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Familial Adenomatous Polyposis (FAP) and its phenotype variant, Gardner's syndrome, constitute a rare autosomal dominant inherited disorder. They are characterised by the development, generally during the second and third decades of life, of multiple adenomatous polyps in the colon and rectum. These polyps have a high risk of subsequently becoming malignant, which normally occurs in the third and fourth decades of life. The phenotypical features of FAP can be very variable. As well as colorectal polyps, these individuals can present with extra-colonic symptoms, among which are particularly: gastro-duodenal polyps, dermoid and epidermoid cysts, desmoid tumours, congenital hypertrophy of the retinal pigment epithelium, disorders of the maxillary and skeletal bones and dental anomalies. In this paper the most important aspects of this syndrome are reviewed, showing an example based on a well documented clinical case. The importance of odonto-stomatological examinations should be pointed out, among others, as a means of reaching a presumptive diagnosis, whose confirmation is vital to the patient.

An intra-abdominal desmoid tumour included in Gardner's syndrome is described. Some aspects of this unusual tumour are reviewed, emphasizing the value of CT to evaluate shape, location, size and relation with adjacent structures and therefore to decide the therapeutic plan, to avoid the need for exploratory laparotomies which are, moreover, so inadvisable in these patients. Although the, treatment of choice is surgery, it must be performed in accordance with CT findings. When surgery is not possible is incomplete or after recurrences, alternative methods are recommended.

Gardner's Syndrome comprises numerous pathological entities, one of which is the intraabdominal desmoid tumour which can provoke obstructive uropathy through ureteral trapping. Contribution of one case treated with resection of the trapped ureter, reanastomosis and plasty with peritoneal flap. Discussion of the diagnosis, evolution, and the different therapeutical choices found in the literature.

Desmoid tumours are benign neoplasias manifesting themselves clinically as aggressive fibromatoses. They may be sited both in mesenteric and retroperitoneal fat. Owing to the great size they reach and to their local infiltrative character they occasionally lead to the development of intestinal occlusive syndromes and, in exceptional cases, to obstructive uropathy of the upper urinary tract. Their incidence of presentation in the general population is low, however they develop more frequently in patients suffering from colonic polyposis in the family and they form part of the extracolonic manifestations of Gardner's syndrome. In 1987 we treated a young patient, a genetic carrier of familiar colonic polyposis who had presented relapse of a previous desmoid. The location of the tumour in narrow pelvis made its clinical debut as a intestinal pseudo-occlusion and determined a bilateral ureteral obstruction and the formation of a major urinoma. The joint utilization of instrumental maneuvers and surgical approaches enabled us to solve the intestinal and urological commitment satisfactorily. We carry out an overall review and analyse the data published on the incidence, prognosis and treatment of obstructive uropathy in Gardner's syndrome. We also carry out an updating on the biology of the desmoid tumour.