最小2个月,平均年龄（40．17±34．04）个月,术后病理诊断包括肾透明细胞肉瘤9例（9／18,50％）,肾横纹肌样瘤2例（2／18,11．1％）,肾癌2例（2／18,10．1％）,肾中胚层。肾瘤1例（1／18,5．56％）,后。肾间质瘤1例（1／18,5．56％）,后肾腺瘤1例（1／18,5．56％）,肾血管平滑肌脂肪瘤1例（1／18,5．56％）,肾原始神经外胚叶瘤1例（1／18,5．56％）。结果随访9例肾透明细胞肉瘤,8（8／9）例无瘤生存,最长随访时间5年,最短2个月,平均随访时间（24．56±17．27）个月,1例手术化疗后下腔静脉瘤栓残留水平至右心房入口,放弃治疗1．5年后死亡。2例肾横纹肌样瘤经手术、放疗及化疗,随访3年和4年均无瘤存活。2例。肾癌单纯手术切除,密切随访25个月、13个月无复发及转移。1例。肾原始神经外胚叶瘤经手术及化疗,随访16个月无瘤存活。1例肾中胚层肾瘤、1例后。肾间质瘤、1例后。肾腺瘤、1例肾血管平滑肌脂肪瘤均完整切除,现最长随访时间4年,最短1年,无一例复发。结论儿童非肾母细胞瘤肾脏肿瘤所占比例虽小,但临床鉴别诊断困难,病理诊断非常重要,手术、化疗及放疗综合治疗是诊治的首要选择。     

儿童原发性恶性非肾母细胞瘤性肾脏肿瘤诊治特点

目的 探讨儿童原发性恶性非肾母细胞瘤性肾脏肿瘤的临床诊治特点.方法 回顾性分析1993年4月至2008年1月问收治的11例儿童原发性恶性非肾母细胞瘤性肾脏肿瘤患儿的临床资料.根据临床表现及术前影像学检查并于术前行穿刺活检,治疗方法主要为术前介入和/或全身化疗、手术切除、术中热灌注化疗和术后化疗.结果 肾细胞癌6例,无瘤长期(平均32个月)生存率66.7%.中胚性肾瘤3例,均获3年以上无瘤生存.肾透明细胞肉瘤1例,术后复发死亡.肾横纹肌样瘤1例,术后化疗2个月复发,结论儿童原发性恶性非肾母细胞瘤性肾脏肿瘤发病率低,临床表现与肾母细胞瘤相似,术前诊断较为困难,其中肾细胞癌发病年龄多为年长儿,而肾透明细胞肉瘤、中胚层肾瘤和肾横纹肌样瘤则多见于小婴儿.经过多项系统性治疗,中胚层肾瘤预后较佳,肾细胞癌次之.     

儿童肾盏憩室诊治分析

目的探讨儿童肾盏憩室(calyceal diverticulum, CD)的临床特点、诊断及手术治疗方式。方法回顾性分析2010年1月至2021年10月首都医科大学附属北京儿童医院泌尿外科与包头市第四医院小儿外科收治的7例肾盏憩室患儿临床资料, 其中男5例, 女2例;就诊年龄8个月至11岁, 平均年龄3岁11个月。憩室位于左侧3例, 右侧4例;肾上盏4例, 肾中盏3例。憩室长径3～9 cm, 平均6.2 cm。产前超声发现肾脏囊性变3例, 腹痛2例, 无症状腹部包块1例, 体检发现肾脏囊性病变合并高血压1例。3例术前经泌尿系超声检查确诊, 4例术中确诊。7例均行手术治疗, 其中开放肾盏憩室切除术2例, 肾盏憩室剪裁+盏颈扩大术3例, 开放和腹腔镜憩室壁翻瓣卷管输尿管端侧吻合术各1例。结果 7例均获随访, 随访时间3个月至10年, 患儿术后CD相关症状均消失, 术前扩张的肾盏憩室均明显缩小, 无一例复发;1例合并肾积水患儿术后积水明显减轻, 但肾功能恢复较差, 肾核素扫描显示分肾功能为20%, 目前仍在随访中。结论肾盏憩室易发生反复泌尿系感染、慢性腹痛、肉眼血尿、结石等, 易误诊为单纯性...     

