Laparoscopic resection of a pediatric intestinal leiomyosarcoma

Intestinal leiomyosarcomas represent an exceptionally rare tumour in children. We report a 15-year-old female who presented with a long history of symptomatic iron deficiency anemia. Multiple investigations failed to lead to a diagnosis, however, a small bowel video capsule endoscopy demonstrated an ileal polyp. She proceeded to surgery where an ileo-ileal intussusception was found and underwent a small bowel resection. Histopathology confirmed a well-differentiated leiomyosarcoma that stained positive for smooth muscle actin and negative for c-kit, differentiating it from a gastrointestinal stromal tumour (GIST). This case becomes only the fourth reported case in the 21st century. The authors explore the current literature on this tumour in the pediatric population and how in the last two decades the discovery of specific immunohistochemistry staining resulted in most intestinal leiomyosarcoma being classified correctly as GISTs.