Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: a report from the National Wilms Tumor Study Group.

Purpose: To determine if tumor-specific loss of heterozygosity (LOH) for chromosomes 1p or 16q is associated with a poorer prognosis for children with favorable-histology (FH) Wilms tumor entered on the fifth National Wilms Tumor Study (NWTS-5).

Patients And Methods: Between August 1995 and June 2002, 2,021 previously untreated children with FH or anaplastic Wilms tumor, clear-cell sarcoma of the kidney (CCSK) or malignant rhabdoid tumor of the kidney (RTK), were treated with stage- and histology-specific therapy. Their tumors were assayed for LOH for polymorphic DNA markers on chromosomes 1p and 16q.

Quality assessment for Wilms' tumor: a report from the National Wilms' Tumor Study-5.

Background/purpose: Surgical technique impacts both local tumor stage and risk of local recurrence in Wilms' tumor. A surgical quality assurance program was part of National Wilms' Tumor Study-5 to assess protocol compliance.

Methods: Surgical checklists, operative, and pathology reports were reviewed concurrently to arrive at the final local tumor stage.

Hyperplastic perilobar nephroblastomatosis: long-term survival of 52 patients.

Background: This study provides insight into the clinical behavior, diagnostic complexities, and long-term management of patients with hyperplastic perilobar nephroblastomatosis (HPLN).

Procedure: Fifty-two patients with HPLN with available long-term follow-up were retrospectively analyzed for pathologic, radiologic, and clinical features.

Results: The mean age at diagnosis was 16 months; the lesions were bilateral in 49 patients.

Treatment outcomes in adults with favorable histologic type Wilms tumor-an update from the National Wilms Tumor Study Group.

Purpose: To describe the clinical outcomes in adults with favorable histologic type (FH) Wilms tumor (WT) registered in the National Wilms Tumor Studies (NWTS) 4-5. We also describe the results of patients treated in the "modern era" (1979-2001) with surgical staging, central pathology review and stage-appropriate multimodality treatment.

Methods And Materials: Twenty-three adult patients (> or =16 years of age) with FHWT after central pathology review were registered on NWTS 4-5.

Successful outpatient management of the nonpalpable intra-abdominal testis with staged Fowler-Stephens orchiopexy.

Purpose: Previous reports of orchiopexies have shown an 85.9% success rate for staged Fowler-Stephens orchiopexy (FSO). We review our experience with nonpalpable testes (NPTs).

Doxorubicin for favorable histology, Stage II-III Wilms tumor: results from the National Wilms Tumor Studies.

Background: After randomized trials in the 1980s, doxorubicin (DOX) was added to dactinomycin plus vincristine as standard chemotherapy for patients who had Stage III Wilms tumor (WT) of favorable histology (FH). Double-agent chemotherapy was retained for patients with Stage II disease. In this study, the authors reevaluated the efficacy of DOX using extended follow-up and additional patients.

Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group.

Purpose: To evaluate the effect of conventional and standard (ST) versus pulse-intensive (PI) chemotherapy and short-duration versus long-duration chemotherapy on relapse-free survival (RFS) and overall survival rates of patients with clear-cell sarcoma of the kidney (CCSK) entered onto the National Wilms' Tumor Study (NWTS)-4.

Patients And Methods: The 5-year and 8-year RFS rates were determined for patients with CCSK treated on the NWTS-4. After August 6, 1986, 40 previously untreated children younger than 16 years with CCSK were randomly assigned, after the completion of 6 months of chemotherapy, to discontinue (short) or continue 9 additional months (long) of treatment with chemotherapy regimens that included vincristine and either divided-dose (ST) courses (5 days) or single-dose (PI) treatment with dactinomycin and divided-dose (ST) courses (3 days) or single-dose (PI) treatment with doxorubicin.

Characteristics and outcomes of children with the Wilms tumor-Aniridia syndrome: a report from the National Wilms Tumor Study Group.

Purpose: Children with the rare Wilms tumor (WT)-aniridia (WAGR) syndrome have not had systematic evaluation of their clinical and pathologic features. We compared demographics, disease characteristics, and treatment outcomes in a large cohort of WT patients who did or did not have the WAGR syndrome.

Patients And Methods: Clinical and pathology records were reviewed for 8,533 patients enrolled between 1969 and 2002 by the National Wilms Tumor Study Group.

Transurethral resection syndrome after bladder perforation.

The use of irrigating solutions is essential for distension of mucosal surfaces and visualization of the surgical field during resectoscopic resection of bladder tumors (TURBT). TURBT resection may be complicated with bladder perforation associated with intraperitoneal extravasation of irrigant fluid, which may rarely evolve in specific hydroelectrolyte imbalance characterized with hyponatremia, intravascular volume deficit, and renal impairment. We report four cases of TURBT syndrome during bladder surgery complicated by bladder perforation and discuss issues relevant to pathophysiology, diagnosis, and treatment of this rare condition.

Chylous ascites following surgical treatment for wilms tumor.

Purpose: Postoperative chylous ascites is a rare complication of retroperitoneal surgery that has considerable morbidity. We review the pathogenesis and management of chylous ascites following surgical treatment of Wilms tumor.

Materials And Methods: We identified 9 children with chylous ascites after surgical treatment of Wilms tumor.

Successful use of tunica vaginalis grafts for treatment of severe penile chordee in children.

