Intracardiac Wilms' tumor: diagnosis and management

Complete excision of Wilms' tumor may require resection of adjacent organs and removal of intracaval tumor propagation. Extension of tumor to the right atrium can be determined preoperatively guiding a direct and safe approach to intracardiac tumor at the time of nephrectomy. Preoperative ultrasonography of two children with Wilms' tumor demonstrated caval and right atrial tumor. Laparotomy for nephrectomy and abdominal caval exposure was combined with cardiopulmonary bypass and atriotomy. In both patients, tumor contiguous with the renal pelvis extended from the iliac bifurcation into the right atrium with a large atrial mass. In one patient nephrectomy was performed first, and she was then placed on cardiopulmonary bypass. Caval tumor was easily removed through the atriotomy and open renal vein. In the second patient, bypass was instituted first because of cardiac instability. The large right atrial mass extended through an atrial septal defect into the left atrium. The cardiac tumor and a large amount of caval tumor were removed. Bypass was discontinued after repair of the ASD. Tumor remained in the IVC below the renal veins necessitating a separate venotomy. Combined abdominal and cardiac exploration allows safe and complete excision of all gross tumor. Ultrasonography is a sensitive and noninvasive method of diagnosing retrohepatic and atrial tumor extension and can be obtained easily even on very sick patients.     

Bilateral multifocal Wilms' tumor

A three-year-old boy who presented with symptoms of peritonitis was found to have four Wilms' tumors affecting both kidneys. Individual enucleation of three tumors in the right kidney plus left lower nephrectomy were performed. Chemotherapy was administered for one year. The diagnosis of Wilms' tumor was confirmed on each specimen by the histologic studies. The child remains asymptomatic and developing normally six years after the initial surgical treatment. Bilateral partial nephrectomies is the most conservative of the surgical treatments available for bilateral Wilms' tumor.     

Retroperitoneal lymph node dissection for Wilms' tumor.

Nephrectomy for Wilms' tumor was performed on 58 patients over a 20-yr period, and retroperitoneal lymphadenectomy was performed on 35 who presented with no demonstrable metastases. The survival rate was 100% for 19 clinical group I patients with negative nodes. Positive nodes in 9 instances led to 5 long-term survivors and significantly influenced staging as a guide for further therapy.     

Bilateral Wilms' tumor and secondary malignancies

A series of six patients with bilateral Wilms' tumor (nephroblastoma) is presented. Multimodal therapy yielded a survival rate of 83% at 2 yr of follow-up. However, at the end of a later follow-up period only two patients (33%) were alive. of the 4 patients who died. Only 1 died of Wilms' tumor. One died of complications of aggressive chemotherapy and two patients died after 12 and 16 yr following treatment of secondary malignant tumors arising in the irradiated region. Patients with bilateral tumor should be followed at regular intervals for the duration of life for the occurrence of secondary malignant tumors.     

Pleural effusion in Wilms' tumor.

The association of pleural effusion and Wilms' tumor is uncommon. We report three patients who developed pleural effusion as a result of different mechanisms, all of which were related to Wilms' tumor or to its treatment.     

Renin producing Wilms' tumor

A 4-yr-old boy with Wilms' tumor, hypertension, and hyperreninemia is presented. Three times as much renin was extracted from tumor tissue as from adjacent renal parenchyma, suggesting that the tumor itself was the primary source of the renin and cause of the hypertension. Other reported cases of hypertension and increased renin production with Wilms' tumor are reviewed. Hypertension is a very frequent finding in patients presenting with Wilms' tumor. The possibility that many of these neoplasms may be found to hypersecrete renin if properly investigated is discussed.     

Wilms' tumor in crossed fused renal ectopia.

The occurrence of a nephroblastoma in a crossed ectopic kidney has been reported only one previous time. A second patient with these findings is the subject of this report.     

The coexistence of Wilms' tumor and imperforate anus with rectourethral fistula

Wilms' tumor or nephroblastoma is one of the more common abdominal neoplasms in childhood. Its association with aniridia, hemihypertrophy, and genitourinary anomalies is well recognized. We believe this is the first report of Wilms' tumor coexistent with imperforate anus and rectourethral fistula.     

Survival in bilateral Wilms' tumor--review of 30 National Wilms' Tumor Study cases.

Bilateral tumors were found in 33 patients or 5.4% of 606 children enrolled in the National Wilms' Tumor Study. Twenty six of 30 patients (87%) for whom detailed clinical information is available survived 2 yr after conservative surgery and radiation therapy. All patients were given one or more chemotherapeutic agents. A variety of surgical procedures were used including biopsy only, nephrectomy, and partial nephrectomies. Radiation therapy doses ranged widely, but between 1000 and 2000 rad apparently sufficed for local control in most cases. Recommendations regarding management by surgery, radiation therapy, and chemotherapy are given.     

Treatment strategy for nodular renal blastema and nephroblastomatosis associated with Wilms' tumor.

Nodular renal blastema and nephroblastomatosis were present in 8 of 118 patients (6.8%) with Wilms' tumor. Five of these 8 patients (63%) had bilateral Wilms' tumors. Two had hemihypertrophy. Preoperative renal angiograms were accurate in detecting these metanephric anomalies. The surgical approach consisted of removal of the most diseased kidney and biopsy for diffuse tumors and wedge resections for localized tumors for the remaining kidney. Postoperatively, radiation was administered when tumor extended outside the kidney. Chemotherapy consisted of vincristine and dactinomycin for 18 mo and adriamycin for 6 mo. This method of management resulted in tumor-free survival of these 8 patients for 1--44 mo (median 24 mo). Nodular renal blastema and nephroblastomatosis may possibly develop into Wilms' tumor. All of these three conditions respond to surgery, chemotherapy, and radiation. When a Wilms' tumor is encountered, it is better to explore and possibly biopsy the opposite kidney. There is a place for second-look laparotomy in this spectrum of congenital anomalies.     

The use of the portal system for the transplantation of a neonate kidney graft in a child with Wilms' tumor

A single an-encephalus neonate kidney graft was transplanted into the portal system of a 6-year-old recipient who had previously undergone removal of the right kidney and inferior vena cava because of Wilms tumor. The left kidney ceased to function shortly thereafter. The child was supported very poorly on hemodialysis, and showed repeated very high levels of cytotoxic antibodies in her serum. The first cross-negative kidney graft that was available harbored two main arteries and duplicate collecting system with two very thin ureters. These vascular anatomic and pathologic variations of both donor graft and recipient necessitated the use of the portal system for renal graft venous drainage and the aorta for the graft revascularization. The ureters that had pinpoint-like lumen were inserted together into the lumen of the native ureter stump and fixated. One year after the transplantation the serum creatinine level is 1.8 mg/dL.     

Treatment of bilateral Wilms' tumors--a 22-yr experience

A total of 157 children with Wilms' tumors were treated at the Children's Memorial Hospital during the years 1956-1978. Fourteen (or 9%) of these children had bilateral lesions. During the early years, treatment consisted of surgical excision of one kidney with radiation to the second tumor. During the second decade of this study a more aggressive approach to bilateral tumors was used consisting of nephrectomy on the side of the large tumor, with a heminephrectomy on the contralateral side and chemotherapy. More recently, we have attempted bilateral heminephrectomy when possible, together with increasingly aggressive chemotherapy. This series of patients allows us to compare the results of various treatment modalities among our own patients as well as those reported from other pediatric surgical centers.     

Bilateral,multicenteric metanephric adenoma associated with Wilms' tumor in a child: A rare presentation with important diagnostic and therapeutic implications

Metanephric adenoma is an uncommon benign renal tumor that occurs predominantly in adult females and rarely in children. On histomorphology it shows a resemblance to Wilms' tumor, nephrogenic rests and papillary renal cell carcinoma. Multifocality along with multicentricity has not been documented in English literature till date. From a diagnostic and therapeutic viewpoint, recognition of this entity is of the utmost importance, because it shows a favorable clinical outcome. We describe a rare case of bilateral, multicentric metanephric adenoma associated with triphasic Wilms' tumor (stage II) of the left kidney in a male child.     

Intracranial metastatic Wilms' tumor in children: a report of two cases

Two cases are presented of children with intracranial metastasis from Wilms' tumor. Both patients had undergone nephrectomies for Wilms' tumor, one 19 months and the other 32 months prior to clinical presentation of intracranial metastasis. Computed tomographic examination in both cases demonstrated lesions that did not have any particularly unique features. Subsequent craniotomy and removal of the tumor in one case confirmed metastatic intracranial Wilms' tumor.     

Renal transplantation in patients with bilateral Wilm's tumor.

Review of cases and of published reports of patients with bilateral Wilms' tumor revealed a significantly higher mortality rate in those who had received a renal transplant than in those who had not. The increased mortality is attributed to overwhelming sepsis resulting from chemotherapy, radiotherapy and immunosuppression. Growth of the tumor did not appear to be accelerated by immunosuppression and transplantation.     

Nonvisualization of the intravenous pyelogram—A poor prognostic sign in Wilms' tumor?

In a series of 49 consecutive cases of Wilms' tumor from a single institution, there was a 20% incidence of nonvisualization on the initial intravenous pyelogram. Even though nonvisualization was secondary to either gross or microscopic invasion of the renal vein or renal pelvis, this did not forecast a grim progosis. Seven of the nine patients with nonvisualization are presently alive and tumor-free at least 5 yr after resection. The only two deaths in this particular group were unrelated to the Wilms' tumor.     

Second-look laparotomy in advanced ovarian cancer: A critical assessment of morbidity and impact on survival

Background: The routine inclusion of second-look laparotomy in the management of patients with epithelial ovarian cancer is controversial. At issue is the justification of morbidity incumbent upon surgery and the possible survival benefit of secondary cytoreduction. Methods: The rate of major complications of surgery was assessed among 100 consecutive patients with FIGO stage III or IV epithelial ovarian cancer who underwent second-look laparotomy. All patients demonstrated a complete clinical and biochemical (CA125 <35 U/ml) response to first-line therapy. Patients were stratified based on findings at surgery. Patients in group 1 (n=37) had a negative second-look laparotomy. Patients in group 2 (n=35) had only microscopically appreciable disease. Patients in group 3 (n=28) had macroscopic disease. Cytoreductive efforts aimed at resection of all macroscopic disease were carried out for patients in group 3. Results: Thirteen patients (13%) had 15 major complications at surgery. Comparison of the complication rates for patients in groups 1, 2, and 3, of 10%, 8.5%, and 21.4%, respectively, did not achieve statistical significance (p=0.228). The estimated 5-year survival for patients in groups 1, 2, and 3 of 63.9%, 39.8%, and 14.2%, did differ significantly (p<0.0001). Cytoreductive efforts resulted in the resection of all macroscopic disease in 18 of 28 patients (64.2%) in group 3. The median survival for this group of 18 patients was 33 months, and estimated 5-year survival was 20%. These values do not differ significantly from those observed for patients in group 2. Conclusion: The major complication rate associated with second-look laparotomy is not prohibitive Secondary cytoreductive efforts may result in improved survival for patients with epithelial ovarian cancer.