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Objectives: Main objective: to perform a descriptive study of patients with nephroblastoma, diagnostic tests, medical and surgical treatment. Secondary objective: to evaluate the rate of relapse and 5-year survival and risk factors for relapse and death., Materials and Methods: Retrospective study of patients with nephroblastoma treated according to the protocol of the SIOP-2001. Demographic variables, comorbidities and associated syndromes were collected. Other data were tumor location, size, extent and stage. The relapse rate and the development of other secondary tumors as well as the long-term survival were also studied., Results: We collected 33 patients with nephroblastoma. A biopsy was performed in 7 patients (21.2%). The Kaplan-Meir curve for event-free survival (tumor recurrence) was 84% with a 95% CI = [0.73-0.98] and the Kaplan-Meier overall survival curve was 0.93 95% CI [0.85-1]. Recurrence occurred in all patients before the first year., Conclusions: Nephroblastoma is a tumor with a favorable prognosis. The unfavorable histology as well as advanced stages are factors of a poor prognosis. The follow-up must be exhaustive during the first year after the diagnosis.

Introduction: Minimally invasive surgery (CMI) for the treatment of malignant tumors in children begins to have a role for selected cases and reaches similar results than open surgery. We show our first two cases of Wilms tumor treated by laparoscopy describing patients and technique., Material and Methods: Three-year-old girl with macroscopic hematuria is diagnosed of 8 cm mass in the left kidney suggesting Wilms tumor. After 4 weeks of chemotherapy she went under laparoscopic nephroureterectomy. The histological result was Wilms tumor. Chemotherapy was completed seven more months. Five-year-old patient with abdominal pain is diagnosed of renal right mass suggesting Wilms tumor. After 4 weeks of chemotherapy the laparoscopic nephroureterectomy was performed. The histological result was Wilms tumor. Treatment was completes with postoperatory chemotherapy., Results: After 1 year follow-up both patients have clinical and radiological absence of disease., Conclusion: The CMI in selected cases of Wilms tumor fulfills successfully the aims of the surgical treatment.

Aim: To show our experience in the surgical management of bilateral Wilms' tumor., Methods: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up., Results: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV. 100% of patients with metachronous Wilms' tumor was stage I-II. All the tumors had favourable histology. Surgical complications were: 4 bowel pseudobstructions, 2 ureteropielic fistulae and 1 urinary cyst. 15 patients are alive (83%) with a mean follow-up of 12 years. 3 of these patients had a renal trasplant with a good evolution. One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease., Conclusions: Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%). Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure.

Background: The resection of tumor thrombus of the inferior vena cava (IVC) and right atrium (RA) increases the survival rate of patients with renal/adrenal cancer., Objective: To evaluate the surgical procedure in cases of IVC and RA in the treatment of renal and adrenal tumors., Methods: Fourteen patients undergoing surgical intervention (during the period) between January 1997 and June 2007, for resection of IVC and/or RA thrombus due to renal or adrenal tumors, were retrospectively evaluated. The patients (64.2% male) presented with Wilms' tumor, clear cell carcinoma and adrenal adenocarcinoma, and had mean age of 4.5, 60.5 and 2.5 years, respectively. Epidemiological characteristics and intra- and postoperative parameters were evaluated., Results: Suprahepatic IVC tumor thrombus were observed in all the patients, and in 62.4% of them the thrombus invaded the RA. Thrombectomy was performed with extracorporeal circulation with deep hypothermia and total circulatory arrest in 85.7%, with mild hypothermia in the remaining cases. The inferior vena cava was ligated in 7.1% of the cases, and reconstruction with suture was performed in 92.9% of the patients. The duration of orotracheal intubation and length of hospital stay were different, according to the tumor type. Two deaths, due to intraoperative cardiorespiratory arrest, were seen among patients with adrenal adenocarcinoma., Conclusion: IVC and RA tumor thrombi are more frequent in patients with Wilms' tumor. More postoperative complications are seen in patients with adrenal adenocarcinoma, and the postoperative prognosis is better for patients with Wilms' tumor.

Introduction: Wilms tumor surgery with intracaval-atrial extension is a challenge. Modern imaging techniques, precise preoperative thrombus location, and multidisciplinary surgical approach is mandatory. We aim to evaluate the outcome of our patients., Patients and Methods: Between 1992 and 2005, 52 patients with nephroblastomas underwent surgery in our institution. Nine of them had renal-caval (RC) or cavo-atrial(CA) thrombus extension. Four patients presenting short RC intravascular extension were excluded., Results: All cases were treated with pre-postoperative chemotherapy SIOP protocols. The level of the extension was retrohepatic in 2 cases, atrial in 3 patients and it even reached the ventricle in 2 of them. A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumor could be removed under cardiopulmonary by-pass in 3 cases (CPBP). One pulmonary tamponade due to thrombus migration (CAV) occurred and was solved by CPBP. Three cases were stage III, one stage IV and 1 stage II. Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy. In case of CA extension, CPBP and right liver displacement to gain access to retro-hepatic cava are mandatory in order to reduce surgical complications.

Renal tumors can be associated with a thrombus that affects the renal vein and even the inferior vena cava. Radical surgery may require a 2-phase approach involving different anesthetic techniques: an abdominal approach for removal of the kidney and a thoracic approach for extraction of the thrombus, with extracorporeal circulation and in some cases cessation of blood circulation. We present 2 cases in which nephrectomy and thrombectomy were carried out with the support of extracorporeal circulation. The thrombus was in the renal vein and the inferior vena cava, extending to the outlet to the right atrium in both cases. In 1 case a portion reached the bifurcation of the pulmonary artery. The operation was performed under hypothermia to reduce circulation and did not require aortic clamping, cardioplegia, or cessation of blood flow.

Unlabelled: Tumors diagnosed during the first month of life are infrequent: 0.5 to 2% of all childhood neoplasms. This is an interesting group of tumors because their type, relative incidence, natural history and response to treatment differ from those seen in older children., Aim: To contribute the experience of our institution in congenital tumors the last 13 years., Material and Methods: The records of all neonates (< 31 days old) diagnosed with solid tumors since January 1990 to December 2002 have been retrospectively reviewed., Results: Twenty-seven neonates have been diagnosed with tumors in the last 13 years. Thirteen patients (48%) were prenatally diagnosed. Nine babies were diagnosed at the initial neonatal exam (40% of those diagnosedd after birth). Neuroblastoma was the commonest tumor (10 cases, 37%), of which 4 were stage I, 4 stage IV-S and 2 stage III. There were 8 teratomas (3 sacrocoxigeal, 1 retroperitoneal, 1 in the CNS, 1 orbitary and two oronasal), two hepatic tumors (1 hepatoblastoma, 1 hemangioendothelioma, two CNS tumors, two giant nevus (one on a hamartoma), and one each Wilms tumor, infantile fibrosarcoma and myofibroblastic tumor. Treatment was surgical resection alone in 17 cases (68%) and surgery + chemotherapy in 8 (32%) (5 neuroblastomas, one CNS tumor, one Wilms tumor and one presacral teratoma who developed a yolk sac tumor); 3 patients died (11%): one at surgery, one of tumoural airway obstruction at birth and one with craniopharyngioma. Among the 14 tumors that were initially not malignant, two can be locally agressive, one was an immature teratoma, the giant nevus with hamartoma developed in situ melanoma, the other nevus had meningeal melanosis with hydrocephalus, and one mature presacral teratoma developed a yolk sac tumor., Conclusions: Diagnosis of congenital tumors is performed earlier in recent years due to the wide use of prenatal ultrasound. Their natural history is more benign than in other age groups, except for CNS tumors and very large or obstructing tumors. The histological patern is not determinant of the outcome. Complete surgical excision is the treatment of choice, most cases not need adjuvant chemotherapy. We ought to pass this message on to our colleagues in prenatal diagnosis, so parents get reliable information.

Wilms' tumor is a malignant embryonic renal neoplasm that is exceptional in adults. There are not clinical data or radiographic investigations that can distinguish it from renal cell carcinoma. So the diagnostic is based in the pathological evaluation. It may be cystic and must be consider in the differential diagnosis of cystic lesions of the kidney. The prognosis of Wilms' tumor in adults is worse than in children because of the high recurrence, the lower response rate to chemotherapy regimens and the advanced stage at the time of clinical presentation, like an asymptomatic abdominal mass in 75% of the cases. We report a new case of nephroblastoma in a 29 years old woman presenting like a renal colic, with a cystic configuration by abdominal ultrasound initially, that changed into a solid renal mass later. There is not a definitive treatment protocol currently but some authors suggest a combination chemotherapy with carboplatin and etoposide because it is very effective in recurrent or refractory adult Wilms' tumor. Our patient remains asymptomatic and without evidence of recurrence 18 months after the surgery.