微创经皮肾镜取石术治疗婴幼儿肾结石

目的 探讨微创经皮肾镜取石术(MPCNL)治疗婴幼儿肾结石的临床效果和安全性.方法 2008年12月至2009年10月行MPCNL治疗婴幼儿肾结石24例.男18例,女6例.年龄10～42个月,平均23个月.左侧10例,右侧8例,双侧6例,共30侧.结石最长径11～22 mm,平均14.2mm.通过B超、螺旋CT薄层平扫予以诊断.结果 24例患儿,共30侧肾行MPCNL治疗.手术时间25～90min,平均40min.术中出血5～40ml,平均15ml.建立经皮通道成功率为1(00%.术后全部留置肾造瘘管,3～5d后拔除.60%(18/30)的病例留置双J管,1个月后膀胱镜下拔除.所有病例未输血,未发生严重并发症.静脉应用抗生素3～7 d,术后住院4～10 d,出院时尿检阴性.患儿随访6～18个月,生长发育良好,经B超和尿检,22例肾结石消失,并无复发,肾盂积水消失或减轻,尿检阴性.2例患儿有肾结石残留.结石清除率91.7%(22/24).结论 采用MPCNL术治疗婴幼儿肾结石安全有效,术后恢复快、住院时间短,具有微创的优势.     

孤立肾并肾盂输尿管连接部梗阻的手术疗效分析

目的了解孤立肾合并肾盂输尿管连接部梗阻(UPJO)的手术治疗时机对于肾功能的影响。方法回顾性分析2006年1月至2018年1月首都医科大学附属北京儿童医院和北京儿童医院顺义妇儿医院收治的孤立肾合并UPJO 23例患儿临床资料。其中男16例,女7例。首诊年龄为1 d~15岁8个月,平均2岁2个月。全组患儿平均保守治疗时间2年9个月。应用SPSS 20.0软件进行统计学分析。结果23例患儿均接受离断性肾盂成形术(A-H术),手术时年龄10个月~16岁4个月,平均4岁11个月。<1岁者2例,分别为10个月、11个月。术后保留双J管2个月,术后3个月时复查静脉肾盂造影(IVP),显示肾盂积水较前无明显变化或不同程度减轻;3例术前IVP 40 min仍不显影者,术后显影明显改善,在10~20 min时显影。随访1年1个月~10年,平均随访3年9个月,泌尿系统超声提示肾盂扩张程度较术前有不同程度减轻。结论孤立肾并UPJO在严密观察下的密切随访及保守治疗至6个月以后是安全的,首选手术方式为肾盂离断成形术。     

儿童双侧肾母细胞瘤手术治疗的探讨

目的 总结探讨儿童双侧肾母细胞瘤综合治疗的疗效及随访结果。方法 回顾性分析我院白1998年8月至2010年8月手术治疗的双侧肾母细胞瘤患儿临床资料。结果 7例双侧肾母细胞瘤中,男性患儿3例,女性患儿4例,发病年龄最大21个月,最小6个月,平均年龄( 12.71±4.89)个月。临床表现腹部肿块7例(7/7),血尿1例(1/6),贫血2例(2/6),4(4/7)例活检明确病理经术前化疗后手术,3例先手术后化疗,1例化疗2个疗程疾病进展,家属放弃治疗后死亡,5例结束整个疗程随访中,4例带瘤生存,1例随访7个月后残余肿瘤病灶消失,随访时间最长10年,最短10个月,1例术后化疗中,2年存活率85.7％。结论 儿童双侧肾母细胞瘤通过手术、化疗及放疗综合治疗,可以带瘤生存。     

