Desmoid Tumors for Medical Professionals

Abdominal desmoid tumors account for the majority of intraabdominal tumors leading to intestinal transplant. Although desmoid tumors are benign tumors, they are infiltrative and locally invasive in nature, entrap the mesenteric vasculature, and can lead to obstructions and fistulas. Desmoid tumors are generally unresponsive to conventional chemotherapeutic agents, thus complete surgical resection of the intestine is often required. This leaves the patient with a short bowel that is insufficient to support 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 and malignant. Individuals with Gardner’s will commonly contract osteomas, fibromas, lipomas, and desmoid tumors. It is not uncommon for Gardner’s patients to have undergone total proctocolectomy to treat the primary disease and prevent progression to cancer. 


In addition to other resections for desmoids in the intestine, this increases the risks for malabsorption after small bowel resection. Also, small bowel desmoids may 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 a patient in the position to be a good candidate for intestinal and/or multivisceral transplant evaluation.



References:

  1.  Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral transplantation. Gastroenterology Report. 2017;5(1):20-28. doi:10.1093/gastro/gow045.