Causes of death in patients with familial polyposis coli (FPC)

Autopsy reports from patients with familial polyposis coli were obtained from the Roswell Park Memorial Institute Registry as well as clinical data on patients with familial polyposis coli with regard to their causes of death. Although the latter group did not undergo autopsy they were the subject of a close follow-up. From this data it is clear that diagnosis at an early age may not diminish the chances of the patient with familial polyposis to develop cancer. Removal of the colonic mucosa does not provide absolute assurance that patients with this condition will be cured, as these patients remain at considerable risk for developing other benign and malignant lesions of the gastrointestinal tract and perhaps also of other organs. Close follow-up of FPC patients who have had surgery for colorectal polyps or cancer is necessary. Other proliferative abnormalities of FPC patients include an abnormal fibroblastic response, tendency to develop adhesions, and the propensity for developing desmoids. Desmoids may follow a slow but unrelenting course with a lethal outcome. Autopsy findings support the view that there should be no distinction between familial polyposis coli and Gardner's syndrome.