经脐单孔腹腔镜实施小儿重复肾半肾切除术

目的 探讨经脐单孔腹腔镜实施小儿重复肾半肾切除术的可行性和疗效。方法回顾性分析2010年12月至2012年6月作者收治的25例重复肾双输尿管畸形患儿的临床资料。25例重复肾均位于肾上极,其中左侧9例,右侧14例,双侧2例。男性7例,女性18例,年龄2～60个月,平均年龄31个月。术前经彩超、磁共振、静脉肾盂造影等影像学检查确诊。手术均取脐部作为操作通道,用切开法进入腹腔,将单孔多通道套管（Tri port）经脐置入腹腔,行重复肾输尿管切除。结果25例手术均获成功,无中转开放病例。单侧手术时间（64±20）min,双侧手术时间（100±30）min,出血量（10±38）mL,术后住院时间（6.1±1.1）d,术中术后均未发生严重并发症。随访1 ～ 3个月,所有患儿术后均经彩超和肾核素扫描随访,未发现下肾功能丢失或萎缩。原发病症状均获改善。结论经脐单孔腹腔镜实施小儿重复肾半肾切除术创伤小、并发症少、恢复快,标本易于取出,值得临床推广应用。     

儿童马蹄肾合并肾母细胞瘤的诊治分析

目的探讨儿童马蹄肾合并肾母细胞瘤合理的诊治方法。方法回顾性分析2007～2018年我院收治7例马蹄肾合并肾母细胞瘤患儿的临床资料。其中男3例,女4例,就诊年龄1岁至8岁8个月,平均4岁6个月。肿瘤位于左侧4例,右侧2例,峡部1例。主要临床表现:腹痛4例,无症状腹部包块1例,偶然超声检查发现肿物1例,外院手术并放化疗后肿瘤复发1例。4例(其中2例因瘤体破裂,1例因瘤体巨大且合并肺部转移,1例因瘤体巨大)术前化疗后行患侧肾脏切除及峡部肿瘤切除术,术后辅以放疗+化疗;2例肿瘤位于肾脏一极行肿瘤剜除术,术后辅以化疗;外院术后及放化疗后肿瘤复发1例于我院行复发肿瘤切除术+盆腔转移灶切除。结果 5例获随访(含2例肾肿瘤剜除术)6个月至11年,平均44个月,均未发现肿瘤复发,行肿瘤剜除者肾脏功能良好。2例失访,包括外院术后及放化疗后肿瘤复发1例,术前肺转移1例。结论马蹄肾合并肾母细胞瘤较少见,根据肿瘤位置及大小选择个体化治疗方式,如肿瘤位于肾脏一极可行保留肾脏的肿瘤剜除术,术后按病理分型分期辅以化疗和/或放疗。     

儿童肾癌的临床及CT和病理特点分析

目的 探讨儿童肾癌的临床、CT及病理特点,积累其临床诊治经验.方法 回顾性分析2014年至2016年单中心收治的7例儿童肾癌患儿的临床、CT、病理资料,并随访其生存情况.就诊时年龄5～12岁,女4例,男3例,左侧5例,右侧2例.临床表现多为血尿(4/7)、腹部包块(3/7)、腰痛(1/7).CT平扫肿瘤呈低密度,多位于上下极,可见钙化(6/7)及坏死区,增强特点多为“快进快出”(4/7),部分为“快进慢出”(2/7).结果 7例患儿全部获得随访,随访时间8～35个月.1例肿瘤位于左腹膜后,确诊时已发生腹膜后及颈部淋巴结转移,行肿瘤部分切除活检,术后5个月死亡.1例为复发性肾癌,行肾窝肿瘤切除加腹膜后淋巴结清扫,术后5个月出现淋巴结转移,予放化疗后带瘤生存.余5例患儿均行根治性肾切除加淋巴结清扫,术后均无瘤生存.病理多为肾透明细胞(5/7)及乳头状肾细胞癌(2/7),癌细胞呈巢团状、乳头状、腺管样结构分布,可见砂粒体(6/7),核级多为3～4级.4例患儿淋巴结转移,共清扫出60枚淋巴结,淋巴结阳性率为16.7％.5例加做免疫组化,TFE3 (4/5)、CD10 (5/5)、Kb67 (4/5)、E-cardherin (4/5)、Vinentin (3/5)、CK(3/5)阳性率较高,4例确诊为MiT家族异位性肾癌.结论 儿童肾癌多发生于年长患儿,有其特有的临床、CT及病理特点,MiT家族异位性肾癌常见,病理及免疫组化是确诊和分型的主要手段.     

