Intrarenal neuroblastoma masquerading as Wilms' tumor.

OBJECTIVES: To better characterize intrarenal neuroblastoma. METHODS: We retrospectively reviewed the records of 5 children with neuroblastoma initially diagnosed as Wilms' tumor. RESULTS: Five patients, aged 11 months to 3.5 years, were preoperatively diagnosed as having intrarenal Wilms' tumor, prior to operative or histopathologic diagnosis of neuroblastoma. In 3 children the diagnosis of neuroblastoma involving the kidney was confirmed; in the fourth child subcutaneous metastatic disease precluded biopsy confirmation of the intrarenal tumor; and in the fifth child the kidney was severely displaced but not actually involved by neuroblastoma. All patients were hypertensive and 4 patients had elevated urinary catechol levels. Molecular genetic studies showed N-myc amplification in both patients who were evaluated. All patients had poorly differentiated histopathology. One patient is presently alive at 8 months with recurrent disease and another patient is free of disease 13 years after diagnosis. The remaining 3 patients died of the malignancy. CONCLUSIONS: Intrarenal neuroblastoma is a rare entity that clinically and radiographically may resemble Wilms' tumor. Our limited experience indicates that intrarenal neuroblastoma is an aggressive malignancy, and long-term survival is rare.

Metachronous renal cell carcinoma metastasis to the contralateral adrenal gland.

OBJECTIVES: Metachronous metastasis of renal cell carcinoma to the contralateral adrenal gland is very rare. We review our experience with 5 such patients and compare it with reports in the literature. METHODS: The records of all 350 patients who underwent nephrectomy for renal cell carcinoma in our center between 1975 and 1992 were reviewed. Five patients were found to have had solitary metachronous metastases to the contralateral adrenal gland on follow-up. RESULTS: The adrenal metastasis was discovered 18 to 210 months (mean 66.8) after nephrectomy. In 2 patients the lesion was found incidentally on routine computed tomography scan; in the other 3 patients, diagnosis was by ultrasonography, performed because of flank pain and weight loss or routine follow-up. All patients underwent adrenalectomy. Survival ranged from 8 to 64 months (mean 36.4); 3 patients had no evidence of disease at 42, 44, and 64 months postoperatively, and 2 patients died of pulmonary metastasis at 8 and 24 months. Analysis of the clinical data of our 5 patients together with the 9 we found in the published reports revealed that the mean interval between nephrectomy and the appearance of adrenal metastasis was shorter in the patients who died. CONCLUSIONS: The results of adrenalectomy for metachronous metastasis of renal cell carcinoma to the contralateral adrenal gland are unpredictable. The prognosis is somewhat better when the mean interval between the nephrectomy and the appearance of the adrenal metastasis is longer than 18 months. We recommend adrenalectomy because long-term survival is expected in some of these patients.

Does the narrow operating field in perineal radical prostatectomy lead to more positive surgical margins?

AIMS: To assess the risk of leaving cancer-positive surgical margins in the perineal approach for radical prostatectomy as compared to the retropubic approach. METHODS: Seventy-six patients with clinically organ-confined prostate cancer (stage T1-2 NoMo) underwent radical prostatectomy. The 57 patients who underwent retropubic prostatectomy were compared to 19 patients in whom the perineal approach was undertaken. The two groups were compared for pre-operative PSA levels, clinical stage, biopsy Gleasson score, and any correlation between pre- and post-operative stage and grade of the disease and rate of cancer-positive surgical margins. RESULTS: Although there were no significant differences in the rate of organ-confined diseases and specimen Gleasson score in the two groups, the rate of positive surgical margins in the perineal approach was significantly lower (15.7 vs 29.8%) and the rate of extracapsular disease with negative margins was significantly higher (15.7 vs 7%). CONCLUSIONS: The narrow surgical field in the perineal approach for radical prostatectomy does not pose a higher risk for positive surgical margins and it might be the procedure of choice in stage T1C prostate cancer with a Gleasson score of below 7.

Long-term results of lower urinary tract reconstruction with the ceco-appendiceal unit.

