Submucosal Colonic Lipoblastoma Presenting With Colo-colonic Intussusception in an Infant. (July 2018)

• Record Type: Journal Article
• Authors: Brundler, Marie-Anne; Kurek, Kyle C; Patel, Kamlesh; Jester, Ingo
• Is Part Of: Pediatric and developmental pathology. Volume 21:Number 4(2018)
• Page Start: 401
• Page End: 405
• Publication Date: 2018-07
• Subjects: lipoblastoma -- PLAG1 -- immunohistochemistry -- intussusception -- pathological lead point -- pediatric -- colon; Pediatric pathology -- Periodicals; Children -- Diseases -- Periodicals; Diagnosis, Laboratory -- Periodicals; Abnormalities, Human -- Periodicals; Child development -- Periodicals; Pediatrics -- Periodicals; 616.07
• DOI: 10.1177/1093526617705271
• Languages: English
• ISSNs: 1093-5266

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.