Abstract

MONSANTO, Pedro et al. Intestinal diffuse ganglioneuromatosis associated with small bowel adenocarcinomas. J Port Gastrenterol. [online]. 2013, vol.20, n.2, pp.83-88. ISSN 0872-8178.  https://doi.org/10.1016/j.jpg.2012.04.036.

Intestinal ganglioneuromatosis is included in the syndrome of non-hereditary hamartomatous polyposis. It is a rare disorder of the enteric nervous system, characterized by a diffuse hyperplasia of the neural plexus of the intestinal wall. Usually it manifests in the form of constipation or diarrhea associated with abdominal distension and pain. It is often associated with neurofibromatosis type 1 and multiple endocrine neoplasia type 2b and may be one of its first manifestations. More rarely it can present in a sporadic and isolated form. Although regarded as a benign lesion, it can be associated with neoplastic degeneration of the intestinal epithelium. In the present case we describe a diffuse intestinal ganglioneuromatosis of small bowel associated with carcinomas, very rarely described in literature.

Keywords : Intestinal ganglioneuromatosis; Intestinal Adenocarcinoma; Small Bowel; Endoscopy.

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