PURPOSE: We report long-term results in 11 patients born with bladder exstrophy who underwent lower urinary tract reconstruction using a ceco-appendiceal unit. MATERIALS AND METHODS: Four boys and 2 girls underwent lower urinary tract reconstruction using an unaltered ceco-appendiceal unit. In 2 boys and 1 girl the bladder was replaced with a reservoir of terminal ileum, cecum and ascending colon, and the appendix was used as an orthotopic neourethra. In another 2 boys and 1 girl the bladder was augmented, while the appendix was used to create a stoma to the umbilicus. In 5 patients in whom the ceco-appendiceal junction was incompetent the cecum was plicated over the base of the appendix to reinforce the sphincteric mechanism. Four patients underwent augmentation with the appendix brought out as an umbilical stoma, and in 1 the bladder was replaced and the appendix was used as a neourethra. RESULTS: Six patients in whom the ceco-appendiceal junction was unaltered and 5 in whom it was plicated remain continent 5 to 11 and 2 to 7 years postoperatively, respectively. In the initial patient urinary incontinence developed due to high intraluminal pressure, which resolved after detubularization of the urinary reservoir. Another patient underwent revision of the abdominal stoma. CONCLUSIONS: The ceco-appendiceal unit may be used for continent lower urinary tract reconstruction. Ceco-appendiceal junction competence can be tested intraoperatively and the sphincteric mechanism may be reinforced as necessary. The appendix may be ectopically or orthotopically placed and used for intermittent catheterization.

Role of chronic inflammation in the promotion of prostatic hyperplasia in rats.

PURPOSE: Chronic prostatitis is a common histopathological finding in prostatectomized patients. A possible interrelationship between the presence of leukocyte exudate and the extent of the hyperplastic lesions has been suggested. The aim of the present study was to analyze the effect of the immunomodulator compounds, Complete Freund Adjuvant (CFA) and cyclosporin A (CsA), administered alone or together with citral on the induction and extent of rat prostatic hyperplasia. MATERIALS AND METHODS: Adolescent Wistar rats (42 days old) were given citral alone or combined with CFA or CsA for one month. Semiquantitative analysis of the extent of the hyperplastic lesions was made with the histoscore protocol. RESULTS: CsA did not induce hyperplastic changes or abolish the ability of citral to promote hyperplastic changes or to affect the extent of the lymphocytic exudate in the stroma. CFA itself, however, had a proliferative action on the prostatic epithelium, and it augmented the hyperplastic changes induced by citral and even induced atypical transformations of the acinar epithelium. CONCLUSIONS: Immunoinflammatory stimulators might play a role in the prostatic epithelial cell growth and proliferation processes, most probably by modulation of the cytokine system.

Bilateral asynchronous renal cell carcinoma: treatment approach.

Two hundred patients underwent nephrectomy for renal cell carcinoma (RCC) at our service in 1978-89. During follow-up four patients developed RCC in the remaining kidney and underwent a parenchymal preserving procedure as curative treatment (one enucleation, and three partial nephrectomies). The interval between the nephrectomy and the diagnosis of asynchronous RCC was 50-84 months (mean 65 months). None of the patients needed dialysis after the operation, and creatinine level in all remained between 1.6 and 1.9 mg. One patient died from a heart attack 8 years after the partial nephrectomy, two patients have no evidence of disease 24 months and 76 months after enucleation and partial nephrectomy, respectively. The fourth patient, who had resection of metastatic lesion in the scalp 36 months before the partial nephrectomy, is alive 34 months after the procedure but has lately developed recurrence in the scalp. Although relatively rare, it is important to be aware of the possible asynchronous development of RCC in the contralateral kidney after nephrectomy for RCC. In such cases, parenchymal preserving procedure (enucleation, partial nephrectomy) is an alternative that should be considered to improve the quality of life and to avoid the need for dialysis.

Prenatal diagnosis of crossed ectopic multicystic kidney.

The combination of multicystic kidney and crossed ectopia is very rare. In most of the affected children, diagnosis has been made postnatally by ultrasonographic examination to evaluate a palpated abdominal mass. We describe the third male newborn reported in the literature with a prenatally diagnosed crossed ectopic multicystic dysplastic kidney. As with isolated multicystic kidney, crossed ectopic multicystic kidney should be followed closely after birth by repeated ultrasound for spontaneous regression. Only in cases of anomalies of the contralateral (uncrossed) kidney should the question of surgical intervention be raised.

Involution rate of multicystic renal dysplasia.

