肾母细胞瘤切除术中探查对侧肾脏是必须的吗?

目的：探讨肾母细胞瘤切除术中对侧肾脏探查的必要性。方法：1979年10月至1994年12月，共81例肾母细胞瘤患儿均于术前接受B超、CT和IVU检查，所有患儿均在本院接受手术并化和放疗。15例患儿接受了MRI成像检查。结果：76例患儿术前诊断和为单侧或双侧（仅3例）肾母细胞瘤，与手术结果完全一致，4例患儿术前怀疑为神经母细胞瘤，1例疑为畸胎瘤。3例双侧者均为小肿瘤侧半肾切除和大肿瘤侧全肾切除，单侧者均未探查对侧肾脏。术后病理证实为肾母细胞瘤。根据NWTS的分期标准，Ⅰ期34例，Ⅱ期23例，Ⅲ期15例，Ⅳ期6例，Ⅴ期3例，结果：78例单侧患儿随访6－20年，5年生存率为79．49％，患儿死于转移，复发和化疗或放疗并发症，无一例患儿发现有对侧肾母细胞瘤。结论：肾母细胞瘤切除术中无必要探查对侧肾脏。     

神经母细胞瘤,肾母细胞瘤的细胞培养在临床的应用

对我院手术切除的神经母细胞瘤6例、肾母细胞瘤14例的新鲜肿瘤标本及区域淋巴结（所取标本与病理标本等分）进行原代细胞培养。结果本组20例瘤体的细胞培养涂片检查与病理切片诊断一致。淋巴结细胞培养与病理切片诊断对照结果为：神经母细胞瘤病理切片阳性率33．3％，细胞培养阳性率50％；肾母细胞瘤病理切片诊断阳性率33．3％，细胞培养阳性率66．6％。可见常规的病理切片的局限性对于微小的转移灶，不一定被包含在片中而受到假阴性的影响，细胞培养可以弥补这一不足。     

肾母细胞瘤CD44V6表达及其意义

目的 探讨ＣＤ４４Ｖ６在不同病理类型肾母细胞瘤中表达的差异及其意义。方法 用免疫组化方法,研究１７例不同病理类型肾母细胞瘤和１３例正常肾组织中ＣＤ４４Ｖ６的表达。结果 肾母细胞瘤ＣＤ４４Ｖ６阳性率为４７．１％,明显高于正常肾组织（７．７％,１／１３）。其中,间变型肾母细胞瘤ＣＤ４４Ｖ６阳性率１００％（５／５）,非间变型阳性率２５．０％（３／１２）,两者差异有显著性意义（Ｐ＝０．００９〈０．０２５）     

小儿恶性肿瘤突变型P53蛋白的检测及临床意义

采用免疫组织化学方法检测小儿常见恶性肿瘤突变型Ｐ５３蛋白的表达情况并探讨其临床意义，用ＤＯ－１Ｐ５３单克隆抗体，ＬＳＡＢ法测定４５例小儿恶性肿瘤石蜡切片的Ｐ５３表达，１９例神经母细胞瘤３例阳性，均为ＩＶ期患儿，其中１例阳性为睾丸转移瘤的发瘤阴性，１６例肾母细胞瘤４例阳性，其中３例预后不良型均为阳性，１０例恶性淋巴瘤５例阳性，均为Ⅲ，Ⅳ期患儿，结果表明突变型Ｐ５３蛋白的表达与三种肿瘤的临床分期，组织     

小儿常见恶性实体瘤多药耐药性蛋白的定位检测

对４９例小儿常见恶性实体瘤冰冻切片进行多药耐药蛋白（Ｐ－１７０）的免疫组化定位检测。术前化疗肿瘤多药耐药性蛋白阳性率为肾母细胞瘤４４％（４／９），神经母细胞瘤１００％（７／７），横纹肌肉瘤７５％（３／４），术前未化疗肿瘤多药耐药性蛋白的阳性率为肾母细胞瘤２３％（３／１３），神经母细胞瘤４０％（４／１０），横纹肌肉瘤３３％（２／６）。小儿恶性肿瘤多药耐药性检测对揭示肿瘤耐药、复发的原因，估计化疗效果和判断预后以及指导化学治疗具有一定意义。     

