Robert A. Garrett

Bilateral Wilms Tumor

Despite institutional variation in the treatment of synchronous bilateral Wilms tumor, a 76 per cent over-all 3-year survival rate has been achieved. We reviewed 145 patients with stage V disease (synchronous bilateral Wilms tumor) registered with National Wilms Tumor Studies 2 and 3. The majority of the patients (94, or 65 per cent) underwent initial surgical resection followed by chemotherapy with or without radiation, with a 3-year survival rate of 82 per cent. However, survival of 45 patients who underwent initial biopsy, postoperative chemotherapy and second-look surgery was 57 per cent at 3 years, a result not significantly different statistically from the 82 per cent figure. Only 38 per cent of the patients had all tumor resected at 1 or more operations. Therefore, emphasis is being placed on chemotherapy as the predominant treatment modality for this disease. Unfavorable histology was found in 10 per cent of the patients and 6 patients (4 per cent) had discordant histology, that is unfavorable histology on 1 side with contralateral favorable histology. Better prognostic factors were patient age less than 3 years at diagnosis, lower stage of the most advanced lesions, favorable histology and negative nodal involvement. Currently, routine bilateral biopsy of tumors is recommended, followed by chemotherapy according to the stage of the most advanced lesion. Impressive shrinkage of tumor can be expected with the hope that surgery will allow renal preservation (excisional biopsy or partial nephrectomy) rather than nephrectomy.

Colonic Adenocarcinoma: A Delayed Complication of Ureterosigmoidostomy

Two cases are reported of adenocarcinoma of the colon developing 26 and 33 years after the patients had undergone ureterosigmoidostomy. The literature is reviewed and the clinical implications are discussed.

Congenital posterior urethral polyps.

Congenital posterior urethral polyps are benign growths that can cause a variety of symptoms in young boys. They are pathologically distinct growths usually discovered by voiding cystourethrography. We present 5 cases of congenital posterior urethral polyps, and discuss the presentation and pathophysiological status of these lesions. These lesions must be considered in the differential diagnosis of voiding dysfunction in young boys.

Perirenal urinary extravasation associated with urethral valves in infants.

We present 7 cases of urethral valves and perirenal urinary extravasation, and review 37 cases reported previously in the literature. The extravasation usually is from the kidney (66 per cent) and often is associated with reflux (70 per cent). It appears to be a pressure-related phenomenon. Although the mortality associated with the condition is significant (32 per cent) renal function is usually preserved. Prompt treatment with correction of metabolic acidosis, and decompression of the tense abdomen and the urinary tract is recommended.

Retroperitoneal fibrosis in children (Eosinophilic and idiopathic)

Two cases of retroperitoneal fibrosis in children are presented; one was idiopathic while the other was in association with eosinophilic cystitis, heretofore not reported. A literature search of patients under eighteen years of age reviews presentation, treatment, and associated diseases.

Pediatric urethral and perineal injuries.

Perineal and urethral injuries in the young are fortunately uncommon. Their sequelae are often more difficult to manage than the original injury. Self-inflicted injuries are quite unusual and iatrogenic injury to the urethra is seldom encountered today. Most injuries are the result of external trauma. The management of these injuries and their sequelae are described in detail.