OBJECTIVE: To document the involution rate and long-term results of management of multicystic dysplastic kidney. MATERIALS AND METHODS: Data were collected retrospectively for all 23 infants (16 boys) with multicystic dysplastic kidney who were treated at our center over the last 19 years (1977-1995). The diagnosis was based on prenatal ultrasound in 18 patients and on palpable abdominal mass in 5, and confirmed in all patients by postnatal ultrasound and radioisotope scan. Voiding cystography was performed in 18 patients to exclude vesicoureteral reflux. Mean follow-up was 46 months (range, 3 months to 5 years) and included serum creatinine measurements and renal ultrasonography. RESULTS: Two groups of patients were identified. Ten (43.6%) with other urologic abnormalities (group A) and 13 patients without other urologic abnormalities (group B). Vesicoureteral reflux was observed in 4 patients. Nephrectomy was performed in 4 patients, all from group B. The other 19 patients were treated conservatively. Complete involution was observed in 8 patients in group A and 6 in group B after a mean follow-up period of 9.2 and 10 months, respectively. Two patients, 1 from each group, later underwent nephrectomy not because of no involution but because of an increase in the size of the kidney involved. CONCLUSION: Patients with multicystic renal dysplasia have significant associated urologic malformations, and the natural history of the disease is unpredictable. All patients require appropriate investigation of the urinary tract and long-term follow-up. The most outstanding finding of the study is the much higher involution rate of multicystic renal dysplasia and the rate of associated urologic abnormalities than that reported in the literature. Surgery remains an option for the patients in the absence of no involution. multicystic dysplastic kidney, surgery, conservative.

Incidental finding of rectal carcinoma during transrectal US for prostatic diseases.

During transrectal ultrasound (TRUS), rectal carcinoma was an incidental finding in seven patients among a series of 5,000 TRUS examinations. TRUS was performed in seven patients with symptoms characteristic of prostatic diseases. All seven patients underwent examination by at least one physician before TRUS and, except for abnormal prostatic findings, no tumors were detected during digital rectal examination (DRE). The tumors were clearly visualized with TRUS and were easily palpated during DREs performed after TRUS. They were large and were located mainly along the posterior and lateral walls of the rectum. All the tumors were diagnosed by means of proctoscopy; the biopsy findings were positive, and the pathologic staging indicated advanced disease: adenocarcinoma of the rectum with a minimum grade of Dukes C. It is recommended that, in addition to evaluation of scans obtained in the transverse plane, the multiplane transducer be used to evaluate the longitudinal plane of the rectum for detection of possible undiscovered tumors.

Cytokeratin 20: a new marker for early detection of bladder cell carcinoma?

PURPOSE: Cytokeratins are constituents of the intermediate filaments of epithelial cells in which they are expressed in various combinations depending on epithelial type and degree of differentiation. Of the 20 known cytokeratins, cytokeratin 19 is expressed in normal urothelium cells, whereas the recently identified cytokeratin 20 (CK-20) is expressed in urothelial carcinoma but not normal urothelium cells. We examine whether CK-20 expression can be used as a bladder tumor marker for transitional cell carcinoma in cells isolated from urine. MATERIALS AND METHODS: The reverse transcriptase polymerase chain reaction method was used to determine expression of CK-20 in cells separated from urine of patients with bladder carcinoma. Cells were obtained from urine of 192 patients stratified into 3 groups of 21 healthy young volunteers without a history of transitional cell carcinoma, 27 with a negative bladder biopsy for transitional cell carcinoma and 144 with bladder transitional cell carcinoma. The parameters were tumor stage and grade, tumor size, number of tumors, urinary cytology and CK-20. RESULTS: CK-20 amplification band (370 base pairs) was obtained with messenger ribonucleic acid extracted from transitional cell carcinoma cells of bladder tumor. CK-20 in the urine samples of the control group was negative (no false-positive results, specificity 100%). Among the 27 patients with pTo disease CK-20 was negative in 20 (specificity 74.1%). In the 7 patients with positive CK-20 histology showed chronic inflammation in 2, atypical hyperplasia in 3 and metaplasia in 1. In 1 patient who had a known history of transitional cell carcinoma the urothelium was normal. Among 144 patients with bladder transitional cell carcinoma CK-20 was positive in 131. Sensitivity of the method was much higher than urinary cytology (91 versus 56.3%, p <0.0001). We demonstrated no correlation between CK-20 and tumor grade. CONCLUSIONS: Our results indicate that CK-20 is a potential marker for bladder cancer. The noninvasive detection method assesses urothelial cells from the voided urine specimen using reverse transcriptase-polymerase chain reaction. The CK-20 marker was significantly more sensitive than urinary cytology.

