儿童膀胱内翻性乳头状瘤合并膀胱结石1例

膀胱内翻性乳头状瘤(inverted papilloma of bladder, IPB)是一种罕见的良性肿瘤, 儿童IPB更为罕见。天津市儿童医院收治1例膀胱内翻性乳头状瘤合并膀胱结石患儿, 经尿道膀胱镜检查后, 考虑膀胱肿瘤位于左侧输尿管开口处, 遂行膀胱肿物切除、左侧输尿管膀胱再吻合、膀胱造瘘以及膀胱切开取石术治疗, 术后恢复良好, 随访18个月未见肿瘤复发。     

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。     

儿童非横纹肌软组织肉瘤临床预后因素及治疗对策

目的 分析18岁以下儿童和青少年的非横纹肌肉瘤的生存率,并探讨常见肿瘤的治疗策略。方法 对1989年1月到2002年12月治疗并随访的67例非横纹肌肉瘤病例的资料进行分析,其中滑膜肉瘤16例,恶性纤维组织细胞瘤9例,纤维肉瘤9例,脂肪肉瘤7例,骨外尤文氏肉瘤/原始神经外胚叶瘤7例,腺泡状软组织肉瘤6例,平滑肌肉瘤4例,恶性外周神经鞘瘤、透明细胞软组织肉瘤、血管肉瘤各2例,上皮性软组织肉瘤、促纤维增生性小圆细胞肿瘤、恶性肾外横纹肌样肿瘤各1例。引入年龄、性别、肿瘤大小、外科病理分期、是否接受化疗和放疗等因素,应用SPSS10.0统计软件,采用COX回归和χ^2检验进行统计学分析。结果 影响非横纹肌肉瘤生存率的唯一临床因素是外科病理分期。手术完全切除比肿瘤残留或转移者的生存率更高,Ⅰ、Ⅱ期和Ⅲ、Ⅳ期的2年EFS分别为89.74%和17.86%,差异有统计学意义。无肿瘤残留者生存率高于肿瘤残留或不能切除者。结论 儿童和青少年非横纹肌软组织肉瘤罕见,各肿瘤的临床特征不同,尚无统一治疗方法。治疗原则仍以达到无肿瘤残留为目标的外科手术治疗为主;除尤文氏瘤/原始神经外胚叶瘤和滑膜肉瘤已经证实化疗有效外,其他肿瘤的术后辅助化疗和放疗尚存在争议。新辅助化疗对于部分不能切除的肿瘤可以提高手术切除率。成立全国性的协作组可以尽快积累病例,增加治疗经验,制定规范的治疗方案,是改善儿童非横纹肌软组织肉瘤疗效的有效途径。     

儿童睾丸旁肿物的诊治分析(附27例报告)

目的总结儿童睾丸旁肿物的诊治经验, 以期提高儿童睾丸旁肿物术前诊断水平, 制定合理个体化治疗方案。方法回顾性分析2010年1月至2021年5月经北京儿童医院手术治疗的27例睾丸旁肿物患儿临床资料。患儿平均年龄为7.4岁, 范围为0.3～14.5岁。肿物位于左侧12例, 右侧14例, 双侧1例。患儿术前均接受体格检查及超声检查。自发现睾丸旁肿物至接受手术的平均时间为4.5个月, 范围为1周至3年。患儿经腹股沟切口精索高位结扎瘤睾切除术8例, 经腹股沟切口保留睾丸睾丸旁肿物切除术1例, 经阴囊切口保留睾丸单纯睾丸旁肿物切除术18例。6例睾丸旁横纹肌肉瘤患儿均无淋巴结及远处转移, 均按低危组以长春新碱+放线菌素D+环磷酰胺组合化疗;1例白血病转移患儿术后继续化疗;其余患儿术后未予其他治疗。结果病理结果为良性肿物17例, 恶性肿瘤10例。良性肿物包括附睾囊肿10例, 脾组织(脾性腺融合)1例, 肾上腺残基瘤1例, 附睾平滑肌瘤1例, 婴儿纤维性错构瘤1例, 附睾纤维性假瘤1例, 脂肪母细胞瘤1例, 淋巴管瘤1例。恶性肿瘤包括睾丸旁横纹肌肉瘤(胚胎型)6例, 黑色素神经外胚层肿瘤1例, 高分化脂...     

