Advertisement
Original Communications| Volume 129, ISSUE 3, P277-281, March 2001

Intestinal transplantation for the treatment of desmoid tumors associated with familial adenomatous polyposis

      Abstract

      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. (Surgery 2001;129:277-81.)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Farmer KCR
        • Hawley PR
        • Phillips RKS.
        Desmoid disease.
        in: Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London1994: 128-142
        • McAdam WAF
        • Goligher JC.
        The occurrence of desmoids in patients with familial polyposis coli.
        Br J Surg. 1970; 57: 618-631
        • Jones IT
        • Fazio VW
        • Weakley FL
        • Jagelman DG
        • Lavery IC
        • McGannon E.
        Desmoid tumors in familial polyposis coli.
        Ann Surg. 1986; 204: 94-97
        • Anthony T
        • Rodríguez-Bigas MA
        • Weber TK
        • Petrelli NJ
        Desmoid tumors.
        J Am Coll Surg. 1996; 182: 369-377
        • Doi K
        • Iida M
        • Kohrogi N
        • Mibu R
        • Onitsuka H
        • Yao T.
        Large intra-abdominal desmoid tumors in a patient with familial adenomatosis coli: their rapid growth detected by computerized tomography.
        Am J Gastroenterol. 1993; 88: 595-598
        • Rodriguez-Bigas MA
        • Mahoney MC
        • Karakousis CP
        • Petrelli NJ.
        Desmoid tumors in patients with familial adenomatous polyposis.
        Cancer. 1994; 74: 1270-1274
        • