Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration.

PURPOSE: Idiopathic retroperitoneal fibrosis is an uncommon disease of unknown etiology that may involve the ureters and other retroperitoneal structures. Surgical ureterolysis as well as medical treatment with steroids have been used to treat these patients. However, there is no agreement as to the dose and duration of steroid. We adopted a regimen of long-term steroid use in this prospective study. MATERIALS AND METHODS: We treated 12 patients with idiopathic retroperitoneal fibrosis with a regimen of steroids during a 10-year period. Tissue diagnosis was established by biopsy of all lesions and ureteral obstruction was managed with insertion of a nephrostomy tube or a ureteral stent. The initial dose of prednisolone was 60 mg. on alternate days for 2 months and was tapered during the following 2 months to a daily dose of 5 mg. The total duration of prednisolone use was 2 years. RESULTS: Of the 12 patients 11 who completed this treatment regimen have been followed for a duration of 26 to 132 months (median 63.1) after discontinuation of treatment. Good response in the form of relief of symptoms and regression of the mass occurred in 9 cases and there were 2 failures. In 1 case the retroperitoneal mass did not regress and surgical ureterolysis was required. In the 2nd case symptoms recurred after discontinuation of steroid and a further small dose of steroids was required. Function deteriorated in 1 of 19 functioning renal units. No steroid related serious side effects developed. CONCLUSIONS: This regimen of steroid may be used as the primary mode of treatment for the majority of patients with idiopathic retroperitoneal fibrosis with minimal complications. Patients with idiopathic retroperitoneal fibrosis should be followed periodically for the rest of their lives.

An unusual complication of penile prosthesis following urethroplasty.

We report a case of an adult who had undergone transpubic urethroplasty for a 5-cm long posterior urethral stricture. A malleable penile prosthesis (AMS 600R) was implanted 19 months later for the trauma-related impotence. The patient was discovered to develop a complete obliteration of the urethra after removal of infected penile prosthesis 18 months later. Perineal urethroplasty cured his restricture. Suggestions are made to prevent urethral restricture if penile prosthesis is required after urethroplasty.

Prostatic carcinoma: a nutritional disease? Conflicting data from the Kingdom of Saudi Arabia.

PURPOSE: The incidence of prostate cancer in Saudi Arabia has been reported to be low at 1.4 to 2.1/100,000 person-years. We prospectively evaluated the true incidence of this disease and its association with dietary factors. MATERIALS AND METHODS: From 1994 to 1997 inclusive Saudi men older than 50 years treated at our institution for various presenting symptoms and diseases were randomly selected from various departments. They were examined prospectively with digital rectal examination, and total and free prostate specific antigen measurement. Transrectal ultrasound and prostatic biopsy were performed when either test was abnormal. Nutrition questionnaires and detailed interviews with a nutritionist were completed to assess the type of diet, and amount of saturated and polyunsaturated fat consumption of patients with prostatic carcinoma and controls. RESULTS: For the 2,270 Saudi men screened we noted an incidence of 3.1/100,000 person-years. Our nutritional survey revealed that recent fat consumption was greater than 120 gm. per person daily, of which about 40% was from meat and dairy products. Saturated fat comprised about 50% of the total fat intake. There was no difference in the amount of fat in the diet of men with and without prostatic carcinoma. CONCLUSIONS: The incidence of prostatic carcinoma in the Kingdom of Saudi Arabia is low despite a high saturated fat diet in recent years. This finding contradicts most western clinical studies, which indicate a positive association of a high fat diet with prostatic carcinoma.

Construction of a neourethra in girls: follow-up results.

Female urethral injuries may cause serious morbidity with loss of the entire urethra in some cases. We review the results of construction of a neourethra by the flipped anterior bladder-tube technique in five girls aged 3-18 years (mean 8 years). All had pelvic fractures and had their initial management elsewhere; four were run over by motor vehicles and one suffered a pelvic gunshot injury. Three patients came with a suprapubic cystostomy, one was totally incontinent, and another had a urethrovaginal fistula. Three had an associated vaginal injury. All five patients underwent construction of a neourethra using a flipped anterior bladder-wall tube (FABWT) and have been followed for 18-80 months (mean 44 months). All patients were continent in the daytime and three passed urine spontaneously. Two were managed by clean intermittent catheterization (CIC), one per urethram and other via a continent abdominal stoma (Mitrofanoff). The FABWT technique should be considered as an option in the management of females with severe urethral injury.

Feasibility of adrenalectomy with radical nephrectomy.

OBJECTIVES: To evaluate the justification of routine removal of ipsilateral adrenal gland as part of radical nephrectomy for renal cell carcinoma (RCC). METHODS: The medical records, pathologic specimens, and computed tomographic (CT) scans of 77 patients who underwent radical nephrectomy and ipsilateral adrenalectomy for RCC were reviewed. Comparison was made between radiologic analysis and pathologic findings regarding involvement of the adrenal gland. RESULTS: The size of the renal tumor varied between 3.5 and 19 cm (mean 8.5). The upper pole was involved in 45%, the lower pole in 28%, and the midpole in 18% of the patients, and in 9% the whole kidney was involved by the tumor. Histologic findings showed that 72 (94%) of the 77 adrenal glands were normal and 70 of these were normal on CT as well. Two adrenal glands involved by metastases showed heterogeneous contrast entrancement on CT. The benign lesions of three adrenal glands were also picked up as abnormal on CT. In 2 patients adrenal glands could not be visualized on CT because of a paucity of retroperitoneal fat. CONCLUSIONS: Adrenalectomy with nephrectomy may not be performed in patients with RCC in whom CT demonstrates normal adrenal glands. In patients with adrenal abnormality on CT, magnetic resonance imaging can separate metastases from incidental benign adrenal adenoma, further reducing the number of patients requiring removal of the adrenal gland.

Metastatic transitional cell carcinoma of the ovary from superficial bladder tumour.

A 60-year-old female underwent transurethral resection of superficial grade 2 transitional cell carcinoma (TCC) of the urinary bladder. Six months later, intravesical Mitomycin C instillation was given following two superficial recurrences. A further recurrence of TCC grade I was resected transurethrally 39 months later. Forty-eight months after the original diagnosis the patient developed an abdominal mass, which turned out to be TCC grade 3 involving the left ovary. She underwent laparotomy which showed a left ovarian mass. Total abdominal hysterectomy, bilateral oophorectomy and partial omentectomy were carried out. She developed pelvic metastases 3 months later and died.

Sarcomatoid renal cell carcinoma: the chromophobe connection.

Eleven cases of sarcomatoid renal cell carcinoma were studied to determine the relative frequency of various subtypes of renal cell carcinoma that may be associated with sarcomatoid transformation. The epithelial components in these tumors were subcategorized according to established histologic criteria into chromophobe carcinoma (n = 6 cases), clear cell carcinoma (n = 3), papillary carcinoma (n = 1), and indeterminate (n = 1). In nine cases, material was available for immunohistochemical and DNA ploidy studies. The sarcomatoid component in all cases showed positivity for epithelial membrane antigen cytokeratin, indicating an epithelial derivation of these cells. Staining for mesenchymal markers was mostly negative, except for vimentin, which reacted strongly in all cases. DNA ploidy studies using flow cytometry and cell image analysis provided very similar results. Five of five chromophobe sarcomatoid carcinomas showed hypodiploid cell lines in the epithelial areas, whereas the sarcomatoid components mostly showed aneuploid peaks. In the remaining cases, DNA ploidy pattern was more variable. These findings indicate that chromophobe carcinoma may be the most frequent epithelial component associated with sarcomatoid renal cell carcinoma.

Successful treatment of idiopathic retroperitoneal fibrosis with steroids.

