DICER1-related gastrointestinal polyps

Polyps in the small intestine have been described in children with PPB and/or DICER1 variation. Generally, these have been seen in individuals from birth to 10 years of age. This can result in intestinal obstruction, and surgery may be required.

Presenting symptoms

Intestinal polyps may present with abdominal pain, constipation, bloody stool and may result in intestinal obstruction.

Treatment

When intestinal obstruction occurs, surgery may be required.

Observation

No systematic imaging studies or endoscopy are indicated in asymptomatic individuals, but families and health care providers should be aware of the risk, albeit low, for intestinal obstruction and the need for imaging evaluation and/or endoscopy if clinically indicated.

Contact us

The International Pleuropulmonary Blastoma / DICER1 Registry
910 East 26th Street, Suite 40-LLO8
Minneapolis, MN 55404

How you can help find a cure

We can find a cure for PPB, SLCT and other DICER1-related cancers if we all work together. This includes clinicians, pathologists, geneticists, radiologists, laboratory scientists, coordinators, philanthropic volunteers and donors—and most of all, patients and families. Each research advancement in this area has been made possible by the generous sharing of clinical information and tissue and blood samples from individuals and/or their family members.

To contribute clinical information and/or blood/tumor samples, please consider enrollment in The International PPB/IDCER1 Registry.