双侧肾母细胞瘤的诊治进展

双侧肾母细胞瘤有同时发生和异时发生两种表现形式,同时性双侧肾母细胞瘤的发生率明显高于异时性。本文主要介绍同时性双侧肾母细胞瘤。根据多个文献报道,在肾母细胞瘤中,同时性双侧肾母细胞瘤发生率约为3．6％～8％。其中Hamilton等在3335例肾母细胞瘤中检出188例双侧肾母细胞瘤,后者发病率占全部肾母细胞瘤的5．6％。双侧肾母细胞瘤起病较早,发病年龄中位数为2．3岁,明显低于单侧肾母细胞瘤3．9岁的中位数年龄。双侧肾母细胞瘤组织学预后不良型较单侧多见,约为10％。双侧肾母细胞瘤合并畸形的发生率较高,常见的有睾丸下降不全、尿道下裂和输尿管重复畸形等。因此,双侧肾母细胞瘤的治疗具有较高的挑战性。其理想的治疗结果是,既保证较高的治愈率,又维持正常的肾功能,防止发生终末期肾功能衰竭。现就其诊治进展综述如下。     

双侧Wilms瘤的外科治疗

双侧Ｗｉｌｍｓ瘤（ＢＷＴ）占ＷＴ的３．０％～１０．０％，其疗效极差，１９７７年Ｂｉｓｈｏｐ［１］报道３０例，其２年存活率为８７．０％，说明疗效明显提高。我们曾经治疗３例。例１女，２岁。因右上腹肿块入院。经临床、实验室、腹部超声及ＩＶＰ检查，诊断为双侧...     

一侧肾母细胞瘤术后对侧肾复发一例

一侧肾母细胞瘤术后对侧肾复发一例郭一滨，刘贵麟患儿；女，１岁。因无痛性肉眼血尿于１９８６年３月入院。体检：右肋下可触及１０．５ｃｍ×６．５ｃｍ肿物。质硬，压痛（±）。ＩＶＰ右肾盂未显影，左肾盂显影正常。Ｂ超右侧腹可探及１０ｃｍ×８ｃｍ×７ｃｍ圆形低回...     

儿童实体肿瘤中血管内皮生长因子的表达

目的 探讨血管内皮生长因子在儿童实体肿瘤中表达的意义。方法 对２０例肾母细胞瘤和２５例神经母细胞瘤，１５例对照组标本进行血管内皮生长因子（ＶＥＧＦ）免疫组化分析。结果 ＶＥＧＦ在神经母细胞瘤中的表达阳性为１３例（１３／２５），在肾母细胞瘤中表达阳性为１２例（１２／２０），ＶＥＧＦ的表达与肿瘤的临床分期、病理类型无统计学意义，而与肿瘤的转移存在显著性差异（Ｐ〈０．０５）。对照组阳性为２例（２／１５）     

