Bannayan-Riley-Ruvalcaba syndrome: spectrum of intestinal pathology including juvenile polyps.
Pediatr Dev Pathol
; 3(2): 155-61, 2000.
Article
em En
| MEDLINE
| ID: mdl-10679034
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.
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Coleções:
01-internacional
Temas:
Geral
/
Prevencao_e_fatores_de_risco
/
Hereditariedade
/
Tipos_de_cancer
/
Colon_e_reto
Base de dados:
MEDLINE
Assunto principal:
Síndrome do Hamartoma Múltiplo
/
Polipose Adenomatosa do Colo
/
Neoplasias Intestinais
Tipo de estudo:
Diagnostic_studies
Limite:
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Pediatr Dev Pathol
Assunto da revista:
PATOLOGIA
/
PEDIATRIA
Ano de publicação:
2000
Tipo de documento:
Article
País de afiliação:
Estados Unidos