Idiopathic retroperitoneal fibrosis (IRF) may cause ureteric obstruction with renal damage. Ureterolysis with intraperitonealization of the ureter is commonly used as primary treatment. We gave corticosteroids for two years to six patients, four males and two females, between 40 and 56 years of age. one of the patients is in his tenth month of treatment and another in the fourth month. Between 4 and 74 months (mean 40.4 months) after initiation of treatment, kidney function had improved, or was preserved in previously functioning renal units, and the patients were free from symptoms. We conclude that steroids may be used as primary treatment of IRF after histological or cytological diagnosis to exclude retroperitoneal malignancy. Steroids should also be part of the treatment strategy in patients with a more aggressive disease who may need surgery. Patients with IRF should be followed for the rest of their lives after discontinuation of steroid therapy.

Delayed management of posterior urethral disruption in children.

OBJECTIVE: To review the results of delayed operative treatment of total disruption of the posterior urethra in children. PATIENTS AND METHODS: Twelve boys (3-12 years, mean 7.5) with a suprapubic catheter were referred after primary management in other hospitals. All but one had been involved in road traffic accidents. In addition to their urethral injury, they all had pelvic fractures and the majority also had other major associated injuries. Six to 14 months after injury, abdominoperineal transpubic urethroplasty was performed in 11 patients according to Turner-Warwick. In one case a perineal anastomotic urethroplasty was performed. RESULTS: After the operation, voiding cysto-urethrography showed a wide anastomosis in all cases. After 3 to 45 months follow-up, there were no strictures. Eight boys were continent, two were totally incontinent, two had stress incontinence and one nocturnal enuresis. All patients with confirmed erections after injury also had erections after their operation. CONCLUSION: Primary suprapubic cystostomy and delayed repair can be used successfully for the treatment of posterior urethral disruption in children.

Xanthogranulomatous pyelonephritis: King Faisal Specialist Hospital experience.

Clinicopathologic features in a series of 11 cases of xanthogranulomatous pyelonephritis are reviewed. There were seven males and four females ranging in age from 6-56 years with an average age of 34 years. The disease was unilateral in ten cases and bilateral in one. There was associated urinary tract obstruction in eight cases due to renal or ureteric stones (5), ureteric stricture (2), and carcinoma of the urinary bladder (1). Two of the patients were renal transplant recipients in which native kidneys were involved by xanthogranulomatous pyelonephritis. In one of these cases, a small renal cell carcinoma was found in one kidney along with amyloidosis involving both kidneys. Radiologic findings were mostly non-specific. These findings are briefly discussed in light of the salient features of this disease as published in the literature.

Polyurethane internal ureteral stents in treatment of stone patients: morbidity related to indwelling times.

The morbidity and complications associated with use of internal polyurethane ureteral stents in a series of 290 stone patients treated endourologically or with extracorporeal shock wave lithotripsy were retrospectively reviewed. Of the 299 stents retrieved 141 were also tested for patency to relate the rate of luminal blockage with stent caliber, indwelling time and clinical evidence of obstruction in the stented tract. Stent indwelling times ranged from a few days to 18 months: 11.3% were indwelling longer than 6 months and 1.9% were lost to followup. Incrustation occurred in 9.2% of the stents retrieved before 6 weeks, 47.5% indwelling 6 to 12 weeks and 76.3% thereafter. In 19 cases over-all (6.4%) an auxiliary procedure was required to decrease incrusted stone burden and enable stent retrieval. Other complications included stent migration (3.7%), infection (6.7%) and breakage (0.3%). Despite a 30% rate of luminal blockage in stents retrieved after indwelling times up to 3 months, the incidence of clinical obstruction in stented tracts up to 3 months was 4%, confirming other reports that significant urine flow occurs around rather than through hollow, vented stents. Our findings underline the importance of restricting the use of stents to stone patients who will be reliable at followup. Morbidity was minimal if stent indwelling times did not exceed 6 weeks.