Desmoid Tumors and Gardner's Syndrome

Abdominal desmoid tumors account for the majority of intraabdominal (inside the abdominal cavity) tumors leading to an intestinal transplant. Although desmoid tumors are benign (not harmful) tumors, they are infiltrative (grow into healthy tissue) and locally invasive in nature, entrap the mesenteric vasculature, and can lead to obstructions and fistulas. Desmoid tumors are generally unresponsive to normal chemotherapeutic agents, thus complete surgical removal of the intestine is often required. This leaves the patient with a short bowel that is insufficient to support your nutritional needs. (1)


Desmoid tumors are commonly associated with Gardner’s syndrome. Gardner’s syndrome is a form of familial adenomatous polyposis (FAP) that is characterized by multiple colorectal polyps and various types of tumors, both benign (not harmful) and malignant (harmful). Individuals with Gardner’s will commonly contract different types of tumors, such as osteomas (bone tumor), fibromas (tumor of connective tissue), lipomas (tumor of fat tissue), and desmoid tumors (connective tissue).


It is not uncommon for Gardner’s patients to undergo a total proctocolectomy (removal of colon, rectum, and anus) to treat the primary disease and prevent progression to cancer. Apart from a total protocolectomy, resections for desmoid tumors in the intestine can be performed, but multiple resections can lead to short bowel syndrome and insufficient absorption. Small bowel desmoids may also be treated with radiation, which can lead to radiation enteritis in the remaining bowel and impede absorption. 


The infiltrative nature of the desmoid tumors and the extensive resection they require will often place the patient in the position to be a candidate for intestinal and/or multivisceral transplant evaluation.



Reference:

  1.  Matsumoto CS, Subramanian S, Fishbein TM. Adult Intestinal Transplantation. Gastroenterology Clinics of North America. 2018;47(2):341-354. doi:10.1016.



Desmoid Tumors and Gardner's Syndrome