单纯上或下患肾部输尿管膀胱外再植治疗完全性重复肾畸形

目的 评价采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形的疗效.方法 从2009年12月1日起至2014年12月31日间,我科采用单纯上或下患肾部单根输尿管膀胱外再植治疗完全性重复肾畸形患儿27例,均为单侧病变,女19例,男8例,年龄2个月至8岁.所有患儿经影像学检查明确诊断为完全性重复肾畸形且均为单根输尿管病变.其中上输尿管异位开口者9例;重复上肾上输尿扩张积水伴上输尿管末端囊肿者10例;重复下肾部输尿管单纯反流者8例.临床症状主要表现为正常排尿间歇性滴尿或发热性尿路感染.所有患儿术后随访观察11个月至6年,随访项目包括泌尿系统超声、同位素和膀胱造影等.结果 所有患儿手术过程顺利,平均手术时间63 min,术后平均住院时间为5d,术后所有患儿临床症状均消失.术后有1例患儿因血尿检查发现再植输尿管开口处结石形成,予以行膀胱镜下钬激光碎石术.术后6个月复查超声示患肾部积水均有好转,同位素示患肾部功能改善或稳定,正常肾部功能未见异常改变.7例患儿术后复查排泄性膀胱尿道造影检查未见膀胱输尿管反流等发生.结论 经腹股沟皮纹小切口单纯上或下患肾部单根输尿管膀胱外再植术治疗完全性重复肾畸形是一种安全、有效的方法,值得临床推广.     

Low incidence of adverse events in outpatient pediatric renal allograft biopsies

In 1999, our center implemented a policy of outpatient protocol biopsies as standard practice for the clinical management of pediatric renal allograft recipients. In order to determine the safety of this procedure, we conducted a retrospective chart audit of all outpatient renal allograft biopsies performed at our center. Biopsies were performed under conscious (midazolam) or procedural (propofol/fentanyl) sedation. Localization of the lower pole of the renal allograft was achieved with renal ultrasound. Using a Biopty gun with a 16-gauge needle, two cores were obtained. Patients were discharged four h post-biopsy. Patient demographics, hospital length of stay (LOS), specimen adequacy (per Banff criteria) and major and minor adverse events were recorded in a central database. Data were expressed as mean +/- SD. From June 1999 to July 2004, we performed 162 biopsies in 43 pediatric renal allograft recipients. Most patients underwent extraperitoneal transplantation (42/43, 97.7%) and were greater than five yr of age at biopsy (129/131 biopsies, 98.5%). The majority of these procedures (131/162, 80.9%) were conducted in the outpatient department, with 113 of 131 (86.3%) being obtained for protocol (n = 89) and one-month follow-up acute rejection therapy (n = 24) indications. Patients underwent 3.7 +/- 2.7 biopsies (range = 1-11). Specimen adequacy was achieved in 119 of 124 (96.0%) of documented cases. The overall incidence of adverse events was 12 of 131 (9.2%) biopsies, all of which were minor in severity. Macroscopic hematuria was the most common minor adverse event, occurring after 11 of 131 (8.4%) biopsies. While macroscopic hematuria prolonged LOS (adverse events vs. no adverse events: 23.0 +/- 26.0 vs. 8.6 +/- 4.1 h, p = 0), none of these episodes required major surgical or radiographic interventions. We conclude that in patients greater than five yr of age with extraperitoneal renal allografts, outpatient protocol biopsies using a 16-gauge needle are sufficiently safe to justify their inclusion in the routine clinical management of pediatric renal allograft recipients and in pediatric clinical trials.     

