Panayotis P. Kelalis

Renal Lesions in Tuberous Sclerosis

The renal lesion classically associated with tuberous sclerosis is angiomyolipoma. Renal cysts are less frequent, occurring alone or in conjunction with angiomyolipomas. We reviewed the records of 274 patients with tuberous sclerosis registered at our clinic. Of 95 patients evaluated for renal lesions 51 (54 per cent) had positive findings: 45 had angiomyolipomas, 17 had renal cysts and 11 had both lesions. These lesions usually are multiple and bilateral, and are diagnosed most effectively with computerized tomography or ultrasound. Surgery (10 kidneys) was done for life-threatening hemorrhage or suspected malignant lesions.

Calcified Renal Masses: A Review of Ten Years Experience at the Mayo Clinic

Of 2,709 renal masses seen in a 10-year period, 111 contained roentgenographically visible calcium. This was found in 1 to 2% of the simple cysts and in 10% of renal-cell carcinomas. Calcium located non peripherally (within the mass) indicated a malignant lesion in 87% of cases. In another 8%, the masses were indistinguishable from renal-cell carcinoma on vascular studies, and this type of calcification required surgery; only 5% were benign cysts while peripheral eggshell calcification without calcium in the mass was usually associated with benign simple cysts, the risk of malignancy was still about 20%.

The progression of vesicoureteral reflux nephropathy

The relations among renal function, proteinuria, and glomerular lesions were studied in 54 patients with reflux nephropathy. The clinical course to end-stage renal disease was not appreciably altered by late surgical correction of the reflux, occurrence of urinary tract infection, or hypertension. All patients with progressive renal disease had significant proteinuria. Mesangial glomerular lesions can occur in the absence of proteinuria detectable by routine analysis, whereas lesions similar to those seen in idiopathic focal sclerosing glomerulopathy were present in the renal biopsies from proteinuric patients. Deposition of immunoproteins was limited to glomeruli undergoing sclerosis. Similarly, electron-dense deposits were confined to areas of mesangial alterations. Our results suggest that mesangial alterations occur early in the course of reflux nephropathy and may lead to the development of focal sclerosis. At later stages, counterproductive mechanisms of adaptation to the loss of viable nephrons might result in an acceleration of the clinical course to renal failure.

Transitional Cell Carcinoma of the Bladder in Children and Adolescents

A review of our records between 1950 and 1980 identified 12 patients with transitional cell carcinoma of the bladder who were less than 21 years old. The tumors were low grade and low stage, and were associated with an excellent prognosis. Only 1 patient had a solitary recurrence. This study supports the contention that transitional cell carcinoma of the bladder is a less aggressive disease in patients who are in the first 2 decades of life than in older patients.

Hydronephrosis in renal ectopia : incidence, etiology and significance

The incidence, cause and significance of hydronephrosis in patients with renal ectopia are unknown. Therefore, we conducted a retrospective review of 77 patients with 82 ectopic kidneys. Of 82 kidneys 46 (56%) had hydronephrosis. Dilatation of the renal pelvis was the result of primary ureteropelvic or ureterovesical obstruction in 52%, grade 3, 4 or 5 vesicoureteral reflux in 26% and extrarenal collecting systems with malrotation that produced apparent ureteropelvic junction obstruction in 22%. Surgery was performed in 41 patients, including primary nephrectomy in 18, ureteral reimplantation in 14 and ureteropelvic junction repair in 8. Hydronephrosis was present in 15 of 58 contralateral nonectopic kidneys, and 11 required surgery mostly for vesicoureteral reflux. Solitary ectopic kidneys with no associated obstruction or reflux demonstrated normal renal function. Our data indicate that the prognosis in renal ectopia relates directly to the associated urological disease and timely surgical intervention, not to ectopia alone.

Intracaval and Atrial Involvement with Nephroblastoma: Review of National Wilms Tumor Study-3

We reviewed the records of 77 children enrolled in the National Wilms Tumor Study-3 who had involvement of the inferior vena cava with tumor thrombus. None of these patients received preoperative chemotherapy or radiotherapy. Ultrasonography and inferior venacavography were more helpful than computerized tomography in the preoperative diagnosis. The median followup for the group was 2.7 years. The 3-year survival rates for patients with intracaval involvement were determined by stage (88, 89 and 62 per cent for stages II, III and IV, respectively). The most important prognostic factor was histological type. The level of vena caval involvement had no effect on survival; all 16 patients with atrial involvement survived 3 or more years. We continue to recommend appropriate surgical excision of the tumor and thrombus when it is technically feasible.

A Case of Pediatric Ureteroscopic Lasertripsy

The standard treatment for ureteral calculi in children has been open surgical removal. Recently, we removed successfully a lower ureteral calculus in a young child with ureteroscopy and the pulsed dye laser. The improvements in smaller rigid and flexible ureteroscopes, in conjunction with lasertripsy, will expand its applications in the treatment of pediatric urolithiasis.

Extended Followup of Bilateral Wilms Tumor: Results of the National Wilms Tumor Study

The long-term survival of patients with synchronous bilateral Wilms tumor is not well defined. Retrospective review of 185 patients registered with the National Wilms Tumor Study from January 1974 to July 1986 with stage V tumors suggests that the long-term outcome remains good. Over-all survival is 83%, 73% and 70% at 2, 5 and 10 years, respectively. Unfavorable histology, age at diagnosis and the most advanced stage of the individual tumors remain the most important prognostic variables. No significant difference in survival was noted between patients undergoing initial surgical resection of the tumor and those managed with initial tumor biopsy followed by chemotherapy with or without radiotherapy and subsequent surgical resection. Survival does not appear to be compromised by attempting to conserve native renal function with renal-sparing operations.

Prepubertal Yolk Sac Testicular Tumors - Report of the Testicular Tumor Registry

In 1980 the Section on Urology of the American Academy of Pediatrics established a registry of prepubertal testicular tumors. A total of 181 yolk sac tumors has been reported to the registry. Complete followup is available for 154 of these 181 patients. Most of the patients presented with low stage disease. Radical orchiectomy without adjunctive retroperitoneal lymphadenectomy, chemotherapy or radiotherapy was sufficient therapy for most stage 1 cancer patients. alpha-Fetoprotein is a reliable marker for this tumor. Chest x-rays and abdominal computerized tomography are reliable for staging. When metastases appeared they did so within 14 months of presentation so that 2 years of followup after any evidence of disease seems to be adequate. Approximately two-thirds of the patients with metastases were salvaged by chemotherapy, radiotherapy and/or an operation.

Preoperative therapy for intracaval and atrial extension of Wilms tumor

Background. Primary surgical management of Wilms tumor with intravascular tumor extension is associated with significant morbidity. Retrospective analysis of a group of Wilms tumor patients with intravascular tumor extension treated with preoperative therapy was performed to evaluate the feasibility of this approach.