肾母细胞瘤血清蛋白质标记物检测与分期模型构建研究

目的筛选出肾母细胞瘤患儿特异性血清蛋白质标记物，建立肾母细胞瘤临床分期模型与CT分期进行对照分析，并评价其临床应用价值。方法应用SELDI-TOF-MS技术检测80例血清标本（术前肾母细胞瘤30例，其他恶性肿瘤30例，正常儿童20例），用ZUCI-Protein Chip Data Analyze System分析软件进行数据处理，结合支持向量机（support vector machine，SVM）建立肾母细胞瘤临床分期模型。结果筛选出2个m／z位于4153．9和3257．6的蛋白质标记物，区分肾母细胞瘤Ⅲ和Ⅳ期与肾母细胞瘤Ⅰ和Ⅱ期蛋白质谱差异表达模型的敏感性为100％，特异性为93。8％；区分肾母细胞瘤与正常儿童、腹腔实体肿瘤及肾脏其他恶性肿瘤的特异性是100％，敏感性是100％、80．0％、100％；临床分期模型可以特异性地将各期区分开来，其特异性及敏感性均为100％；通过肾母细胞瘤早期诊断模型中的2个m／z（6984．4，6455．5）血清标记物进行分析得出肾母细胞瘤各期情况如下：Ⅳ期相对于Ⅲ期低表达；Ⅲ期相对于Ⅱ期低表达；Ⅱ期相对于Ⅰ期低表达；Ⅰ期相对于正常儿童低表达；后者相对高表达；临床分期越晚，m／z强度就越低表达。蛋白芯片分期准确性与病理一致，达到100％，在分期定性问题上优于CT（Ⅰ期100％，Ⅱ期85．0％，Ⅲ期85．0％，Ⅳ期75．0％）。结论用SELDI-TOF-MS结合SVM建立的肾母细胞瘤临床分期模型可弥补CT在肾母细胞瘤分期定性问题匕的不足。     

儿童肾母细胞瘤HMGB1、RAGE及VEGF的表达及意义

目的 探讨高迁移率族蛋白-1(HMGB1)、晚期糖基化终末产物受体(RAGE)和血管内皮生长因子(VEGF)在小儿肾母细胞瘤组织中的表达及其在肿瘤血管浸润、淋巴结转移中的意义.方法 收集郑州大学第一附属医院2003年1月至2010年1月小儿外科手术切除经病理证实为肾母细胞瘤的石蜡标本50例、15例同期相应瘤旁组织和7例正常肾组织标本.应用免疫组织化学SP染色辅以计算机图像分析系统分析结果的方法,研究HMGB1、RAGE及VEGF在各组标本中的表达及意义.结果 HMGB1、RAGE在肾母细胞瘤组织中的表达(144.46±13.55、138.18±12.26)与两者在瘤旁组织和正常肾组织中表达(107.47±5.27、103.91±4.29;100.98±4.82、100.82±3.32)相比差异均有统计学意义(P＜0.05),HMGB1在瘤旁与正常肾之间表达差异有统计学意义(P＜0.05).VEGF在瘤体组与瘤旁组中(147.57±13.77,140.28±7.85)和在正常肾组织组中的表达(106.38±1.92)相比差异显著(P＜0.05).三者在临床分期Ⅲ～Ⅳ期和预后不良组织型组及淋巴结转移组的肾母细胞瘤组织中的表达(148.69±12.17、147.73±6.71、163.14±7.50;157.88±4.44、155.29±3.97、169.17±4.42;152.11±7.36、151.56±5.46、163.83±7.20)显著高于临床分期Ⅰ～Ⅱ期和预后良好组织型及无淋巴结转移组(P＜0.05)(139.23±5.83、133.30±11.23、140.85±8.87;139.52±5.22、135.39±9.71、143.94±10.39;138.61±5.59、133.00±8.75、141.18±8.95).相关分析显示肾母细胞瘤组织中HMGB1与RAGE、VEGF的表达均成正相关(r=0.424,P=0.002;r=0.453,P=0.001),RAGE与VEGF之间无明显相关(r=0.237,P=0.101).结论 HMGB1、RAGE和VEGF的高表达与肾母细胞瘤的临床分期、病理类型、淋巴结转移有关,参与了肿瘤血管浸润及淋巴结转移,HMGB1/RAGE可能是促进肾母细胞瘤转移的一条信号通路,给我们靶向治疗肾母细胞瘤提供了新的思路.

