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PubMed · 16148644

Polyps in the exstrophic bladder. A cause for concern?

Abstract

PURPOSE: The role of environmental injury in carcinogenesis is widely recognized. Malignancy in exstrophic bladders has been reported most frequently in untreated adults and those undergoing surgical treatments which involve the mixing of fecal and urinary streams. The question of whether the closed exstrophic bladder has a similar potential for malignancy has not been resolved. The polypoid appearance of the exstrophic bladder template raises the concern of premalignant lesions. We characterized the histology of these lesions and analyzed their microscopic features with particular reference to predisposition for dysplasia. In doing so, we attempt to address the aforementioned question and set the stage for definitive quantification of the risk of malignancy in these patients with careful, long-term followup. MATERIALS AND METHODS: Under institutional board review, the slides of 38 patients with classic bladder exstrophy who had polyps excised at the time of closure were reviewed by a single genitourinary pathologist (JIE). The most common findings were reported for polyps resected at primary and secondary closure, respectively, and a comparative analysis was performed. RESULTS: Of the 38 cases 24 were primary closures and 14 were secondary closures. Six of the primary closures were delayed by 6 weeks or greater. The 2 basic types of polyps observed were fibrotic and edematous. Both types were associated with overlying reactive squamous metaplasia in approximately 50% of cases. Varying degrees of fixed on file Brunn's nests, cystitis cystica and cystitis glandularis were noted. Cystitis glandularis was observed in a significantly greater percentage of secondary closures (p = 0.0014). CONCLUSIONS: Although no dysplasia was noted, cystitis glandularis is associated with the development of adenocarcinoma of the bladder. The finding of cystitis glandularis suggests a more severe epithelial injury and it follows that the significant majority of these cases (10 of 14, 71.4%) were observed with polyps resected during secondary closure. These patients warrant future surveillance with urine cytology and cystoscopy as they enter adult life.

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BibTeXRIS

T E Novak, Y Lakshmanan, D Frimberger, J I Epstein, J P Gearhart. 2005. Polyps in the exstrophic bladder. A cause for concern?. https://doi.org/10.1097/01.ju.0000179240.25781.1b

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MTHFR 677 TT genotype in a mother and her child with Down syndrome, atrioventricular canal and exstrophy of the bladder: implications of a mutual genetic risk factor?

Apart from Husmann and Vandersteen [in: Gearhart JP, Matthews R (eds) The Epispadias-Exstrophy Complex. Kluwer, New York, pp 199-206, 1999], we report only the second case of Down syndrome (DS) associated with exstrophy of the bladder (EB). Besides the appearance of DS, the newborn exhibited a complete atrioventricular canal (CAVC) and classical EB, including diastases of the symphysis, an epispadic penis and an open bladder plate. Despite current recommendations, the mother had not supplemented her intake of folic acid during the periconceptional period. In a comparable case, Al-Gazali et al. (Am J Med Genet 103:128-132, 2001) found the homozygous 677T allele of the methylenetetrahydrofolate (MTHFR) gene 677C-->T polymorphism in a mother and her child with DS and cervical meningomyelocele. They found that the mother, who also had not supplemented her folic acid intake, had a secondarily altered folate status with an increased homocysteine level, suggesting that the homozygous TT mutation in the MTHFR gene in both mother and her child had contributed to the presentation of DS and a neural tube defect. The combined clinical findings of the present case and the observations of Al-Gazali et al. led us to investigate the 677C-->T polymorphism in our mother-child pair. Likewise we found that mother and child were homozygous for the mutant 677T allele. Our findings support the suggestion of Al-Gazali et al. that the MTHFR 677TT could be a mutual genetic risk factor for the co-occurrence of trisomy 21 and midline defects, the risk of which may be reduced by periconceptional folic acid supplementation.

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