Gardner syndrome. Early presentation with a desmoid tumor. Discovery of multiple colonic polyps.

Department of Pediatrics, New York Medical College, Metropolitan Hospital Center, New York, New York 10029.

A 14-year-old patient who was eventually found to have Gardner syndrome initially presented at the age of 3 years with a desmoid tumor involving the scalp. A careful review of the family history revealed a high incidence of colonic cancer, which prompted endoscopic evaluation of the patient. The discovery of adenomatous polyps in the colon confirmed the diagnosis of Gardner syndrome. In patients with hard or soft tissue tumors, the possibility of Gardner syndrome should be kept in mind, and a thorough family history taken. Early diagnosis may prevent malignant transformation of colonic polyps.

PMID: 2805561 [PubMed - indexed for MEDLINE]