Treatment outcomes in patients less than 2 years of age with small, stage I, favorable-histology Wilms' tumors: a report from the National Wilms' Tumor Study.

PURPOSE: Retrospective analyses were performed to determine the effect of tumor weight and therapy modifications on outcome in patients less than 2 years of age with stage I favorable-histology Wilms' tumors. PATIENTS AND METHODS: The 4-year relapse-free and overall survival percentages for patients randomized to different treatment regimens in National Wilms' Tumor Studies (NWTS)-1, -2, and -3 were calculated and compared. RESULTS: The 4-year relapse-free survival percentages of patients whose specimen weight was less than 550 g were found to be 89.1% on NWTS-1, 96.0% on NWTS-2, and 93.2% on NWTS-3. There was no evidence that the relapse-free survival of these patients had improved over time (P value for trend = .99). The 4-year relapse-free survival percentage for similar age and stage patients whose specimen weight was 550 g or greater was significantly poorer than that of patients with smaller tumors (P = .02). CONCLUSION: Changes in the NWTS treatment regimens over a period of more than 20 years have not improved on the excellent prognosis of patients who are less than 2 years of age at diagnosis and who have a stage I, favorable-histology Wilms' tumor with specimen weight less than 550 g. These data could be used as the basis for a future trial in which a subgroup of such patients is treated with nephrectomy only.

Effect of duration of treatment on treatment outcome and cost of treatment for Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of the administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 905 previously untreated children aged younger than 16 years with stage II favorable histology (FH) WT (low-risk [LR]), stages III to IV FH WT, or stages I to IV clear-cell sarcoma of the kidney (high-risk[HR]) were randomized after the completion of 6 months of chemotherapy to discontinue (short) or continue for 9 additional months (long) treatment with chemotherapy regimens that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either divided-dose (STD) courses (3 days) or single-dose (PI) treatment with doxorubicin. RESULTS: The 4-year relapse-free survival (RFS) rates after the second randomization for LR patients were 83.7% for the 190 patients treated with short and 88.2% for the 187 patients treated with long chemotherapy (P = .11). The 4-year RFS rates after the second randomization for HR FH patients were 89.7% for the 256 patients treated with short and 88.8% for the 246 patients treated with long chemotherapy (P = .87). The charge for treatment with the short PI treatment regimens for all children with stages I through IV FH WT was approximately one half of that with the long STD treatment regimens. CONCLUSION: The short administration schedule for the treatment of children with WT is no less effective than the long administration schedule and can be administered at a substantially lower total treatment cost.

Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group.

PURPOSE: The National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either STD courses (3 days) or PI treatment with doxorubicin. RESULTS: The 2-year relapse-free survival (RFS) rates for LR patients were 91.3% for 544 randomized to treatment with PI and 91.4% for 556 randomized to treatment with STD chemotherapy (P = .988). The 2-year RFS rates for HR patients were 87.3% for 299 randomized to treatment with PI and 90.0% for 288 randomized to treatment with STD chemotherapy (P = .865). CONCLUSION: We conclude that patients treated with PI combination chemotherapy for LR or HR WT or clear cell sarcoma of the kidney have equivalent 2-year RFS to those treated with STD regimens. PI drug administration is recommended as the new standard based on demonstrated efficacy, greater administered dose-intensity, less severe hematologic toxicity, and the requirement for fewer physician and hospital encounters.

Polycystic kidney disease: recognition of the "adult form" (autosomal dominant) in infancy.

This case report and reports from the literature emphasize that the so-called adult form of polycystic renal disease can be diagnosed in infancy. The tern "adult form of polycystic disease" probably should be abandoned and the disease considered the autosomal dominant form of polycystic kidney disease.

Malignant potential of the cryptorchid testis.

A follow-up study of 224 male patients from Rochester, Minnesota, diagnosed with cryptorchidism during the period from 1935 through 1974 was conducted to assess the frequency of subsequent testicular neoplasms. Age-specific rates for testicular neoplasm in this community were used to estimate an expected number for comparison with that observed. Two malignant testicular neoplasms have occurred in this cohort; thus, the standardized morbidity ratio was 11.4 (95% confidence interval, 1.4 to 41.1). Of the 158 patients born in the community, 11% were known to have birth weights of less than 2,500 g, approximately twice the expected occurrence. Increased risks were also noted for inguinal hernia, hypospadias, Down's syndrome, cleft lip or palate (or both), clubfoot, and congenital heart disease, for which the standardized morbidity ratios were 3.6, 4.7, 17.8, 11.3, 11.7, and 2.7, respectively. The increased risk for malignant neoplasms observed in this cohort of patients with cryptorchidism is consistent with that found in earlier studies.