儿童尤文肉瘤类肿瘤14例临床分析

目的 提高对儿童尤文肉瘤类肿瘤的认识及疗效,评价应用上海儿童医学中心横纹肌肉瘤-2002方案化疗(简称RS-2002方案)的有效性及安全性.方法 制定并实施以个体因素相关的诊断治疗方案,对1997年9月至2006年9月上海儿童医学中心收治的初治尤文肉瘤类肿瘤患儿进行分析.连续病例总数为19例,家长放弃治疗5例,其余14例进入治疗方案,男9例,女5例;中位年龄7.04岁(范围:1.58～11.67岁),<10岁9例;就诊时为局限性疾病9例,伴远处转移性病变5例.所有病例均经外科手术获得病理学明确诊断,分型为尤文肉瘤9例,原始神经外胚层肿瘤5例.采用多学科联合综合治疗模式,根据疾病的扩散程度进行临床分组,不同组别接受不同强度的化疗方案.肿瘤局部控制方法为手术切除及(或)局部放疗.对患儿治疗前后、治疗过程中及停药随访中进行影像学跟踪评估.运用SPSS 11.0统计软件对患儿临床数据进行统计分析,生存率分析采用Kaplan-Meier法.结果 随访至2007年4月30日,13例患儿存活.11例处于无复发状态,中位随访期为41个月(7～115个月);3例复发,疾病复发距诊断的中位时间为23个月(16～30个月).10年总生存率(OS)为88.9%±10.5%,10年无疾病复发率(DFS)为72.2%±13.8%.1例患儿发生第二肿瘤.无治疗相关死亡发生.结论 多学科联合的综合治疗模式及RS-2002化疗方案能有效并安全地治疗儿童尤文肉瘤类肿瘤.     

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.     

儿童复发性肾母细胞瘤临床特点和治疗经验

目的 探讨儿童复发性肾母细胞瘤临床特点和治疗经验.方法 回顾性研究2003年1月-2011年4月收治的17例儿童复发性肾母细胞瘤临床资料.其中9例采用手术联合术前术后化疗、放疗治疗方法.采用SPSS 17.0软件进行统计学处理,运用Kaplan-Meier曲线进行生存率分析.结果 本组病例按美国肾母细胞瘤研究组织(MWTSG)临床分期分为Ⅰ期4例、Ⅱ期8例、Ⅲ期3例、Ⅳ期1例、V期1例.原发瘤与复发瘤病理类型一致,其中预后良好型12例,预后不良型5例.全组有9例共接受12次再次手术,其中根治性切除1次,肿瘤单纯切除11次,术后化疗9例,放疗3例.再次手术组1 a累积生存率高于未再次手术组,差异有统计学意义(P＜0.05).结论 尽管肾母细胞瘤可能反复复发,但病理类型仍然与原发瘤保持一致.治疗策略在于尽可能完整切除复发瘤和转移瘤,同时联合术前术后化疗、放疗可以提高患者生存率.     

输尿管内翻性乳头状瘤12例

目的探讨输尿管内翻性乳头状瘤的诊断和有效治疗方法。方法1996~2004年我院收治输尿管乳头状瘤患儿12例,均行病变切除、输尿管及肾孟输尿管成形术。结果12例患儿经手术治疗后随访中未出现腹痛、血尿等症状。肿物组织检查,光镜下显示肿瘤表面为增生的移行上皮,中心为毛细血管和含少量平滑肌束的纤维结缔组织,伴炎性细胞浸润。病理诊断为内翻性乳头状瘤。均未发现复发或转移。结论小儿输尿管乳头状瘤在术前诊断上有一定困难。术后病理检查,可确诊。局部切除病变的输尿管并行肾盂输尿管成形术具有良好的疗效。术后随访很有必要。     

