Gardner's Syndrome Associated with Giant Desumoid Tumor in the Submandibular Region, Report of a Case

Masanori Nishioka, Masashi Ishikawa, Norikazu Hanaki, Toru Kikutsuji, Yutaka Kashiwagi and Hisashi Miki

Department of Surgery, National Kochi Hospital

It has been reported that desmoid tumors usually develop in the abdominal wall or cavity after prophylactic colectomy in most cases of Gardner's syndrome. We describe a rare case of desmoid tumor that developed 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 resonance imaging showed that the mass, 11 cm× 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 examination 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 examination. The patient's lockjaw has been gradually improving since the operation. Colon cancer and desmoid 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 important for managing patients with polyposis and unresectable desmoid tumors.

Key words
Gardner's syndrome, desmoid tumor, sulindac

Reprint requests
Masanori Nishioka Department of Surgery, National Kochi Hospital2-25 Asakuranishimachi, Kochi City, 780-8507 JAPAN

Accepted
February 23, 2000

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