The incidence and outcome of mumps orchitis in Rochester, Minnesota, 1935 to 1974.

A population-based study of mumps orchitis over a 40-year period in Rochester, Minnesota, is reported. All medical records for patients with orchitis, atrophic testis, parotitis, and diagnosed or suspected mumps for the population of Rochester were reviewed, and detailed abstracts were prepared for all those with mumps orchitis. Information abstracted included the relationship of testicular to parotid involvement, the presence of unilateral or bilateral testicular involvement and subsequent atrophy, the presence of other complications of mumps, the treatment of mumps orchitis, and the presence of other major diagnoses. Also investigated was the number of subsequent congenital malformations in male offspring of these patients. The age distribution of the patients with orchitis (median age, 29 years) differed appreciably from those with mumps (median age, 8 years). There was no apparent increase in genitourinary malformations in the male offspring conceived after the occurrence of mumps orchitis in the fathers. Of the 132 men who had orchitis, 2 subsequently had testicular neoplasms. Both of these patients were in the group of 47 who were noted to have an atrophic testis after the occurrence of orchitis.

Incidence of malignant testicular tumors in the population of Rochester, Minnesota, 1935 through 1974.

Twenty-four cases of testicular neoplasms detected in male Rochester residents over a 40-year period were identified using the Rochester-Olmsted County epidemiologic data base. This resource contains nearly complete medical information on residents of Rochester, Minnesota, and the surrounding community. An incidence rate of 3.7/100,000 males per year was calculated. This agrees well with other rates reported in the literature which range from 2.5 to 3.7/100,000 per year; there was no detectable trend over time.

Urethroplasty for hypospadias: long-term results.

A retrospective study of patients who had undergone multi-staged hypospadias repair at the Mayo Clinic was undertaken to identify long-term problems associated with this surgery. Patients were queried concerning their satisfaction with quality of urinary stream; penile erection and ejaculation; sexual function and fertility; and cosmetic appearance.

In search of marker for genetic susceptibility to reflux nephropathy.

The frequency of 40 HLA antigen specificities was determined in 44 patients with end-stage renal disease secondary to reflux nephropathy and compared with those observed in 526 control subjects. Higher than normal frequencies were observed for HLA-B12 in female patients, and HLA-B8 in combination with HLA-A9 or HLA-BW15 in male patients, and HLA-BW15 in patients of both sexes. A comparison of the frequency of HLA antigens in the reflux patients with a history of hypertension versus those without a history of hypertension revealed that HLA-A3 was found with higher frequency and HLA-A1 and HLA-B8 with lower frequency in association with hypertension. These results suggest that the major histocompatibility antigens might be in linkage disequilibrium wih a gene (or genes) determining the susceptibility to renal damage by vesicoureteral reflux and that this linkage, or the clinical expression of this linkage, might be influenced by variations in relation to the sex of the patients.

Correction of total incontinence in male and female epispadias.

Between 1946 and 1980, 54 patients with epispadias and total urinary incontinence were evaluated at our institution. Factors critical to the achievement of complete continence include deferral of surgery until at least 3 yr of age, the patient having a well-developed bladder with adequate capacity and musculature, and maturation of the prostate at puberty in males. Complete continence was obtained in 9 of 11 female patients (82%). Fourteen of 35 male patients obtained complete urinary continence in the initial postoperative period. Complete urinary control was delayed until puberty in 11 additional patients, for an overall success rate of 25 of 35 males (71%). The significant increase in continence with the onset of puberty in males demonstrates the importance of expectant management of the child's progress in relation to the potential contribution of prostatic maturation in the development of urinary control.

Extrarenal Wilms' tumor: results of the National Wilms' Tumor Study.

Extrarenal Wilms' tumor is extremely rare and occurs predominantly in children. Eight cases of extrarenal Wilms' tumor were reported to the National Wilms' Tumor Study from 1980 to 1986. Patients were followed in the study and not randomized to a particular treatment protocol. Seven patients had a favorable histology. One tumor located in the sacrococcygeal region showed immature teratoma with nephroblastic tissue. The embryogenesis of extrarenal Wilms' tumor is controversial; however, tumor containing teratomatous elements most likely represents a different embryologic origin and, therefore, should be classified separately. All eight patients were treated with operative excision and chemotherapy. Seven of the eight patients were disease-free with a mean follow-up of 34.3 months. It can be inferred from this small group of patients that the prognosis is comparable to intrarenal Wilms' tumor in the National Wilms' Tumor Study.

