| Literature DB >> 28420321 |
Marie-Anne Brundler1,2,3, Kyle C Kurek2, Kamlesh Patel4, Ingo Jester4.
Abstract
Lipoblastoma is a benign adipose tumor typically presenting in infancy in superficial soft tissues of extremities. Intestinal complications secondary to intraabdominal or retroperitoneal involvement are exceedingly rare. We describe a unique case of a primary intestinal lipoblastoma arising from the submucosa of the transverse colon in an otherwise healthy 18-month-old boy. He presented with a history of reducible rectal prolapse, rectal bleeding, and episodic abdominal pain and was initially treated for constipation. Imaging identified a short colo-colonic intussusception, confirmed at laparotomy, and a fatty mass thought to arise from the mesentery. Pathological examination of the resected transverse colon revealed a submucosal tumor composed of a mixture of mature adipose tissue, foci of myxoid mesenchymal tissue with desmin positive, HMGA2 negative spindle cells, and scattered lipoblasts, characteristic of lipoblastoma. Lipoblastoma should be considered as a potential albeit rare cause of intussusception in young children, where a pathologic lead point is infrequently identified.Entities:
Keywords: PLAG1; colon; immunohistochemistry; intussusception; lipoblastoma; pathological lead point; pediatric
Mesh:
Year: 2017 PMID: 28420321 DOI: 10.1177/1093526617705271
Source DB: PubMed Journal: Pediatr Dev Pathol ISSN: 1093-5266