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。     

保留肾单位手术治疗儿童肾细胞癌

目的 探讨保留肾单位手术治疗儿童肾细胞癌的安全性和可行性.方法 对我院1973年1月至2016年12月收治的11例行保留肾单位手术治疗儿童肾细胞癌的临床资料进行回顾性分析.男8例,女3例;年龄4.5～13.5岁,平均7.5岁;左侧5例,右侧6例.临床表现:无痛肉眼血尿4例,腹部包块1例,行B型超声检查偶然发现6例,血尿病史1～7个月,平均3个月.结果 11例患儿均行开放性经腹膜保留肾单位手术,肿瘤直径2.2～6.9 cm,平均3.3 cm.肿瘤位于肾上极4例,肾中极背侧1例、肾中极腹侧1例、肾下极5例.手术时间88～175min,平均107 min,术中阻断肾蒂时间19～25min,平均20.4 min,出血量10～100ml,平均35ml.本组手术均安全进行,无继发出血、漏尿等并发症.术后病理提示9例为Xp 11.2易位/TFE3基因融合相关性肾癌,1例为透明细胞癌,1例为嫌色细胞癌.11例均获随访,随访时间25～129个月,平均53.2个月,无复发及死亡病例.结论 儿童肾癌应用保留肾单位手术的指征应十分严格,在此前提下,保留肾单位手术治疗儿童肾癌安全、可行.     

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.     

畸胎瘤样肾母细胞瘤治疗体会及文献回顾

目的 探讨儿童畸胎瘤样肾母细胞瘤临床诊断和治疗方法,提高临床对该病的认识.方法 回顾我院2007年至2016年间诊治的4例儿童畸胎瘤样肾母细胞瘤,男2例,女2例,年龄2～40个月,平均年龄21个月;原发灶:右肾3例,左肾1例;根据COG分期为Ⅰ、Ⅱ、Ⅲ、Ⅳ期(肺及骨髓)各1例,分析其临床表现、诊断及治疗方法.结果 4例患儿均行肾脏肿瘤根治性切除术,其中Ⅲ、Ⅳ期患儿术前行化疗再行根治术;3例术后辅以化疗,术后复发1例(化疗不敏感);3例早中期患儿预后可(2例随访时间超9年,1例随访3年),1例Ⅳ患儿再次手术后化疗加放疗中.结论 儿童畸胎瘤样肾母细胞瘤是肾母细胞瘤的罕见病理类型,临床症状通常难与经典肾母细胞瘤鉴别,主要依靠术前影像学及病理诊断,患侧肾脏肿瘤根治性切除术为主要治疗手段,早中期TWT患儿预后良好,至于保肾肿瘤切除术在该类型肾母细胞瘤的应用,暂未见文献报道,本组病例数较少,也未涉及.对于晚期TWT患儿,可能因为其独特的病理特征,其预后可能比经典肾母细胞瘤更差.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

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。     

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.     

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...     

谨慎诊断儿童肾错构瘤

目的 儿童肾错构瘤需谨慎诊断.方法 选取2例外院诊断为儿童肾错构瘤的患儿为研究对象,通过影像学检查重新评估病情,作为诊断参考,两例患儿均予手术切除肿物治疗,以肿瘤病理检查为诊断依据.结果 两例患儿肿瘤病理诊断分别为右肾母细胞瘤和肾透明细胞癌.结论 肾错构瘤在儿童中发病甚少,肾错构瘤与肾恶性肿瘤在儿童术前临床表现及影像学...     

隐蔽切口法在机器人辅助腹腔镜下儿童肾肿瘤根治术标本取出中的应用体会

目的 初步探讨隐蔽切口法(Hidden Incision)在机器人辅助腹腔镜下儿童肾肿瘤根治术(Robot-assisted laparoscopic radical nephrectomy,RAL-RN)标本取出中应用的价值。方法 回顾性分析2015年8月至2019年2月收治的6例机器人辅助腹腔镜下肾肿瘤根治性切除患儿的临床资料。其中男4例,女2例;平均年龄3岁11个月;肾母细胞瘤3例,Xp11.2易位/TFE3基因融合相关肾癌2例,后肾腺瘤1例;肿瘤位于左肾3例,右肾3例。术中均采取健侧60°卧位,目镜Trocar位于脐缘(病灶对侧缘),操作孔1位于前正中线脐上6~7 cm(具体取决于患儿腹壁空间),操作孔2进皮点位于前正中线与下腹部腹横纹交点,进腹腔点在进皮点上方约1.5 cm处(Trocar于皮下潜行)。如需辅助孔,则辅助孔Trocar置于患侧下腹部腹横纹延长线上。所有患儿均实施根治性切除术,标本切除后完整置入标本袋,延长下腹部切口(切口长度大于等于瘤肾最小径),标本袋经下腹部腹横纹切口完整取出。结果 6例患儿中,采用辅助孔3例,未采用辅助孔3例。切口长度5~8 cm,平均6.5 cm。所有标本均经下腹部腹横纹切口通道完整取出,无一例术中中转开放。术后住院4~7 d,平均4.5 d。术后伤口无活动性出血、裂开、感染等情况发生。术后随访4~46个月,平均25个月。术后伤口恢复可,未见明显瘢痕增生。结论 隐蔽切口法可安全、有效地应用于儿童RAL-RN术中标本的取出。     

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively.Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.     

