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

详细信息    查看全文

• 作者：Giampiero Soccorso ; Florian Puls ; Cathy Richards ; Howard Pringle ; Shawqui Nour
• 关键词：Colocolic intussusception ; Ganglioneuroma
• 刊名：Journal of Pediatric Surgery
• 出版年：2009
• 出版时间：January 2009
• 年：2009
• 卷：44
• 期：1
• 页码：e17-e20
• 全文大小：572 K

文摘

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.