Purpose: Prior reports have suggested that tunica vaginalis free grafts for the treatment of chordee are associated with a high failure rate and residual curvature. We reviewed our experience with tunica vaginalis free grafts for the treatment of severe chordee associated with scrotal and perineal hypospadias.

Materials And Methods: The records of 25 children with scrotal or perineal hypospadias associated with severe ventral chordee requiring a corporal graft to straighten the phallus treated from 1996 to 2001 were reviewed.

Influence of radiation therapy delay on abdominal tumor recurrence in patients with favorable histology Wilms' tumor treated on NWTS-3 and NWTS-4: a report from the National Wilms' Tumor Study Group.

Purpose: This study was undertaken to determine whether radiation therapy (RT) delay of >or=10 days had an adverse impact on abdominal tumor recurrence among children with favorable histology (FH) Wilms' tumor enrolled in National Wilms' Tumor Study (NWTS) 3 and 4.

Methods And Materials: A total of 1226 patients with Stage II-IV FH tumors who received flank or abdominal RT in NWTS-3 and NWTS-4 were included in this analysis. Recurrent disease in the operative bed was classified as flank recurrence.

Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.

Background/purpose: Cystic partially differentiated nephroblastoma (CPDN) is a rare variant of Wilms' tumor thought to be more favorable than standard nephroblastoma. The purpose of this report is to examine the outcome of children with CPDN, after nephrectomy, treated with vincristine and dactinomycin based chemotherapy (+/- doxorubicin) or no chemotherapy.

Methods: Patients were registered with the National Wilms' Tumor Study Group (NWTSG) and data were collected prospectively.

Outpatient management of phimosis following newborn circumcision.

Purpose: We reviewed our experience in treating patients with a trapped penis due to phimosis following newborn circumcision. The outcome of treatment of this condition at the outpatient clinic was examined. Possible etiological factors leading to this complication were determined.

The occurrence of Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study Group (NWTSG).

Background/purpose: An increased incidence of Wilms tumor has been noted in patients with a horseshoe kidney. These represent a difficult diagnostic and therapeutic challenge. The charts of all National Wilms Tumor Study Group (NWTSG) patients with Wilms tumor occurring in a horseshoe kidney were reviewed.

Recent progress in the biology and treatment of Wilms' tumor.

The survival rate of patients with Wilms' tumor has rapidly improved in the last few decades. As we enter into the new millennium, overall survival for all Wilms' tumor patients exceeds 80%. Two large cooperative groups prospectively studied children with nephroblastoma: the National Wilms' Tumor Study Group, and the International Society of Paediatric Oncology.

Treatment with nephrectomy only for small, stage I/favorable histology Wilms' tumor: a report from the National Wilms' Tumor Study Group.

Purpose: Children younger than 24 months with small (< 550 g), favorable histology (FH) Wilms tumors (WTs) were shown in a pilot study to have an excellent prognosis when treated with nephrectomy only.

Patients And Methods: A study of nephrectomy only for the treatment of selected children with FH WT was undertaken. Stringent stopping rules were designed to insure closure of the study if the true 2-year relapse-free survival rate was 90% or lower.

Controversies in the management of Wilms' tumor.

Wilms' tumor is the most common malignant renal tumor of childhood; it represents 5% to 6% of childhood cancers in the United States. The survival rate of children with Wilms' tumor has improved dramatically, partly due to large multicenter studies conducted by the National Wilms' Tumor Study Group and the International Society of Pediatric Oncology. To ensure optimal patient outcome, the surgical management of these patients must be appropriate.

Intravascular extension of Wilms tumor.

Objective: To define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms tumor.

Methods: Children on a collaborative study of Wilms tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists and surgical and pathology reports were reviewed.

Advantages of rectus fascial slings for urinary incontinence in children with neuropathic bladders.

Purpose: Many surgical procedures to improve outlet resistance in children with neuropathic bladders are obstructive and increase the detrusor leak point pressure. In contrast, fascial slings are designed to achieve continence by increasing the Valsalva or stress leak point pressure without altering the detrusor leak point pressure. We evaluate the effectiveness of fascial slings in achieving continence in pediatric patients with neuropathic bladder.

Surgical complications after primary nephrectomy for Wilms' tumor: report from the National Wilms' Tumor Study Group.

Background: Surgical complications are a recognized morbidity of the treatment of patients with Wilms tumor. This study examines the incidence of surgical complications in the most recently completed study from the National Wilms' Tumor Study Group (NWTSG).

Study Design: The fourth National Wilms' Tumor Study (NWTS-4) enrolled 3,335 patients from August 1986 to August 1994.

Wilms' tumor. Overview of National Wilms' Tumor Study Group results.

Since its inception in 1969, the NWTSG has performed successful studies that are now the model for the management of pediatric malignancies. Future studies may use genetic markers to stratify high-risk patients beyond the traditional staging system. Therapy will continue to be evaluated to determine the minimal therapy necessary to achieve the best outcome for children with Wilms' tumor.

Renal failure in the Denys-Drash and Wilms' tumor-aniridia syndromes.

Nearly 6000 patients enrolled in four clinical trials of the National Wilms' Tumor Study Group during 1969-1995 were followed until death or for a median of 11.0 years of survival for the onset of renal failure (RF). Thirteen of 22 patients with Denys-Drash syndrome and 10 of 46 patients with the Wilms' tumor aniridia syndrome developed RF.