Management of Wilms tumor: current standard of care

Wilms tumor is the most common renal malignancy in children. In the 1930s, overall survival for children with Wilms tumor was approximately 30%. Use of multidisciplinary therapy, guided by results from multi-institutional, randomized trials, has substantially improved overall survival to about 90%. Management of Wilms tumor differs substantially between Europe and the US. In Europe, the International Society of Pediatric Oncology protocols call for management of patients with presumptive Wilms tumor with neoadjuvant chemotherapy followed by nephrectomy and further chemotherapy. In the US, protocols developed by the National Wilms Tumor Study Group advise primary nephrectomy followed by a chemotherapy regimen tailored to the pathologic tumor stage. Despite these disparate strategies, overall survival is similar in patients managed according to European and US protocols. Patients with Wilms tumor now have excellent survival. Therefore, current goals aim to reduce the morbidity associated with therapy. Important complications of treatment for Wilms tumor include cardiomyopathy, renal failure, and increased risk of a secondary malignancy. Currently, the role of laparoscopic surgery in management of Wilms tumor remains extremely limited.     

Treatment strategy for children with Wilms tumor

Wilms tumor has an excellent prognosis according to the findings of the National Wilms Tumor Study (NWTS). The NWTS has been aiming to reduce the intensity of treatment for low-risk groups. Pathologic assessment or statistical analysis has identified the appropriate staging system suited to this treatment regimen. The Japanese Wilms Tumor Study (JWiTS) has been following the NWTS-5 regimen to increase survival rates and tumor-free survival rates from 1996. This study aims to establish a central review system of pathology and to study the presence of WT1 mutation in tumor samples. In addition, JWiTS focuses on developing an online database that can be shared over the Internet. However, the prognosis in patients with malignant rhabdoid tumor of the kidney (MRTK) is still poor. JWiTS has started to consider a new regimen for MRTK.     

Renal leiomyoma in a child: a rare renal tumor

Renal leiomyoma is a rare benign smooth muscle tumor of the kidney. Most cases are reported in adults, with very few cases described in children. We report a case of right renal leiomyoma in a 6-year-old boy which was suspected of being a Wilms tumor. Contrast-enhanced computed tomography could not differentiate leiomyoma in the kidney, and the mass was diagnosed as a Wilms tumor. Right radical nephrectomy was performed. Diagnosis of renal leiomyoma could only be achieved after histopathologic examination and immunohistochemistry. We recommend that total nephrectomy has to be done in most cases of pediatric renal tumors to avoid the risk of malignancy. There may be a role for nephron sparing surgery, provided the tumor is small, and one has a strong suspicion for the lesion being benign.     

Pediatric malignancies: neuroblastoma, Wilm's tumor, hepatoblastoma, rhabdomyosarcoma, and sacroccygeal teratoma

Common pediatric malignancies are reviewed: neuroblastoma, Wilms tumor, hepatoblastoma, rhabdomyosarcoma, and sacrococcygeal teratoma. Elements of presentation, diagnosis, staging, treatment, and longterm prognosis are discussed, with particular attention to surgical management.     

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.     

Pulmonary metastasectomy in pediatric patients

This article describes the historical development of pediatric pulmonary metastasectomy but demonstrates that progress has been slow in understanding its proper applications. Because many pediatric metastatic tumors are rare, surgeons have grouped together patients of different histologies for the generation and analysis of case series. By examining tumor types individually, however, it is seen that certain histologies (adrenocortical carcinoma, alveolar soft part sarcoma, osteosarcoma) mandate surgical metastasectomy for patient survival. Other pediatric tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive, and the application of metastasectomy is controversial. In the case of still other types of tumor (neuroblastoma, differentiated thyroid cancer, rhabdomyosarcoma), metastasectomy is seldom performed except in highly unusual situations. Techniques for minimally invasive biopsy and for muscle-sparing thoracotomy are described for pediatric patients.     

Extrarenal Wilms tumor in children with unfavorable histology: a case report

Extrarenal Wilms tumor is extremely rare, and only 25 cases in children have been reported to date in Japan. A 2-year-old girl presented with a large left lower quadrant abdominal mass. Abdominal computed tomography revealed a retroperitoneal tumor located below the left kidney. At laparotomy, the tumor was encapsulated without evidence of metastasis to other abdominal organs. Pathologic diagnosis of the tumor was extrarenal Wilms tumor with diffuse anaplasia. After complete tumor resection, chemotherapy was administered according to the treatment protocol (Regimen I) of the Japan Wilms Tumor Study Group. Cyclophosphamide and etoposide were administered in combination with vincristine and doxorubicin. Two years after treatment, the patient has had no evidence of recurrence.     

Renal primitive neuroectodermal tumor in childhood with intracardiac extension

Primary primitive neuroectodermal tumors of the kidney are exceptionally rare and usually affect children and young adults. We report the first pediatric case of renal primitive neuroectodermal tumor presenting with tumor extension along the inferior vena cava to the right ventricle. This case highlights that when considering a renal tumor with significant intravascular extension in the pediatric age group, although the most likely diagnosis remains Wilms tumor, other rare entities may also demonstrate similar clinical and imaging features.     

Polypoid renal pelvic lesions in children

A case is presented of gross hematuria and a large polypoid renal pelvic mass in a 15-month-old boy. Operation revealed a Wilms tumor with limited parenchymal involvement and rupture into the collecting system. This rare presentation of nephroblastoma and its differentiation from other rare benign and malignant renal pelvic lesions occurring in the pediatric patient are discussed. The advantages and disadvantages of radical and conservative surgical approaches to these rare lesions are reviewed. Preoperative cystoscopy and retrograde pyelography are mandatory in all patients with renal tumors presenting with gross hematuria, regardless of age. Radical nephrectomy with total ureterectomy is recommended for Wilms tumor with extension into the collecting system.     

Intussusception following resection of Wilms tumor

Postoperative intussusception is a documented complication of pediatric surgical and pediatric urologic abdominal operations. In contrast to "primary" intussusception's triad of crampy abdominal pain, palpable abdominal mass, and "currant jelly" stools, postoperative intussusception is generally characterized by abdominal pain and vomiting. An abdominal mass is not usually palpable, and few children have bloody stools. Proper diagnosis and treatment may be delayed because of similar abdominal symptoms in children who may be receiving radiation and chemotherapy, or with prolonged ileus. Two children operated on for Wilms tumor demonstrate the need for awareness of this potential problem in the postoperative patient.     

Wilms tumor presenting with abdominal pain: A special subgroup of patients

Background: Although significant progress has been made in the management of children with Wilms tumor, two major controversies still exist: the extent of radiographic evaluation necessary before surgery and the role of preoperative chemotherapy. This study sought to determine whether patients with Wilms tumor who presented with abdominal pain defined a special subset of patients who might require a more extensive preoperative work-up and neoadjuvant chemotherapy. Methods: From 1970 to 1995, 250 children were treated for Wilms tumor at a single pediatric institution. A retrospective chart review determined presenting signs and symptoms for each patient. Results: Thirty-four (14%) patients (mean age 5.5 years) sought medical attention with a chief complaint of abdominal pain. The stage distribution for these patients tended to be higher and was significantly different (P<.001, x²-analysis) from those presenting without pain. Four (14%) of 29 patients with tumor available for analysis were found to have anaplastic histology. Conclusion: These data suggest that patients with Wilms tumor who present with abdominal pain represent a special subgroup that tends to be older and has an increased incidence of tumor rupture, anaplasia, and higher stage. These patients may benefit from a more extensive preoperative evaluation and consideration of neoadjuvant chemotherapy.Presented at the 50th Annual Cancer Symposium of The Society of Surgical Oncology, Chicago, Illinois, March 20–23, 1997.     

A case of renal cell carcinoma after successful treatment of Wilms tumor

This case report documents the eighth reported case of renal cell carcinoma (RCC) occurring after treatment of Wilms tumor. Although secondary malignancies after treatment of Wilms tumors are not infrequent, RCC as the second malignancy is rare. We discuss a 17-year-old girl whose RCC was diagnosed 12.5 years after diagnosis of a Wilms tumor. In addition, we review the literature on the subject. Renal cell carcinoma has been proposed as a consequent of chemoradiation; however, a genetic susceptibility must be considered. Because it is routine to assess the functional status of the remaining solitary kidney by annual ultrasonography, we recommend assessing for the presence of secondary renal malignancies and perhaps continuing routine ultrasounds long-term.     

巨大成人型肾母细胞瘤3例报告并文献复习

目的探讨巨大型成人肾母细胞瘤的诊断和治疗方法,提高该病的诊断率与治愈率。方法回顾性分析南京医科大学第一附属医院2007至2008年间收治的3例巨大成人肾母细胞瘤的临床表现、分期、治疗及预后。结果 3例患者按照美国国家肾母细胞瘤研究组分期：Ⅱ期2例,Ⅲ期1例。3例均行根治性肾切除术,术后辅以放疗和化疗。所有病例均获得随访,随访2～3年,3例均生存。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。     

Preoperative chemotherapy for Wilms tumor

Preoperative chemotherapy was administered to 19 children with Wilms tumor judged clinically to be unresectable at M. D. Anderson Hospital between January 1, 1962, and September 1, 1980. After 2 to 4 doses of vincristine, marked reduction in tumor size occurred in 16 patients. After chemotherapy 16 tumors could be resected completely, another required irradiation to reduce the tumor, and only 2 tumors could not be excised. Pathologically the most dramatic changes occurred in the undifferentiated interstitial stroma, followed next by changes in the nodular blastema. Differentiated elements were apparently not affected. No serious complications were attributed to the preoperative drug treatment. This experience suggests that in selected instances preoperative chemotherapy can affectively facilitate the therapy of Wilms tumor.     

Multimodal treatment of advanced adult Wilms tumor

We report 2 cases of advanced adult Wilms tumor that were treated with surgery, radiation and chemotherapy. The first patient had relapse of a Wilms tumor in the liver 2 years after nephrectomy. Combination chemotherapy, consisting of actinomycin D and vincristine, radiation therapy and final resection of the liver metastasis were successful and the patient has been free of disease for 4 years. The second patient had undergone transcatheter embolization of the renal artery elsewhere with the tentative diagnosis of an inoperable renal cell carcinoma metastatic to both lungs. A left renal tumor, weighing 4,500 gm., and a tumor thrombus in the vena cava extending to the right atrium were removed, and histologically diagnosed as a Wilms tumor. Subsequent chemotherapy and radiotherapy resulted in complete disappearance of the lung metastases. We conclude that multimodal treatment, namely a well timed combination of surgery, chemotherapy and radiotherapy, could potentially eradicate the disease even at an advanced stage.     

Cavectomy for the treatment of Wilms tumor with vascular extension

PURPOSE: Vascular extension to the vena cava occurs in 4% of Wilms tumor cases and can reach the right atrium in up to 1%. When this happens the thrombus is usually not adherent to the vessel wall, and there is blood flow around it. Preoperative chemotherapy can cause thrombus regression and even resolution. If the thrombus persists after chemotherapy, surgery will be a challenge. On the other hand, if the thrombus invades the vessel wall, its removal may not be feasible. In this situation cavectomy is a good surgical strategy because it provides complete resection. The prerequisite for cavectomy is the absence of blood flow in the vena cava on preoperative Doppler ultrasonography. We report 3 cases of Wilms tumor with vena caval invasion in which cavectomy was performed, and discuss the principles, indications and operative technique. MATERIALS AND METHODS: A total of 171 patients with Wilms tumor were treated at our institution between 1984 and 2004. Of these patients 6 with intravascular extension of thrombus within the right atrium were treated with extracorporeal circulation, cardiac arrest and profound hypothermia, and 3 were treated with cavectomy. RESULTS: There were no instances of surgical complications or postoperative renal failure in our patients who underwent cavectomy. All remain well and free of disease. CONCLUSIONS: Cavectomy is a safe procedure for treating pediatric patients with Wilms tumor when there is extension and invasion of the vena cava wall without blood flow.     

Acute pancreatitis induced by ifosfamide therapy

Ifosfamide used in the treatment of pediatric solid tumors is known to have serious adverse effects, including acute pancreatitis, a rare complication of therapy. This report describes a young girl who developed acute pancreatitis while being treated for recurrent Wilms tumor with the ifosfamide, carboplatin, and etoposide regimen. She recovered completely and without sequelae from ifosfamide-induced pancreatitis soon after the drug was stopped. Abdominal pain in patients on anticancer treatment is a common occurrence, but it is rarely investigated. Patients who receive ifosfamide as part of a chemotherapy regimen should be carefully monitored, and symptoms or signs suggestive of acute pancreatitis should be promptly investigated. Withholding the drug usually leads to complete recovery.     

Nephroblastoma in adults

Nephroblastoma, or Wilms tumor, is the most common renal neoplasm in children and accounts for approximately a fifth of all malignant growths in this age group. However, the incidence of Wilms tumor in adults is much less common, with less than 200 cases having been reported in the literature. In addition, since there are at least 53 synonyms for this tumor an adequate computer search for articles relating to this subject is difficult. Because of the scarcity of this disease in adults definite treatment modalities have not been accepted thoroughly until recently. The rationale for the present treatment modalities is based largely on the National Wilms Tumor Study groups 1 to 3. We present radiologic and histologic findings of this tumor in 2 additional cases. The current treatment modalities, consisting of chemotherapy with or without adjunctive radiotherapy, are discussed.     

A case report of Drash syndrome in a 46,XX female.

Progressive renal insufficiency, male pseudohermaphrotidism, and Wilms tumor are known as the triad of Drash syndrome. The renal involvement has been reported in association with Wilms tumor with or without pseudohermaphroditism. The nephropathy progresses rapidly, is unresponsive to steroids, and eventually requires dialysis. The case we report is a 46,XX phenotypic female child who had diffuse mesangial sclerosis (DMS) and developed Wilms tumor 3 years after initiating dialysis for end-stage renal disease (ESRD). Any child presenting with DMS should be considered at risk for development of Wilms tumor and should be investigated for the presence of XY gonadal dysgenesis. Once ESRD develops in such a child, prophylactic nephrectomy and possibly gonadectomy should be undertaken.