儿童恶性肾横纹肌样瘤3例临床分析及文献回顾

目的通过总结3例原发于肾脏的儿童恶性横纹肌样瘤的临床资料,探讨儿童肾横纹肌样瘤的诊断、治疗及预后。方法收集我院近8年来收治的3例病理明确诊断的原发于肾脏的恶性横纹肌样瘤（男2例,女1例）,对其发病特点、病理特点、治疗及随访等临床资料进行总结分析,并复习相关文献。结果根据国际儿童肿瘤组织肾横纹肌样瘤的临床分期诊断,3例患儿均为Ⅲ期。均行手术、化疗及放疗治疗。化疗以ICE与VDC交替方案为主,化疗周期分别为6、6、15周期,放疗采用外放疗。随访至2012年7月（3例随访时间分别是8、14、8个月）。2例获完全缓解（CR）,1例复发后经手术、化疗及放疗后病情部分缓解（PR）。结论恶性肾横纹肌样瘤是一种少见、病理形态独特的肿瘤。治疗应在明确诊断和确切分期的基础上,行手术、化疗和选择性放疗的综合性治疗。     

婴幼儿肾脏恶性横纹肌样瘤诊治分析

目的探讨儿童肾脏恶性横纹肌样瘤(malignant rhabdoid tumor of the kidney,MRTK)的临床及病理特点。方法回顾性分析2009年1月至2015年4月本院收治的15例MRTK患儿临床、病理及随访资料。结果 15例患儿中,男性7例,女性8例,年龄3~28个月,平均年龄12.6个月。左侧6例,右侧9例。血尿10例,腹部包块4例,超声发现1例。按NWTS 3进行肿瘤分期:Ⅰ期2例,Ⅱ期5例,Ⅲ期5例,Ⅳ期3例。术前予化疗1例,肾动脉栓塞1例。予保留肾单位的肿瘤剜除术1例,瘤肾切除14例。病理检查结果均提示肾脏恶性横纹肌样瘤。术后化疗14例,放疗4例。11例获随访,9例死亡,2例无瘤存活分别2年、6年,4例失访。结论儿童肾脏恶性横纹肌样瘤好发于婴幼儿,血尿多见,进展迅速,恶性度高,易发生肺脑转移,需要手术+化疗+放疗的综合治疗,预后差。     

Successful treatment of inflammatory myofibroblastic tumor with malignant transformation by surgical resection and chemotherapy

Inflammatory myofibroblastic tumor (IMT) is a tumor composed of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. The authors present the case of a 7-year-old boy with an abdominal mass diagnosed as IMT with malignant transformation. The tumor recurred twice after attempts at resection and was initially treated with vincristine and etoposide. After a third recurrence and incomplete resection, he was treated with cisplatin, Adriamycin, and methotrexate. He is disease-free after 2 years, representing successful combined surgery and chemotherapy in the treatment of malignant IMT. The use of chemotherapy for aggressive myofibroblastic tumors is reviewed.     

小儿肾脏原始神经外胚层肿瘤一例及文献复习

目的 报道1例发生在儿童左肾的原始神经外胚层肿瘤(PNET),并通过文献复习,提高临床医师对此罕见疾病的认识.方法 通过回顾性分析1例发生在儿童肾脏的PNET的临床表现、病理形态特点及免疫表型特征,并结合相关文献复习.结果 术前未能明确诊断,术后经病理证实为PNET.男性患儿,7岁,因左腹部疼痛入院,影像学发现肾占位,行肾切除术.光镜下,肿瘤由核圆浓染、形态一致的原始小圆细胞组成,瘤细胞弥漫成片分布,形成特征性的Homer-Wright菊形团结构,核分裂象易见,可见大片坏死.免疫组化示:突触素(Syn)、神经元特异性烯醇化酶(NES)和波纹蛋白(Virnentin)阳性,而CgA、CD99、S-100、Melan-A、HMB45、CK、EMA等均阴性.患儿术后予环磷酰胺、阿霉素及长春新碱化疗及放疗.3个月后失去随访.结论 发生在儿童肾脏的PNET是一种临床上非常罕见的小圆细胞恶性肿瘤,高度恶性且极易复发和转移,术前常被误诊为肾母细胞瘤,主要依靠组织病理学和免疫组织化学确诊.目前主要治疗方法是手术切除加术后化疗,采用合理的综合治疗方案可延长生存期.     

Multiple fibroepithelial polyps of the upper ureter in a 17-year-old boy--case report and review of the literature.

