Document Detail

Bannayan-Riley-Ruvalcaba syndrome: spectrum of intestinal pathology including juvenile polyps.
MedLine Citation:
PMID:  10679034     Owner:  NLM     Status:  MEDLINE    
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.
A Lowichik; F V White; C F Timmons; A G Weinberg; T S Gunasekaran; K Nathan; C M Coffin
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  3     ISSN:  1093-5266     ISO Abbreviation:  Pediatr. Dev. Pathol.     Publication Date:    2000 Mar-Apr
Date Detail:
Created Date:  2000-03-17     Completed Date:  2000-03-17     Revised Date:  2006-05-08    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  155-61     Citation Subset:  IM    
Division of Pediatric Pathology, University of Utah School of Medicine and Primary Children's Medical Center, Department of Pathology, 100 North Medical Drive, Salt Lake City, UT 84113, USA.
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MeSH Terms
Adenomatous Polyposis Coli / pathology*
Diagnosis, Differential
Hamartoma Syndrome, Multiple / pathology*
Intestinal Diseases / pathology
Intestinal Neoplasms / pathology*

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