Intestinal transplantation for the treatment of desmoid tumors associated with familial adenomatous polyposis.
BACKGROUND
Desmoid tumors associated with familial adenomatous polyposis (FAP) are locally invasive. Often occurring in the mesentery of the intestine, they sometimes recur after resection. Complications can include intestinal failure and dependence on parenteral nutrition. We describe 9 patients who underwent intestinal transplantation for the treatment of desmoid tumors associated with FAP.


METHODS
Records of patients undergoing intestinal transplantation for desmoid tumors at 2 transplant centers were reviewed for patient age, sex, type of graft, procedure date, tumor site, desmoid complications, medications, extracolonic manifestations, status at follow-up, and length of survival.


RESULTS
Nine patients with FAP and intestinal failure caused by desmoid tumors were treated with isolated intestinal (n = 6), multivisceral (n = 2), or combined liver-intestinal transplantation (n = 1). Desmoid tumors recurred in the abdominal walls of 2 patients. Two patients died: one as a result of sepsis, the other because of a rupture of a mycotic aneurysm of the aortic anastomosis. One graft lost to severe rejection was replaced with a second intestinal graft. Eleven to 53 months after transplantation, 7 patients were alive, well, independent of parenteral treatment, and leading apparently normal lifestyles.


CONCLUSIONS
Transplantation of the intestine alone or as part of a multivisceral transplantation may help rescue otherwise untreatable patients with complicated desmoid tumors.
Chatzipetrou
M A
MA
Department of Surgery, Division of Liver and GI Transplant, University of Miami School of Medicine, FL 33136, USA.
Tzakis
A G
AG
Pinna
A D
AD
Kato
T
T
Misiakos
E P
EP
Tsaroucha
A K
AK
Weppler
D
D
Ruiz
P
P
Berho
M
M
Fishbein
T
T
Conn
H O
HO
Ricordi
C
C
D011125:Adenomatous Polyposis Coli / Q000150:complications*; D000328:Adult; D005260:Female; D005350:Fibroma / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D006084:Graft Rejection / Q000601:surgery; D006801:Humans; D007422:Intestines / Q000637:transplantation*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D012086:Reoperation; D016019:Survival Analysis; D014781:Viscera / Q000637:transplantation