Objectives: Multilocular cystic nephroma is a rare benign entity grouped among the cystic non genetic diseases, which usually presents with a clinical picture and radiological features indistinguishable from malignant neoplasias, making impossible to rule out malignancy preoperatively., Methods: We report two cases of multilocular cystic nephroma which were treated with different surgical attitudes despite their clinical and radiological similarities, because on the second case intraoperative pathologic study of the specimen was performed., Results: The diagnosis of multilocular cystic nephroma was confirmed in both cases, with the difference that radical nephrectomy was performed in the first case whereas the intraoperative study of the tumor in the second allowed to perform tumorectomy with renal parenchyma preservation., Conclusions: We consider that in the presence of a multiloculated renal mass of complex appearance and benign clinical behavior, the intraoperative study of the tumor will avoid performance of radical surgery.

Nephroblastomatosis is a complex of pathological conditions defined by the persistence of nephrogenic elements after the conclusion of nephrogenesis (beyond week 36 of gestation) that conserve the capacity to evolve into nephroblastoma. Luckily, this malignant transformation is exceptional (< 1%(1)), which is why the disease is considered to have a good prognosis. The nephrogenic remains are frequently associated with beckwith-Wiedemann syndrome, hemihypertrophy and aniridia, in which case there is a greater risk of the development of Wilms tumor. Consequently, these children must be followed-up closely. The difficulty lay in knowing when to act, which children should be treated, and what treatment is appropriate because what little has been published in the literature is debatable. We report the cases of two patients, one with unilateral nephroblastomatosis and the other bilateral, who required surgical treatment after not responding to chemotherapy; and we expose our therapeutic algorithm and follow-up strategy.

Objective: Wilms' tumor or nephroblastoma is the most common renal tumor of childhood. The incidence in adults is rare, estimated at about 1% of all cases and approximately 200 cases have been described in the world literature. We report a new case of adult Wilms' tumor and realize a short review., Methods/results: We describe a case of Wilms' tumor in a young male, aged 15 years, with associate genitourinary abnormalities. The patient underwent radical nepherctomy with postoperative chemotherapy., Conclusions: The Wilms' tumor in the adult population is exceptional, and the definitive diagnostic is based in the findings of the pathological analysis. The application of therapeutic protocolos for children to adults offers an smaller percentage of cure and poorer prognostic. Therapheutic protocolos more aggressive are necessary for increase the worse prognostic in the adult, but because of the rarity is difficult to define the most effective form of treatment.

Unilateral Wilms' tumor has been treated according to 9301 SIOP protocol, with good results. The new pre protocol that SIOP is developing has a high inclusion rate. From 1993 to 1999, 11 patients with unilateral Wilms' tumor were treated in our center; in 6 cases preop chemotherapy was done, in the other 5 cases pre and postoperative chemotherapy were used. Nine of the 11 patients could be included in the pre protocol this was due to a thrombosis of the vena cava in one case, and in the other the middle renal in area was widely affected. Wilms' tumor has a good prognosis with the actual protocol, SIOP. New pre protocol could give a better quality of life due to the amount of functional renal parenchyma, without decreasing the actual high cure rate.

Introduction: The 30-40% of the oncologic patients have pulmonary metastases. Lung can be the only organ affected. In selected patients, exeresis of the pulmonary nodules can mean their healing., Material and Methods: Between 1982-1997, twenty two patients presented metastases, 13 could be operated and 16 thoracotomies were done. There were 53% boys and 47% girls whose ages ranged from 3 to 15 years. We have considered: pulmonary tumour location, disease free interval, number of metastases, surgical technique and incomplete pulmonary tumour resection., Results: Primary tumours were: Wilms tumours 23%, bone tumours 67% (Ewing and osteosarcoma). Disease free interval was < 2 years in 8 patients (61%) and > 2 years in 39%. X-Ray and CT were performed in every case and 66% presented a solitary nodule. Surgical techniques were: metastasectomy in two cases (12%), wedge resection in 8 (50%) and lobectomy in six cases (38%). We made thoracoscopy in two patients. There weren't postoperatory mortality but the patients with tumorectomy had an incomplete surgical resection. The overall survival is 54 percent and the 5 years survival is 23 percent (3 patients)., Conclusions: The patients with a DFI < 2 years have a survival of 25% compared with 100% for patients who have a DFI > 2 years. The pulmonary resection in selected patients can offer better survival. We can use the thoracoscopy in same selected patients.

Objective: To report on a case of cystic partially differentiated nephroblastoma in an adult female patient that was treated by partial nephrectomy., Methods/results: A 21-year-old female patient with multicystic lesions was submitted to partial nephrectomy involving the lower pole of the right kidney. The presence of embryonal blastema in the septae that separated the cysts was diagnostic of cystic partially differentiated nephroblastoma., Conclusions: Cystic partially differentiated nephroblastoma can be considered to be a well differentiated variant of nephroblastoma (Wilms' tumor). Due to its benign nature, treatment is by conservative renal surgery, if technically possible.

Objective: To report a case of adult Wilms' tumor, a kidney tumor that is more common in children and has a scanty incidence in adults., Methods/results: A case of Wilms' tumor in a young male, aged 16 years, is described. The patient underwent partial surgical resection initially and nephrectomy due to tumor recurrence, with postoperative chemotherapy and radiotherapy., Conclusions: Although approximately 200 cases of adult Wilms' tumor have been reported in the literature, few meet currently accepted diagnostic criteria. The diagnosis of adult Wilms' tumor based on the findings of currently available diagnostic methods is only presumptive until the surgical specimen is analyzed. Most cases are treated as renal tumor and it is the findings of the pathological analysis that defines its nature and the need for chemotherapy and/or radiotherapy, as well as long-term follow-up. The application of therapeutic protocols for children to adults has not obtained the same percentage of cure or prognosis, although a large multicenter study is warranted to confirm the foregoing.

Objective: We comment and update the surgical treatment for pulmonary metastases (PM) within a multidisciplinary approach for paediatric cancer., Material and Methods: We analyse patients with PM who have been operated between 1976-1996. Scientific literature published in the last 25 years (Cancerlit and Medline) was reviewed., Results: PM from 13 patients were removed. Seven were males and 6 females with a mean age 5 4/12 years (range: 11 months- 12 3/12 years). Diagnoses were Wilms' tumour (7), osteosarcoma (3), Ewing sarcoma (1), rabdomiosarcoma (1), Yolk sac tumour (1). PM were unilateral in 7 cases and bilateral in six cases. PM appeared synchronically in four patients and metacronically in nine cases (3 of these after chemotherapy). All patients received chemotherapy and four of them local radiotherapy. Surgery consisted on radical segmentectomy and only one patient needed lobectomy due to a local relapse. Nowadays five patients (38%) are in complete remission with a mean follow-up from surgery of 11 11/12 years (range: 6 3/12-20 years)., Conclusions: Metastasectomy is an important surgical technique in global treatment of children with PM and for a selected group of patients it can offer the only opportunity for curation.

Objective: To describe an additional case of multilocular cystic nephroma, with special reference to the sonographic features of a renal cystic lesion., Methods: The clinical and radiological features of a kidney tumor in a 61-year-old female patient were retrospectively analyzed., Results: The preoperative diagnosis was that of a complex multicystic renal mass suggestive of a renal carcinoma. Radical nephrectomy was performed; histopathological analysis of the surgical specimen disclosed a multilocular cystic nephroma. An ultrasound scan 18 months earlier had demonstrated a simple renal cyst in the same site., Conclusions: Multilocular cystic nephroma is a tumor that is difficult to diagnose preoperatively. Making an accurate differential diagnosis is of interest, since it will permit conservative treatment. The case described herein supports the hypothesis with regard to the pathogenesis of this lesion, since it had previously showed sonographic features of a simple renal cyst.

Objective: To report on a patient with Wilms' tumor treated by partial nephrectomy with 31 years survival., Methods: Herein we describe a 33-year-old patient who had undergone surgery for a right renal tumor at age 2 years and 10 months. A partial nephrectomy was performed because the patient had left ureterohydronephrosis. Histological analysis of the surgical specimen disclosed a nephroblastoma or Wilms' tumor., Results/conclusions: The progressive deterioration of the left urinary tract, despite attempts to correct this condition, warranted its complete suppression. The patient has remained symptom-free and leads an active social and working life 31 years after the first operation, which makes this an exceptional case in the world literature.

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms.

We review a case of multicystic right dysplasia containing nodular renal blastema in a 3-year-old girl with left Wilms tumor. In relation to this finding the management of the asymptomatic multicystic dysplastic kidney in discussed.

Since Gross established the basic rules for nephrectomy in Wilms' tumour (WT) in 1953, the management of nephroblastoma has been more straightforward; however, some cases with intravascular involvement, currently detected by ultrasound (US), may represent a daunting challenge for the surgeon. Inferior vena cava with tumour thrombus induced by WT can be asymptomatic, and if undetected, contribute to poorer prognosis for two main reasons: possible neoplastic cells inside the thrombus and higher morbidity risk of surgery. From 1979 to 1993, 81 WT were studied by routine US. Intracaval thrombosis was diagnosed in four (5%), in one of which the thrombus extended to the right atrium. In our experience, the surgical strategy in each of the four cases (100% survival), depended on the length of the thrombus and whether or not it infiltrated the vena cava wall. If the thrombus can be easily removed: complete resection. However in cases of atrial thrombus and more particularly if the thrombus involves the intima, we suggest the thrombus not be touched since the problem may be solved by preoperative and postoperative chemotherapy. Thus, the favourable prognosis would be maintained and superfluous risky surgery avoided.

We report a case of multilocular cystic nephroma in a 45-year-old female patient who had been referred for urological evaluation on detecting a mass in the left lumbar region. Patient work up included CT evaluation, which proved to be the most useful in making the diagnosis. The literature is reviewed with special reference to the nature of this lesion and the existing controversy on whether it is an embryonary tumor or a congenital malformation.

Wilms tumour is an infantile neoplasia with low incidence in adults. Worldwide literature describes 260 cases but only 50 meet the diagnostic criteria described by Kilton. This paper presents one case diagnosed in our service and includes a review of the literature.

We report an anesthetic technique to control hypertension in a Wilms' tumor in stage I in a 25-month-old child weighing 10 kg. He was treated with actinomycin D and vincristine during 4 weeks before surgical resection, developing inadequate ADH secretion due to vincristine. General anesthesia was used, with atropine, thiopental and atracurium as muscular relaxant. Systemic intraoperative anesthesia with narcotics was not given. A catheter was placed in the epidural space, with entrance through L3-L4 and its extreme in L1. Radiological control of the distal end of the catheter was carried out with iohexol before the administration of two 0.3% bupivacaine doses without adrenaline of 3 ml each, and intraoperative normal blood pressure was achieved. It was concluded that combined superficial general anesthesia techniques, associated with continuous epidural blockade, can be useful to control hypertension in Wilms' tumor.

We report on an adult patient who underwent surgery for Wilms' tumor or nephroblastoma with a prolonged survival. At 13 years post-operatively, the patient is alive and disease-free, a father of two children, and has an active working and social life. Recent blood analyses, chest X-ray and abdominal ultrasound scan were unremarkable. We reviewed the cases of Wilms' tumor reported in the world literature highlighting the survivorship reported by some of the authors. To our knowledge, of the adult patients reported with this malignant tumor type, ours has the longest survival.

Two new cases of cystic partially differentiated nephroblastoma, which were treated by nephrectomy, are reported. Their gross appearance is undistinguishable from a multilocular cyst, with the only difference being the presence of buds of blastema within the cystic septa. While cystic partially differentiated nephroblastoma as well as multilocular cyst have never been reported to be associated with local or metastatic recurrence and therefore their treatment consists of simple nephrectomy, the presence of blastema suggests a differentiates stage of Wilms' tumor, thus demanding a close surveillance of the patient.

A female child, 5 years 10/12 old, is presented with a nephroblastoma inserted on a horse-shoe kidney. She is in a satisfactory state 14 months after surgery. The authors emphasize the favorable outcome with a treatment of combined surgery, radiotherapy and vincristine.

This report deals with three patients admitted with acute abdomen and hypovolemic shock, but at the operation, solid tumors traumatically broken were found. this finding is infrequent and is a serious problem for the surgeon who must solve the emergency and carry out the adequate surgical treatment with oncologic criterion.

Four patients diagnosed of having a tumor of Bolande and treated at our institution in the past ten years are presented. In two of them, early diagnosis was aided by prenatal echographic techniques; the other two diagnoses were suspected ar three and ten days of age because of an abdominal mass. The four of them underwent a complete study which included: a plain abdominal film, an ultrasound scan and an intravenous urogram; the last three patients were also surveyed by a CT scan of the abdomen. Surgery was undertaken early on all the patients, the time of operation being in every instance within the first month of life. A laparotomy was performed and, once the contralateral kidney had been carefully examined, a nephroureterectomy was implemented, having been able to preserve in all the cases the suprarenal gland in view of the benign nature of the tumor. A histologic study confirmed the diagnosis and revealed the complete delimitation of the tumor by the renal capsule. The authors underscore the importance of this tumor's suspicion in the face of any solid renal mass detected by prenatal echography or in the neonatal period, since early diagnosis and treatment are imperative in order to prevent the ensuing complications in the rare and unfortunate cases that show a malignant tumoral trend.