Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A cystectomy for indications other than transitional cell cancer of the bladder or general bladder cancer is frequently performed in cooperation with other surgical specialties such as general surgery or gynecology. In these cases the urological procedure as well as the oncological and surgical concepts of other specialties have to be combined. We studied our cystectomy patients who had undergone such a combined procedure for a non-urothelial indication concerning perioperative and postoperative complications.A total of 204 cystectomies were performed by the Department of Urology at the University of Hamburg, Germany between 1995 and 2003. Bladder cancer was the indication for cystectomy in 162 patients, but 42 patients had a non-urothelial indication for this procedure. These patients included 12 cases of advanced rectal cancer, 9 cases of advanced cervical cancer, 6 cases of advanced sigmoid cancer, 4 cases of advanced prostate cancer, 1 case of prostate sarcoma, 5 cases of complex vesicointestinal fistulae, 2 cases of urachal cancer, 1 leiomyosarcoma, 1 rhabdomyoma, and 1 rhabdomyosarcoma, respectively. Perioperative and postoperative complications of those patients were compared to patients who underwent cystectomy for transitional cell cancer of the bladder.Those 42 patients who underwent cystectomy for non-urothelial indications included 14 male and 28 female patients. The mean age was 58.2 years with a range of 3-78 years. For urinary diversion 30 ileum conduits, 4 sigma conduits, and 8 ileum neobladders were used. The mean operative time was 6.25 h. The mean blood loss was 2200 ml. An average of four red blood cell concentrates (RBC) had to be given. Postoperative hydronephrosis had to be treated in three (7%) patients unilaterally and in two (5%) patients bilaterally with a temporary nephrostomy. Postoperative urinary leakage lasting more than 30 days was found in two (5%) patients. A deep vein thrombosis as well as an ileus was found in five (12%) patients each, respectively. There was no perioperative mortality in this study. When comparing the complications of those patients with the 162 patients who underwent cystectomy for bladder cancer, the only significant difference ( p=0.033, chi-square test) was a higher ileus rate in the patients with cystectomy for a non-urothelial indication. Complications with cystectomy for non-urothelial indications are in large comparable to those for transitional cell carcinoma of the bladder. The higher ileus rate in non-urothelial patients can be explained by the more radical procedures in this group of patients. Even though the group of patients undergoing cystectomy for indications other than bladder cancer was small in this trial, the procedure is standardized in combination with other specialties. Larger patient numbers and a longer follow-up will lead to more data in this special group of patients.
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PMID:[Cystectomy for indications other than bladder cancer]. 1499 Nov 19

Costello syndrome is a rare congenital anomaly syndrome with a predisposition to specific tumors, including neuroblastoma, rhabdomyosarcoma, and transitional cell carcinoma of the bladder. The increased risk for solid tumors led to the proposal of a tumor screening protocol. A screening test for neuroblastoma consists of measuring catecholamine metabolites in urine, an assay that may also be used for diagnostic confirmation of a suspected catecholamine secreting tumor. We report eight patients with Costello syndrome with elevated catecholamine metabolites, vanillylmandelic acid (VMA) and/or homovanillic acid (HVA), in urine. Each patient had additional laboratory and/or imaging studies. None of the patients was found to have a neuroblastoma or another catecholamine secreting tumor. In two cases, the assays were performed because the patients were symptomatic with diaphoresis and hypertension, respectively, and in the other six cases the assays were performed in order to screen for neuroblastoma. The pathophysiology for the catecholamine metabolite abnormality in these patients with Costello syndrome remains unclear. However, it appears that in this patient group an elevation above the normal limit, defined as 2 standard deviations (SD) above the mean for age, is more likely to be a variant, rather than a sign of a neuroblastoma. Thus, it may be prudent not to use this assay as a screening test, and to take the frequently elevated results into consideration when interpreting diagnostic assays.
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PMID:Elevated catecholamine metabolites in patients with Costello syndrome. 1521 56