Abstract：

Objective This study aim to assess the expressions of high mobility group box chromosomal proteinl ( HMGB1) and receptor foradvanced glycation end products (RAGE) and vascular endothelial growth factor (VEGF) in Wilms'tumor and their clinical significance both in the tumor blood vessel infiltrates and in the lymph node metastasis. Methods Fifty cases of Wilms'tumor samples, which had been confirmed by pathology, were collected from The First Affiliated Hospitals of Zhengzhou University from january 2003 to january 2010. And 15 cases were taken from the adjacent kidney tissues at the same time. Other 7 cases were normal kidney tissues. The expression of HMGB1, RAGE and VEGF were detected by the Immunohistochemical SP staining in each group of specimens. The expression intensity was analyzed by computer image processing and their significance in each group of specimens were studied. Results The expressions of HMGB1 and RAGE in the Wilms'tumor tissues (144. 46 ± 13. 55、138.18 ±12. 26) were compared with those in the adjacent kidney tissues and in the normal kidney tissues( 107. 47 ± 5. 27、103. 91 ± 4. 29; 100. 98 ± 4. 82、 100. 82 ± 3. 32). The expressions of HMGB1 in the adjacent kidney tissues and in the normal kidney showed significant differences. There was a significant differences between the Wilms' tumor group and the adjacent kidney tissues or in the normal kidney tissues ( F ＜ 0.05 ). The expressions of VEGF proteins in Wilms'tumor tissues and in the adjacent kidney tissuess(147. 57 ± 13. 77,140. 28 ± 7. 85) comoared to those in the normal kidnev tissues(106. 38 ± 1. 92)were remarkably different(P＜0. 05). The expressions of HMGB1 ,RAGE and VEGF proteins in Wilms' tumor tissues of stage Ⅲ-Ⅳ and high risk histopathology and lymph node metastasis group (148. 69 ± 12.17、147. 73 ± 6. 71、163.14 ± 7. 50; 157. 88 ± 4. 44、155. 29 ± 3. 97、169. 17±4.42;152. 11 ± 7. 36、151. 56 ± 5. 46、163. 83 ± 7. 20)were significantly higher than those of stage Ⅰ-Ⅱ and low risk histopathology and no lymph node metastasis group(P＜0. 05) (139. 23 ± 5. 83、133. 30 ± 11. 23、140. 85 ± 8. 87; 139. 52 ± 5. 22、135. 39 ± 9. 71、143. 94 ± 10. 39; 138. 61 ± 5. 59、133. 00 ±8. 75、141.18 ± 8. 95). There was a positive correlation between the expressions of HMGB1 with RAGE and VEGF(r = 0. 424, P = 0. 002; r = 0. 453, P = 0. 001), but the correlation between the RAGE and VEGF is not clear (r = 0. 237, P = 0. 101). Conclusions There was a high expression of HMGB1 ,RAGE and VEGF in the Wilms'tumor tissues and they are partly related to the clinical stages and pathological type and lymph node metastasis of Wilms'tumor. They perhaps participated in the tumor blood vessel infiltration and the lymph node metastasis and the pathway of HMGB1/RAGE is perhaps the main mechanism correlated with the metastasis of Wilms'tumor.     

Immunohistochemical analysis of gamma catenin in Wilms' tumors

The aim of our study was to investigate the expression of gamma-catenin in normal kidney and in Wilms' tumor by immunohistochemistry and to correlate the results with tumor stage, histological type, and prognostic group. We investigated 28 cases of Wilms' tumor, 2 Wilms' tumor metastases in lungs, and 1 specimen of normal renal tissue. Expression of gamma-catenin was detected in 14 cases. There was a weak inverse relationship between gamma-catenin expression and tumor stage. Expression of gamma-catenin was detected in various histologic types of Wilms' tumor, but there was no statistically significant correlation, except in cases with diffuse anaplasia that were negative. In 2 metastatic cases and in the case of bilateral Wilms' tumor gamma-catenin immunostaining was not observed Our findings suggest an absence of strong correlation between the loss of gamma-catenin and unfavorable outcome.     

