2000 Volume 33 Issue 6 Pages 740-744
It has been reported that desmoid tumors usually develop in the abdominal wall or cavity after prophy-lactic colectomy in most cases of Gardner's syndrome. We describe a rare case of desmoid tumor that devel-oped in the submandibular region in a young female with Gardner's syndrome. A 14-year-old woman came to our hospital with a complaint of lockjaw caused by a giant tumor in the submandibular region. Magnetic reso-nance imaging showed that the mass, 11cm×10cm in size, was a hypointense area on the T1W1 image and slightly hyperintense area on the T2W1 image. It pressed against the pharynx and larynx. Colonoscopic ex-amination revealed five small polyps in the colon. Because the tumors adhered to the surrounding organs through rigid connective tissue and bones, complete extirpation of the tumor was impossible, and the patient was treated with sulindac postoperatively. The tumor was diagnosed as a desmoid tumor by pathological ex-amination. The patient's lockjaw has been gradually improving since the operation. Colon cancer and de-smoid tumor are the most common causes of death in Gardner's syndrome. The number and size of adenomas and desmoid tumors have been reported to be reduced by sulindac therapy. Sulindac chemoprevention is im-portant for managing patients with polyposis and unresectable desmoid tumors.
