Augusto Lauro, Chiara Zanfi, Sara Pellegrini, Fausto Catena, Loris Pironi, Antonio Pinna

S. Orsola Hospital - Liver and Multiorgan Transplant Center, Bologna, Italy

Aim: Patients affected by Gardner’s Syndrome are subjected to death at young age, usually after multiple abdominal surgeries. Nowadays alternative therapies other than surgery are uneffective and abdominal evisceration followed by TPN seems to be the mean for a long term survival. Unfortunately recent studies showned a lower survival of these patients under TPN treatment comparing to standard TPN- patients. Transplantation seems to be an alternative. We review our experience regarding intestinal transplantation for Gardner’s Syndrome.

Subjects and Methods: Average waiting  time in our list for transplantation was 259 days: we registered 1 death in list before transplant. Actually we have in list  5 patients for Gardner’s syndrome, the oldest since 2005. Eleven patients were transplanted in our center: 9 were on TPN at time of transplant;when evisceration was performed , average size of  desmoid tumors was greater than 10 cm in 9 patients (in two remaining cases the procedure was performed elsewhere).

Results: Average age at transplant was 34 years. Five patients were transplanted with a multivisceral graft, 6 with isolated small bowel: immunosuppression was represented by FK regimen plus induction with Alemtuzumab in 10 cases, 1 case was treated with Daclizumab. Two patients survived up to now: causes of death were: infections in 7 cases, tumor recurrence in 1 case and intraoperative death in last case. Considering the 9 deaths, 5  patients received an isolated graft while 4 a multivisceral one  (one recipient with liver), one of them was a re-transplant. Average survival after transplant is of 449 days. One survived patient has a desmoid intra-abdominal recurrence.

Conclusions: An early referral for a life-saving intestinal/multivisceral transplant should be considered for patients with desmoids: patients should be referred for listing to transplant centers at time of diagnosis, before TPN and  growth of invasive intra-abdominal desmoids tumors.