A ganglioneuroma of the sigmoid colon presenting as leading point of intussusception in a child: a case report

Department of Paediatric Surgery, University Hospitals of Leicester, United Kingdom.
Journal of Pediatric Surgery (Impact Factor: 1.31). 02/2009; 44(1):e17-20. DOI: 10.1016/j.jpedsurg.2008.10.037
Source: PubMed

ABSTRACT We present a case of intestinal ganglioneuroma (GN) of the sigmoid colon in a 5-year-old girl, which caused intermittent colocolic intussusception. Ganglioneuromas are rare benign tumors of the autonomic nervous system composed of mature ganglion cells and satellite cells. Colonic GNs are uncommon. The unusual intramural proliferation of neural elements in this case resembled the diffuse intestinal ganglioneuromatosis, which is known to be associated with multiple endocrine neoplasia type 2B. However, the specific mutations of multiple endocrine neoplasia type 2B were not found by genetic sequencing. This is the first pediatric case described in the literature of a solitary polypoid GN presenting as a colocolic intussusception. We present a brief overview of intestinal ganglioneuromatous lesions and associated conditions.

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