Multiple polyposis, gardner's syndrome and desmoid tumors

Summary and Conclusions A familial syndrome recently has been described by Gardner which in some respects bears the same relationship to multiple polyposis of the colon that the Peutz-Jeghers syndrome does to polyposis of the small intestine. In addition to familial multiple polyposis, the salient features of the syndrome are (1) multiple epidermoid cystomatosis and (2) a tendency toward proliferation of cells of mesenchymal origin, resulting in the formation of osteomas, fibromas and desmoid tumors. The epidermoid cysts and osteomas produce definite external deformities which may lead the physician to suspect multiple polyposis of the colon. In this study, the records of 201 patients with multiple polyposis were reviewed. Seventeen of the 201 patients were found to exhibit one or more of the abnormalities listed above. Eight of these 17 patients were studied intensively with Gardner's syndrome in mind. Six of the 17 patients had desmoid tumors associated with their abdominal incisional scars, and an additional patient had an extra-abdominal desmoid tumor. Epidermoid cysts were found in 12, and osteomas in eight, of the 17 patients reported upon. It is felt that Gardner's syndrome is the embodiment of a spectrum of pathologic changes which could affect, in variable numbers and combinations, any patient who has multiple polyposis.