Submucosal Colonic Lipoblastoma Presenting With Colo-colonic Intussusception in an Infant

Pediatr Dev Pathol. 2018 Jul-Aug;21(4):401-405. doi: 10.1177/1093526617705271. Epub 2017 Apr 18.

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.

Keywords: PLAG1; colon; immunohistochemistry; intussusception; lipoblastoma; pathological lead point; pediatric.

Publication types

  • Case Reports

MeSH terms

  • Colonic Neoplasms / complications
  • Colonic Neoplasms / diagnosis*
  • Colonic Neoplasms / pathology
  • Humans
  • Infant
  • Intestinal Mucosa / pathology
  • Intussusception / diagnosis
  • Intussusception / etiology*
  • Lipoblastoma / complications
  • Lipoblastoma / diagnosis*
  • Lipoblastoma / pathology
  • Male