Wilms' tumor in the neonate: a report from the National Wilms' Tumor Study.

Renal neoplasms in the neonate are quite uncommon. Twenty-seven of the 3,340 patients (0.8%) registered on the National Wilms' Tumor Studies from 1969 through April 1984, were 30 days old or less. Of these 27 patients, 18 had mesoblastic nephroma, 1 had a malignant rhabdoid tumor of the kidney, and 4 others had nonneoplastic lesions. The remaining four infants were reviewed in detail. All had favorable histology Wilms' tumors; none had distant metastasis at diagnosis. Treatment ranged from surgery alone to excision plus three-drug therapy for 15 months. All fared well. The patient with Stage I rhabdoid tumor died at eight weeks of age in spite of aggressive four-drug therapy. This review supports the view that Wilms' tumor in the neonate is extremely rare.

Surgical evaluation of lymph node metastases in Wilms' tumor.

Because lymph node (LN) involvement continues to be an adverse prognostic factor in the later National Wilms' Tumor Study (NWTS) trials, the NWTS-3 Surgical Committee undertook a project to determine the accuracy of the present method of sampling. The committee concludes that: (1) there is a relatively low potential error rate (less than 10%) that would comprise adequate care when a policy of LN sampling is followed; (2) this does not encourage routine radical LN dissections in Wilms' tumor patients because of the well-known morbidities associated with that procedure; and (3) LN sampling in all Wilms' tumor patients should be performed with a high index of suspicion present in the mind of the operating surgeon.

Superior mesenteric artery injury during nephrectomy for Wilms' tumor.

Iatrogenic injury to the aorta or its major branches during nephrectomy for Wilms' tumor in children is rarely reported but may be more common than is currently acknowledged. We identified four patients with ligation of the superior mesenteric artery (SMA) that occurred during nephrectomy for nephroblastoma and another child in whom SMA thrombosis developed postoperatively. All of the tumors were on the left side. Interruption of the SMA was recognized intraoperatively in all four children, and primary repair was done. In three patients, appearance of the bowel remained normal before repair of the injury. Three of the arteries were repaired by primary reanastomosis, and one was joined with an interpositioned hypogastric artery graft. None of these patients had gastrointestinal complications postoperatively. In the fifth patient, SMA thrombosis developed after repair of an aortic tear during nephrectomy. This patient required subsequent small bowel resection for bowel infarction and died in the perioperative period. Every surgeon treating children with Wilms' tumor should be aware of the possibly distorted vascular anatomy and take precautions to avoid such a significant injury. Attempts at early ligation of the vessels may not be justified until the renal vasculature is clearly identified.

Bilateral massive nephroblastomatosis in infancy.

This report describes an infant with bilateral diffuse nephroblastomatosis, illustrating the typical clinical and pathologic characteristics that allow it to be differentiated from true bilateral Wilms' tumor.

The painful scrotum: torsion vs epididymo-orchitis.

In summary, an acute disorder of the scrotum in a child without previous urologic history must be viewed as torsion until proved otherwise by surgical exploration. No deleterious effects have been reported from such an approach, which requires a minimal period of hospitalization and little discomfort to the child. The shorter the interval between the onset of symptoms and surgical exploration, the greater the likelihood that the testicle will be viable later. The diagnosis of epididymo-orchitis in a healthy child without a history of urologic abnormality or evidence of lower urinary tract infection has no place in the differential diagnosis of the child with the painful scrotum.

Approach to the foetus with congenital hydronephrosis.

Recent technological advances in prenatal ultrasonography have been responsible for the detection of a variety of congenital abnormalities in utero. The widespread use of this study during pregnancy has led to an increased recognition of foetal hydronephrosis. It has been postulated that unrelieved, high-grade urinary obstruction in utero may produce progressive renal damage. This supposition has resulted in the development of techniques for foetal intervention in attempts to correct obstructive uropathy and arrest renal dysplasia. Herein we review the techniques for assessment of foetal renal function and treatment options available for the foetus with hydronephrosis in utero. Most foetuses with hydronephrosis have dilated, low pressure systems with adequate urinary output and amniotic fluid volume and can be followed safely to term delivery. The accumulated experience to date indicates that the foetus with congenital hydronephrosis detected in utero should be managed expectantly with serial ultrasound examinations after delivery to establish an accurate diagnosis and to evaluate the status of the contralateral kidney. Functional tests after birth, such as renal scans and excretory urography, are mandatory to establish the proper diagnosis and assess the need for operative intervention.