Renal cell carcinoma in children under 10 years old: a presentation of four cases

Renal cell carcinoma (RCC) in childhood is a unique disease entity, representing only 2.3-6.6% of all renal tumors in children. Most experience with pediatric RCC is limited to case reports or case series consisting of relatively small numbers of cases. The aim of this study is to present four additional cases of this unique disease entity. The records of four patients presenting to our institution with renal cell carcinoma between 1986 and 2006 were examined. Only patients younger than 10 years were included. The clinical data included age, sex, signs and symptoms, surgery performed and clinical outcome. Data recorded from the pathology reports included tumor size, histologic subtype, grade (WHO), and stage (TNM). There were three boys and one girl aged between 11 months and 10 years at presentation. The classic triad of flank pain, gross hematuria and palpable abdominal mass were not encountered. A histopathological diagnosis of RCC was confirmed in all four cases without any positive lymph node involvement. Due to the increased detection of tumors with the use of imaging techniques such as ultrasound and computerized tomography, an increased number of incidentally diagnosed RCCs are found. The primary choice of the treatment of any stage of RCC is surgical excision. However, the preoperative diagnosis of tumor in children is difficult and the effects of chemotherapy, including immunotherapy, are unclear.     

Wilms’ Tumor: A 24-year Retrospective Study from a Single Center

Medical records of 71 children with Wilms’ tumor at Sisli Etfal Education and Research Hospital between 1990 and 2014 were reviewed. Mean age at diagnosis was 3.11 years (2 days–7 years). Male to female ratio was M/F = 6/10. The incidence of associated anomaly was 16.9%. Clinical manifestations included abdominal mass (89%), hematuria (30%), hypertansion (25%), abdominal pain (15%), fever (5%), restlessness (2%), weight loss (2%), varicocele (1%). Ultrasound (USG) was the most often initial study in a child presenting with abdominal mass. Doppler USG was also made to evaluate the inferior vena cava (IVC) for the presence of tumor extension in children with renal mass. The left kidney was affected in 33 patients (46.5%), the right was affected in 31 patients (43.7%). Two patients was extrarenal (2.8%). And 5 patients (7.04%) were bilateral on the presentation. Preoperative chemotheraphy was done in 14 cases. In 63 patients with unilateral Wilm tm, unilateral radical nefrectomy is performed. In one patient with solitary kidney, nephron sparing surgery (NSS) is performed. In 3 patients with bilateral tm NSS is performed and in 2 patients with bilateral Wilms’ tm NSS is performed in one side and nefrectomy on the other side. Out of 71 Wilms tumor (WT) patients, 17 of them has been out of our follow. And 4 of them are died. Ten of them has metastases. Forty children are under follow with no metastases. Patients with WT needs a multimodal, multidisiplinary treatment with the cooperation of pediatric oncologist and pediatric surgeon and needs close follow-up.     

Renal cell carcinoma in children

Data from four pediatric hospitals concerning 20 children treated for renal cell carcinoma (RCC) from 1964-1978 were reviewed. Median age of the patients (pts) was 11.8 years (range, 14 months-19 years). Twelve were male and eight female; 17 were white and three black. Most patients presented with pain and hematuria with or without a palpable mass. An intrarenal tumor was detected at IV urography (17 pts), arteriography (2 pts), or at surgery (1 pt). Treatment consisted of nephrectomy in 15 pts, renal biopsy (4 pts), or no surgery (1 pt), followed by chemotherapy (5 pts), radiation therapy (1 pt), or both (7 pts). Ten pts died of distant metastases at a median of one year (range, 0.2 to two years) after diagnosis. The other 10 pts (50%) survive free of relapse at a median of 4 years (range, two to ten years) from diagnosis. Proportions surviving free of recurrent disease two or more years by National Wilms' Tumor Study (NWTS) Group were 5/5 in Group 1, 3/7 in Group 11, 1/3 in Group III, and 1/5 in Group IV; by age at diagnosis, 6/6 in those under 11 years old and 4/14 in those 11 or older; and by type of surgery, 10/15 who had nephrectomy and 0/5 with limited or no surgery. The data indicate that radiation and chemotherapy had only minor if any influences on relapse-free survival. We conclude that (1) RCC in children is similar to its counterpart in adults; (2) RCC has a worse prognosis than Wilms' tumor except for the earliest stage; (3) nephrectomy alone is adequate treatment for Group I RCC, and (4) young age (< 11 years old) may be prognostically favorable.     