Management of renal angiomyolipoma: analysis of 15 cases.

OBJECTIVE: We present our experience with 15 patients with renal angiomyolipoma warranting intervention. METHODS: The medical records and radiological studies were reviewed for patient age and sex, tumor location and size, association with tuberous sclerosis, and treatment approach. All patients were regularly followed by ultrasound and computed tomography scan. RESULTS: Presenting symptoms were retroperitoneal bleeding in 9 patients and flank pain in 6. Excluding cases of tuberous sclerosis (mean tumor diameter 11 cm), the mean diameter of the two tumors that bled was 5.4 cm, similar to those in the patients presenting with flank pain. Two patients with retroperitoneal bleeding had tumors < 3 cm. Angioinfarction was performed in 7 patients, partial nephrectomy in 3, and total nephrectomy in 4. One patient with tuberous sclerosis, who was observed only, died of bleeding and sepsis. The mean follow-up period of 4.3 years revealed stable creatinine levels and no recurrent hemorrhage. CONCLUSIONS: The management approach of angiomyolipoma should be aimed at parenchymal preservation which can be effectively accomplished by limited surgery or preferably by selective embolization. Preventive embolization may be feasible even for small tumors. However, any doubt about the diagnosis of angiomyolipoma should be clarified by surgery.

A bioassay evaluation of the urinary antibacterial efficacy of low dose prophylactic antibiotics in children with vesicoureteral reflux.

PURPOSE: We evaluated by means of a bioassay the efficacy of 4 different antibiotics administered in a prophylactic dose to children with vesicoureteral reflux. MATERIALS AND METHODS: A total of 159 urine samples from 53 children taking prophylactic antibiotics with proved vesicoureteral reflux were tested. The children were divided into 4 groups according to the antibiotic given, which included nalidixic acid, cephalexin, cotrimoxazole and cefixime. Urine samples were collected in the morning, at noon and in the evening, and each sample was bioassayed for growth inhibition of a standard Escherichia coli. The urine volume used was specifically determined for each antibiotic, and growth inhibition by this specific volume was equivalent to that produced by standard diffusion disks. In addition, the specific gravity, which reflected urinary concentration of each sample, was measured. RESULTS: Mean patient age plus or minus standard deviation of the 4 groups was 53 +/- 41 for nalidixic acid, 23 +/- 34 for cephalexin, 55 +/- 35 for cotrimoxazole and 47 +/- 35 months for cefixime, respectively. In children less than 2 years old specific gravity was higher in the morning (1.021 +/- 0.0006 versus 1.0008 +/- 0.0004 at 8 a.m. and 2 p. m., respectively, p <0.05). In contrast, in children older than 4 years the specific gravity was higher in the afternoon and evening hours (1.019 +/- 0.003 versus 1.007 +/- 0.003 at 2 p.m. and 8 a.m., respectively, p <0.05). The percentage of patients who demonstrated growth inhibition in all 3 samples of the test day was 7%, 6%, 69% and 44% for nalidixic acid, cephalexin, cotrimoxazole and cefixime, respectively (p <0.001 for cotrimoxazole and cefixime versus nalidixic acid and cephalexin. Divided into morning, noon and evening, the percentage of samples that demonstrated growth inhibition was 85.7%, 21.4% and 7.1% for nalidixic acid, 37.5%, 12. 5% and 6.3% for cephalexin, 100%, 92.3% and 76.9% for cotrimoxazole and 100%, 77.7% and 55.5% for cefixime, respectively. A direct correlation was found between specific gravity and growth inhibition (r = 0.55, p <0.001). CONCLUSIONS: Urine concentration during the day is dependent on age with older children having more concentrated urine in the latter part of the day. Growth inhibition is enhanced by concentrated urine. Compared to nalidixic acid and cephalexin, cotrimoxazole and cefixime produce a sustained bactericidal effect for about 60% of a 24-hour day due to the longer half-life.