ISSN:
1615-5742
Keywords:
Key words: Bannayan-Riley-Ruvalcaba syndrome, juvenile polyposis, ectopic ganglion cells, atypical polyps
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
ABSTRACT Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a disorder that includes juvenile polyposis as part of its pathologic spectrum, and it recently has been shown to share phenotypic and genotypic features with Cowden's disease. In existing literature, descriptions of intestinal pathology in patients with BRRS are relatively sparse and occasionally erroneous. We describe the intestinal pathology in multiple specimens from three children with BRRS. Examination of gastrointestinal biopsies from these children revealed predominantly colonic and rectal polyps with the histology of juvenile polyps. Additionally, two cases with clusters of ectopic ganglion cells within the lamina propria, one in a colonic polyp and one in a duodenal biopsy, and an atypical polyp were observed. Bannayan-Riley-Ruvalcaba syndrome should be included in the list of differential diagnostic considerations when a child or young adult presents with a juvenile polyp, particularly if unusual histologic features such as atypical polyps or ectopic ganglion cells are encountered.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s100249910016
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