Expression of cytokeratin-18-related tissue polypeptide-specific (TPS) antigen in Wilms tumor

BACKGROUND: So far, there is no approved tumour marker for diagnosis or follow-up in Wilms tumour (WT). Tissue polypeptide-specific antigen (TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be of value in the clinical management of WT patients. Cytokeratin 18 fragments are an early indicator of apoptosis and cytokeratin 18 might influence tumour cell behaviour. We investigated TPS expression in specimens of WT and other paediatric renal malignancies PROCEDURE: Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n = 3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were investigated using the monoclonal antibody M3. Additionally, immunoblotting and RT-PCR analysis were performed. Cell culture supernatants were evaluated for TPS release. Serum TPS was measured in five patients at diagnosis, during chemotherapy and after surgical resection. RESULTS: Moderate to strong immunoreactivity for TPS was found in tubular and blastemal components of nearly all (8/9) WT specimens. This was confirmed by Western-blotting. Cystic and epithelial-like portions of CCSKs and RCC showed distinct reactivity (3/3). The supernatant of G-401 but not of SK-NEP-1 showed a time- and cell number-dependent increase of TPS release. Interestingly, TPS synthesis was demonstrated in SK-NEP-1 cells. Median preoperative serum TPS was elevated (293 U/l) compared to healthy children and lowest after surgical resection (49.5 U/l). CONCLUSIONS: This is the first study demonstrating the synthesis and release of TPS by WTs and other paediatric renal malignancies. Considering the elevated levels of TPS in serum of these patients, a further investigation of this marker by larger clinical trials seems to be justified.     

Renal cell carcinoma in long-term survivors of advanced stage neuroblastoma in early childhood

BACKGROUND: Renal cell carcinoma (RCC) is rare in children and comprises only 1-3% of all pediatric primary renal tumors. Recently, several case reports have described RCC developing in patients previously treated for advanced stage neuroblastoma (NB). METHODS AND RESULTS: Our experience with four patients treated for advanced stage NB during early childhood who developed RCC later in life are added to 14 others in the literature. CONCLUSION: These patients and our review of the literature suggest an association between RCC and NB that warrants further study.     

Clinical spectrum of gross hematuria in pediatric patients

Although isolated gross hematuria is a disturbing symptom, there have been few studies of this finding in pediatric patients. Therefore, this study was performed to examine the associated symptoms and causes of gross hematuria in children and adolescents who presented with this problem as their major clinical manifestation. It also determined the long-term outcome of patients in whom no etiology was found. A retrospective review was performed on the medical records of 100 consecutive patients referred for evaluation of gross hematuria between 1992 and 1999. The etiology was determined based on standard urinalysis methods, clinical laboratory tests, and imaging studies. Patients with gross hematuria in whom an etiology was not found were followed up through 2001. Of the 100 patient records reviewed, 18 were excluded because the clinical evaluation was incomplete. The remaining 82 patients (59 M: 23 F) had a mean age of 9.2 +/- 5.0 years. Glomerular gross hematuria was found in 24 patients. A cause was found in all of these patients, most commonly immunoglobulin A (IgA) nephropathy (n=13) and Alport syndrome (n=6). Nonglomerular gross hematuria was found in 56 patients, and the most common etiologies were hypercalciuria (n=9), urethrorrhagia (n=8), and hemorrhagic cystitis (n=7). No etiology was found in 26 patients with nonglomerular gross hematuria. No diagnosis was made in the case of 2 patients whose hematuria could not be defined as glomerular or nonglomerular. Telephone follow-up was performed in 18 of these children 4.0 +/- 3.2 years (range: 1-9 years) after the initial evaluation and showed that only 3 of these patients had had recurrences of gross hematuria. They and all of the other patients remained otherwise well. The urinalysis, including microscopic examination, was the most important diagnostic test in a patient with isolated gross hematuria. Nonglomerular problems were more than twice as common as glomerular diseases as a cause of isolated gross hematuria in pediatric patients The distribution of the etiologies of gross hematuria was consistent with previous studies. Although nearly half of the patients with nonglomerular gross hematuria could not be given a diagnosis, their long-term prognosis appeared to be good.