细胞性纤维连接蛋白在肾母细胞瘤中表达的研究

用过氧化物酶－抗过氧化物酶技术光镜下观察细胞性纤维连接蛋白（ＣＦＮ）在６３例肾母细胞瘤组织中表达强度与分布，探讨其与组织类型、预后之间的关系。结果，ＣＦＮ表达与瘤组织类型有关：上皮型，ＣＦＮ分布以肾小管样结构周围为主，而且表达强度高；间质型，ＣＦＮ多分布在细胞内，两者差异有显著意义（Ｐ＜０．０５）。ＣＦＮ表达情况与肿瘤的临床分期无关。当ＣＦＮ分布在细胞周围，表达强度高者，预后好，生存５年以上者多。ＣＦＮ表达可作为判定肾母细胞瘤预后的指标之一     

Multifocal nephroblastomatosis: clinical significance and imaging

The correlation between nephroblastomatosis and Wilms' tumor has been established in the pathology literature. Two cases of multifocal nephroblastomatosis in the kidney contralateral to a Wilms' tumor are presented. The importance of these lesions and their recognition is discussed.     

Teratoid Wilms' Tumor: Report of a Unilateral Case

A unilateral teratoid Wilms' tumor was removed 2.5 weeks after the institution of chemotherapy. Teratoid Wilms' tumor is an extremely rare renal tumor, and only four cases, all bilateral, have been reported. Because of the finding of deep cortical intralobar nephroblastomatosis, strongly associated with bilateral Wilms' tumors, the patient has been closely followed since surgery without evidence of tumor in the remaining kidney at 2 years.     

Evidence for clonal development of Wilms' tumor.

To assess the clonality of Wilms' tumor, glucose-6-phosphate dehydrogenase (G6PD) enzymes were studied in normal and tumor tissue from 11 black girls who were heterozygous for G6PD. Normal tissues expressed both A and B type G6PD, whereas only a single G6PD enzyme was found in all tumor specimens. These data support the clonal nature of Wilms' tumor. In the one patient with bilateral disease, type B G6PD was found in both a recurrence and a subsequent tumor in the contralateral kidney. This finding is consistent with either the chance occurrence of the same G6PD in independent tumors or persistence of the original malignant clone. Another patient, who presented with the nephroblastomatosis complex (a precursor of Wilms' tumor), also had only type B enzyme detected. Further studies in patients with bilateral disease or the nephroblastomatosis complex, including the use of molecular biologic probes, are needed to test the hypothesis that Wilms' tumor in these cases arises from a somatic mutation as a second event in persons with an underlying genetic alteration.     

Is renal salvage feasible in unilateral Wilms' tumor? Proposed computed tomographic criteria and their relation to surgicopathologic findings

The ability of computed tomographic (CT) criteria to predict the potential for resection with renal salvage in unilateral Wilms' tumor was evaluated retrospectively in 10 children given preoperative treatment for initially inoperable disease. Criteria were (a) tumor involving only one pole and occupying less than one-third of the kidney, (b) functioning kidney, (c) no invasion of collecting system or renal vein, and (d) clear margins between tumor, kidney, and surrounding structures. Review of preoperative CT scans correctly predicted nonsalvageability (as assessed by surgicopathologic findings) in seven cases. Sufficient pathologic data were lacking to confirm positive CT predictors in one case. One patient was rated resectable with salvage on surgicopathologic review, but not by CT criteria, and in one case, a prediction could not be made. The potential for renal salvage may be greater in samples with smaller initial tumor size, and addition of other imaging modalities might enhance the accuracy of prediction. Further studies are needed to assess the feasibility of prospective trials evaluating the risks and benefits of partial nephrectomy in unilateral Wilms' tumor.     

Rare localization of extrarenal nephroblastoma in 1-month-old female infant

Extrarenal occurrence of Wilms' tumor is exceptional and the diagnosis is almost always made after surgery. The exact mechanism whereby a Wilms' tumor occurs in extrarenal tissue is unknown. The tumor is most commonly located in the retroperitoneum or inguinal region. Localization in subcutaneous tissue is extremely rare. In this paper, the case of a 1-month-old female infant with an extrarenal Wilms' tumor located in the lumbosacral region is presented. Surgical excision is the treatment of choice, and the same general therapeutic rules should be followed as when the kidney is affected.