Kasabach-Merritt综合征治疗中自体原位植皮术的应用

目的 对Kasabach-Merritt综合征手术治疗过程中使用自体原位植皮术,评价其治疗效果,提出一种新的手术治疗方式.方法 回顾性分析2010年至2015年间自体原位植皮手术治疗的Kasabach-Merritt综合征患儿23例,病例纳入标准:均为瘤体分布弥散,皮肤大面积(＞5 cm×5 cm)异常,手术切除瘤体后创面缝合困难;非手术治疗方法无效;血小板值在2×109/L～31×109/L.23例Kasabach-Merritt综合征患儿瘤体均位于四肢,上肢6例,下肢17例;男9例,女14例;年龄7 d～9个月.总结该手术治疗方法的手术要点及术中、术后处理,分析治愈率及术后并发症.术后均经病理学检查证实为卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)20例,丛状血管瘤(tufted angioma,TA)3例.结果 23例患儿均在术后1周内血小板值恢复正常,随访1～5年,治愈率为100％;18例患儿自体皮片原位移植全部成活,有3例成活面积约90％,经后续换药处理后愈合,2例原位皮片移植成活约60％,行二期皮肤移植修补手术后治愈,术后并发症少.结论 手术治疗Kasabach-Merritt综合征可作为有效的治疗方法,本组资料显示,自体原位植皮术作为一种新的手术方式,治愈率100％,术后并发症少,其对符合适应证者是一种可选择治疗手段.     

骨卡波西型血管内皮细胞瘤1例并文献复习

目的 探讨儿童骨卡波西型血管内皮细胞瘤(Kaposiform hemangioendothelioma,KHE)的临床特征 、病理特点 、诊疗方法 及预后情况,以提高对该病的诊治水平.方法 以2014年11月9日南京医科大学附属儿童医院骨科收治的1例儿童骨KHE患者为研究对象,并回顾分析其临床资料;检索维普、万方、CN...     

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.     

Nephron-sparing procedures in 11 patients with Wilms' tumor

PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed. The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation. MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination. In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium. All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients. RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range. In six patients primary PN was performed successfully. In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN. In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected. This patient evolved a local relapse 19 months after PN and had to be nephrectomised thereafter. In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis. One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively. The other two stage V tumor patients have creatinine clearance levels within the normal range. CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed. PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed. Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy. PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.     

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。     

儿童腹部手术中发现异位胰腺的外科处理

目的 探讨术中偶然发现异位胰腺的外科处理原则.方法 回顾性分析2008年1月至2016年1月我院小儿腹部手术中偶然发现的7例异位胰腺患儿的临床资料.其中,男3例,女4例;年龄1 d～7岁,平均19个月.术前诊断疾病分别是:先天性胆总管囊肿、环状胰腺、先天性肠闭锁、先天性肥厚性幽门狭窄、先天性巨结肠、先天性肠旋转不良、急性阑尾炎并腹膜炎.经腹开放手术2例,腹腔镜手术5例.异位胰腺位置如下:胃窦部、十二指肠、回肠各1例,空肠3例,回肠梅克尔憩室1例.结果 6例异位胰腺行手术切除,包括:病灶部位肠切除肠吻合5例、胃壁部分切除修补术1例;急性阑尾炎并腹膜炎术中发现的异位胰腺未处理.异位胰腺组织均位于黏膜下层,1例部分侵及肌层.本组根据Heinrich分型:Ⅰ型2例,Ⅱ型3例,Ⅲ型1例.手术顺利,术后原有疾病治愈,无并发症出现,分别随访3个月～1年,恢复良好.结论 小儿腹部手术中偶然发现的异位胰腺,综合考虑其发生并发症及手术风险,应尽可能手术切除.     

Pretreatment, ultrasound-guided cutting needle biopsies in childhood renal tumors

BACKGROUND: The current International Society of Paediatric Oncology (SIOP)-10 protocol does not allow pretreatment histological classification of low-stage renal tumors in children for fear of needle tract recurrences. The aims of this retrospective study were to evaluate the safety, sensitivity, and specificity of ultrasound-guided cutting needle biopsies (UCNB) performed at our institution in pediatric patients with renal tumors. PROCEDURE: Of 28 pediatric patients presenting with a renal tumor between 1988 and 1996, 25 underwent biopsy with the Biopty biopsy instrument (needle diameter 1.2 mm). The preoperative biopsy and nephrectomy slides were reviewed by a SIOP reference pathologist. The patients' hospital records were reviewed and biopsy complications were noted. RESULTS: At review of the nephrectomy slides, the diagnoses were: Wilms tumor (16 patients), with anaplasia in one case, rhabdoid tumor (2 patients), neuroblastoma (2 patients), mesoblastic nephroma (2 patients), clear cell sarcoma (1 patient), malignant teratoma (1 patient), and renal cell carcinoma (1 patient). No needle tract recurrence or other major complication was observed. The only complication was local pain at the biopsy site, which occurred in 24% (6/25) of the cases. The sensitivity of UCNB was 76% (19/25); five biopsies did not yield diagnostic material and one was not concordant. All cases of Wilms tumor were correctly diagnosed by UCNB, but only 33% (3/9) of the other tumors. CONCLUSIONS: In all cases of Wilms tumor a correct diagnosis was made. The overall sensitivity was 76%. UCNB proved to be a safe procedure that was not associated with needle tract recurrence or other serious complications.