成人肾母细胞瘤的诊断与治疗(附9例报告及文献复习)

目的探讨成人肾母细胞瘤的诊断和治疗方法。方法回顾性分析9例成人肾母细胞瘤的临床表现、分期、治疗及预后。结果9例患者按照美国国家肾母细胞瘤研究组（National Wilms Tumor Study,NWTS）分期：Ⅰ期3例,Ⅱ期3例,Ⅲ期2例,Ⅳ期1例。9例均行肾切除术,其中术前分别辅以化疗1例、放疗1例、肾动脉介入栓塞化疗2例。9例术后辅以放疗和化疗。8例获得随访,随访1-6年,7例生存。结论成人肾母细胞瘤是一种比较少见的恶性肿瘤,早期诊断、手术治疗、辅以放疗和化疗等可明显提高治愈率并改善其预后。     

成人肾母细胞瘤的诊断与治疗（附12例报告）

目的探讨成人肾母细胞瘤的诊断和治疗方法。方法回顾性分析12例成人肾母细胞瘤患者的临床资料。结果本组患者年龄16-63岁,表现为肉眼血尿2例,腰腹痛6例,4例为体检发现;术前诊断为肾肿瘤9例,肾盂肿瘤3例。12例均行手术治疗,术后病理均诊断为肾母细胞瘤。按照美国国家肾母细胞瘤研究组（NWTS）分期标准,本组分别为Ⅰ期5例、Ⅱ期3例、Ⅲ期3例、Ⅳ期1例。术后辅以放疗和化疗,平均随访41个月,3年生存率为48%。结论成人肾母细胞瘤是一种比较少见的恶性肿瘤,术前确诊困难,手术治疗并辅以放疗、化疗是其主要的治疗方法。     

成人肾母细胞瘤4例报告并文献复习

目的 提高成人肾母细胞瘤的诊治水平。方法 结合文献回顾性分析4例成人肾母细胞瘤的诊治情况。结果 3例行根治性切除，其中2例死于术后转移；1例行肾部分切除术，至今一年半无瘤生存。结论成人肾母细胞瘤是一种罕见的恶性肿瘤，术前诊断较困难。早期发现及诊断、手术治疗并辅以放疗、化疗等可明显提高治愈率及改善其预后。     

成人肾母细胞瘤的诊断与治疗

目的：探讨成人肾母细胞瘤的诊治方法。方法：回顾性分析7例成人肾母细胞瘤的临床资料。结果：7例均行手术治疗，6例术后辅以放疗及化疗，随访1～7年，4例仍生存。结论：成人肾母细胞瘤术前与其他肾脏恶性肿瘤难以鉴别。治疗上应尽可能手术切除肿瘤，术后根据病理分期辅以放疗和化疗。     

成人肾母细胞瘤的诊疗及预后分析（附10例报告）

目的：探讨成人肾母细胞瘤的诊疗方法和预后。方法：回顾性分析10例成人肾母细胞瘤患者的临床资料。男8例,女2例,年龄16～62岁,平均29岁。就诊症状为单纯肉眼血尿3例、腰腹部肿块或腰腹痛4例（伴血尿2例）、无症状查体发现3例。患者均行B超、IVU、尿脱落细胞学检查,8例行CT平扫＋强化检查。影像学检查提示肾占位6例、肾盂占位3例、输尿管占位1例。CT提示肾门或腹腔淋巴结肿大4例。10例均行手术治疗,根治性肾切除6例,患肾、输尿管全段切除＋膀胱输尿管口袖口状切除4例,2例根治性肾切除患者因肿物与周围粘连严重切除部分腹膜。行腹膜后淋巴结清扫5例。结果：所有病例均于术后病理确诊为肾母细胞瘤,预后良好组织型（FH）4例,预后不良组织型（UH）6例,病理证实淋巴结转移3例。按美国国家肾母细胞瘤研究组（NWTS）分期标准,分别为Ⅰ期4例、Ⅱ期3例、Ⅲ期3例。7例患者接受术后辅助放化疗。随访9例患者1～13年,5例无瘤生存,生存时间为1．5～13年,平均4．4年。1例术后1年死于肿瘤肺转移,1例术后1年死于肿瘤脑转移,2例术后2年内局部复发,再次接受手术。结论：成人肾母细胞瘤为罕见的恶性肿瘤,预后较差,确诊主要依靠病理诊断,目前尚无公认的最佳治疗手段。手术治疗并辅以合理、及时的放疗、化疗是改善其预后的有效措施。     