Benign tumours and primary malignant tumours of the ureter are uncommon in adults and extremely rare in children. The clinical symptoms are flank pain, urinary tract infection, and macro/micro-haematuria. There is an incomplete ureteral obstruction and filling defect on intravenous urography (IVU). Optimum treatment of this lesion results in renal preservation. Uretero-renoscopy is currently the best method available for the identification and histological diagnosis of ureteral polyps. Recommended operative procedures are pyeloureteric junction (PUJ) resection with Anderson-Hynes pyeloplasty, ureteric resection with end-to-end anastomosis or with uretero-cysto-neoanastomosis (UCNA), ureteric resection with renal autotransplantation. Ureteronephrectomy is not indicated. A case of ureteral polyps in a 17-year-old boy with the chief complaint of left flank pain is reported here. The excretory urogram and renal scan showed left hydronephrosis. Resection of the pyeloureteral junction, partial resection of the upper ureter containing the lesions--multiple branching 30-40 mm long polyps with a common basis--and Anderson-Hynes pyeloplasty were performed. The pathological diagnosis was benign fibroepithelial polyps of the ureter. Convalescence was uneventful and after 4 years of follow-up, excretory urogram and ultrasonography showed good renal function and improvement of hydronephrosis.     

Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall

Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents. It is extremely malignant with a high frequency of both metastatic spread and of local recurrence. Cure requires intensive therapy to control both distant and local disease. Surgery and high-dose radiotherapy can achieve equivalent local control; however, radiation is associated with the additional morbidities of second malignancy and a significant adverse impact on both cardiac and pulmonary function. The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor. This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy. The chest wall is a rare site for tumors in children and adolescents. In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors. They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate. This report concentrates on the later tumors. They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve. Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.     

Primitive Neuroectodermal Tumor of the Brain Associated with Malignant Rhabdoid Tumor of the Liver: A Histologic, Immunohistochemical, and Electron Microscopic Study

A 14-day-old white male, born with a large primitive neuroectodermal tumor of the left cerebral hemisphere, was found to have a solitary rhabdoid tumor in the liver incidentally at autopsy. Cells resembling the liver rhabdoid cells were also found by histology, immunohistochemistry, and electron microscopy in the brain tumor. The concurrence of rhabdoid cells in the tumors of the brain and liver suggests a common histogenesis and further supports the previous suggestion that the rhabdoid tumor is of neuroectodermal origin. The rhabdoid tumor in the liver in this case is likely to be a metastatic tumor from the brain rather than a second primary tumor.     

Use of appendix for complete transplant ureteral necrosis

A 3-yr-old boy with posterior urethral valves underwent cadaveric renal transplant. On the ninth day after transplantation the patient developed a urinary leak, with complete ureteral necrosis. There was insufficient length of undamaged ureter to permit ureteroneocystostomy, unavailability of a native ureter to permit ureteroureterostomy, and an inability to mobilize the transplant kidney or bladder sufficiently to permit direct pyelovesicostomy. As the kidney was otherwise functioning perfectly, we decided to create an appendiceal conduit in the hope of salvaging the patient's renal allograft. At present, 7 months post-transplant, the child is clinically well with a serum creatinine of 0.7 mg/dL. Complete ureteral necrosis is an infrequent but devastating complication following renal transplantation. We report a novel method that allowed an otherwise normally functioning cadaveric graft to be salvaged.     

Primitive neuroectodermal tumor of the brain associated with malignant rhabdoid tumor of the liver: a histologic, immunohistochemical, and electron microscopic study

A 14-day-old white male, born with a large primitive neuroectodermal tumor of the left cerebral hemisphere, was found to have a solitary rhabdoid tumor in the liver incidentally at autopsy. Cells resembling the liver rhabdoid cells were also found by histology, immunohistochemistry, and electron microscopy in the brain tumor. The concurrence of rhabdoid cells in the tumors of the brain and liver suggests a common histogenesis and further supports the previous suggestion that the rhabdoid tumor is of neuroectodermal origin. The rhabdoid tumor in the liver in this case is likely to be a metastatic tumor from the brain rather than a second primary tumor.     

Inflammatory myofibroblastic tumor of the trachea

This report described a 2-year-old boy who was presented with severe respiratory distress and stridor. Bronchoscopy and CT revealed a mass in the left anterolateral tracheal wall and histopathology showed a tracheal inflammatory myofibroblastic tumor. Initial removal by rigid bronchoscopy resulted in prompt recurrence of the tumor. Therefore, he underwent tracheal surgical resection. A bronchoscopy at 12 months after surgery did not show any recurrence sign.