肾母细胞瘤综合治疗模式分析

目的 探讨肾母细胞瘤的综合治疗模式,提高肾母细胞瘤的疗效. 方法 回顾性分析1981年7月至2010年6月55例肾母细胞瘤患者资料.术前超声引导下细针穿刺活检确诊18例,术后病理确诊53例.其中Ⅰ期7例,Ⅱ期19例,Ⅲ期21例,Ⅳ期6例,Ⅴ期2例;35例有组织学分型,其中预后良好型30例,预后不良型5例.55例患者中,行肾脏肿物切除术48例,局部扩大切除术2例,双肾肿瘤剜除术1例,肾肿物切除术加肺转移灶切除术2例,2例未行手术治疗;术前化疗18例,术后化疗40例,术后放疗12例.根据年龄、分期、组织分型、治疗方式、治疗时间段及是否放疗分组,应用Kaplan-Meier法比较各组3年和5年总生存率、2年无病生存率及1年无复发生存率,分析各分组因素与肾母细胞瘤预后的关系. 结果 55例患者治疗后3年和5年总生存率及2年无病生存率分别为77.6％、69.0％和52.4％,不同分期对总生存率(P=0.006)、单纯手术与综合治疗对无病生存率(P =0.004)、是否放疗对无病生存率(P=0.03)影响有统计学意义(P＜0.05). 结论 规范的多学科协作的综合治疗模式可以显著提高肾母细胞瘤疗效.     

巨大囊性肾外型成人肾母细胞瘤破裂并肾周积液1例诊治分析

目的报告1例巨大囊性肾外型成人肾母细胞瘤破裂合并大量肾周积液病例,结合文献复习探讨该病的病理及临床特点。方法回顾性分析1例巨大囊性肾外型成人肾母细胞瘤破裂合并大量肾周积液病例的诊治经过。结果开放手术行左侧泌尿系切除术,术后辅助放化疗,随访5个月,未见肿瘤复发。结论成人肾母细胞瘤发病率低,预后较差,巨大囊性肾外型成人肾母细胞瘤极为罕见,手术治疗结合术后放化疗的综合治疗可有一定疗效。肿瘤分期、组织学分型对判断预后有一定指导价值。     

成人前列腺肉瘤7例临床分析

目的 :探讨前列腺肉瘤的诊断、治疗及预后因素。　方法 :回顾性分析我院诊治的 7例成人前列腺肉瘤临床表现、实验室检查、治疗方案、病理类型及预后。　结果 :平滑肌肉瘤 3例 ,横纹肌肉瘤 3例 ,恶性神经鞘瘤 1例。前列腺肉瘤有膀胱颈梗阻为主的复合表现 ,确诊依靠病理诊断 ,病理分型与疾病的进展相关联。 2例行根治性膀胱前列腺切除术者于术后 13个月和 2 1个月死亡 ;2例行全盆腔脏器切除术辅以放疗和 (或 )化疗者 ,随访 15个月仍存活 ;3例仅行放疗和 (或 )化疗 ,平均于初诊后 7个月死亡。　结论 :前列腺肉瘤预后较差 ,早期诊断和完整的外科切除有重要意义 ,密切随访可以早期发现复发。     

多房性囊性肾瘤

目的提高对多房性囊性肾瘤的认识。方法报告１例并结合文献讨论其发病情况,病理学特征,诊断和治疗方法。结果本例术前拟诊肾母细胞瘤,化疗后肿瘤缩小,手术完整切除,术后随访肿瘤无复发。结论确诊依靠病理学,治疗有赖于手术,对于较大肿瘤可行术前化疗,其余无需化疗或放疗,预后良好。     

肾恶性间叶瘤的诊断与治疗（附3例报告并文献复习）

目的：探讨肾恶性间叶瘤的诊断、治疗及预后.方法：回顾性分析3例肾恶性间叶瘤患者的临床资料：男2例,女1例.年龄48～68岁,平均57岁.CT平扫均为肾脏巨大不均质肿物,其中伴有大块状钙化2例,增强扫描不均质肿物有轻至中度强化.3例患者均行根治性肾切除术,术后辅以化疗或放疗.结果：3例患者术后存活6～18个月,平均11个月.结论：肾恶性间叶瘤临床罕见,术前诊断困难,确诊需依靠病理检查.综合分析临床资料及提高对此病的临床认识是避免误诊的关键.根治性切除术是其主要治疗手段,但预后不佳.     

Triple therapy for adult Wilms tumor

Adult Wilms tumor is still considered a rarity. Approximately 170 adult Wilms tumors have been reported. The final diagnosis is usually established with surgery. There is no specific radiologic diagnosis of adult Wilms tumor. We report on a case of Wilms tumor in a twenty-two-year-old black man. The tumor was removed with radical nephrectomy and classified as Stage II. Radiation of the renal fossa (4,000 rad) and chemotherapy with actinomycin D and vincristine were administered after surgery. Six years after surgery the patient is disease free. A review of published cases of adult Wilms tumor is presented, and a plea is made for triple therapy.     

Adult wilms tumor in the renal pelvis: case report with review of the literature

An 84-year-old man was referred to our hospital with gross hematuria. Abdominal computed tomography demonstrated a well-circumscribed enhanced mass in the right pelviureteral junction. Retroperitoneoscopic nephroureterectomy was performed because of a clinical diagnosis of renal pelvic carcinoma. Pathologic examination led to a final diagnosis of an adult Wilms tumor arising from the renal pelvis. This is the first report of a Wilms tumor in the renal pelvis of an adult patient.     

Wilms' tumor in adults

A rare case of metastatic nephroblastoma on the right side in a 28-year-old female patient is presented. Complete remission was achieved by inductive chemotherapy with vincristine, Adriamycin, actinomycin D and cyclophosphamide and subsequent radical nephrectomy. There was no evidence of disease 24 months after the initial diagnosis had been made. The problems specific to the diagnosis and treatment of adult Wilms tumor are discussed. A chemotherapy regimen known to be successful in childhood Wilms tumor was shown to be equally effective in our adult patient.     

Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study

An analysis of information on 2,961 patients with Wilms tumor entered in the National Wilms Tumor Study up to July 1983 revealed 13 tumors occurring in horseshoe kidneys. The correct preoperative diagnosis was made in 6 of the 13 patients. At presentation 5 patients had stage 1, 3 stage 2 and 5 stage 3 disease. Major preoperative or intraoperative tumor spillage occurred in 3 patients. The over-all survival rate was 85 per cent after a mean followup of 45 months. The surgical, pathological and therapeutic aspects of Wilms tumor in horseshoe kidneys were analyzed carefully. The frequency of horseshoe kidneys in patients with Wilms tumor registered with the National Wilms Tumor Study was determined and was used in conjunction with population data to estimate the relative risk of Wilms tumor occurrence in children with horseshoe kidneys.     

Adult Wilms tumor: a case of prolonged survival achieved by multimodal treatment

Wilms tumor is one of the most common malignant neoplasms in children. In the last 2 decades the survival rate of children with this disease has dramatically increased. However, in adults, the guideline for its management is less clear. We herein report the prolonged survival of a patient with advanced adult Wilms tumor after treatment with chemotherapy, irradiation and operations. Adult Wilms tumor should be treated aggressively with a well-timed combination of surgery, chemotherapy and radiotherapy (i.e., multimodal treatment), even if the stage of the disease was advanced. Thereby, we suspect that the prognosis of adult Wilms tumor would be markedly improved.     

Adult Wilms tumor. Treatment with surgery, radiotherapy, and chemotherapy

A twenty-nine-year-old patient with a Wilms tumor on the right side was treated with surgery, radiotherapy, and chemotherapy. Since no study of treatment of adult Wilms tumor is yet available, the combined therapy was instituted in accordance with the one existing for children. The results of the 5 adult cases previously reported which were treated with triple therapy, and the relevant literature, are reviewed.     

Metachronous bilateral Wilms tumor. Report of longest-known survivor and guidelines for conservative management

In a one-year-old black male patient a classic Wilms tumor of the left kidney was treated with left nephrectomy, chemotherapy, and irradiation to the entire abdomen. Two years later a mature Wilms tumor, consisting predominantly of rhabdomyomatous elements, was discovered in the right kidney. Although a second course of chemotherapy was given and 2,000 rad were delivered to the right flank, the mass continued to enlarge with progressive deterioration of renal function. A nephron-sparing procedure was performed, in which a 3,400-g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms tumor with a malignant anaplastic sarcoma arising in the central portion. Currently, he is disease-free with normal renal function more than twenty years after diagnosis of the metachronous bilateral Wilms tumor. We believe he is the longest known surviving metachronous Wilms tumor patient and emphasizes the importance of conservative, meticulous surgery in the management of both unilateral and bilateral Wilms tumors. The role of multimodal therapy (chemotherapy, irradiation, and surgery) as well as the current guidelines for the management of bilateral Wilms tumors as proposed by the National Wilms Tumor Study are reviewed.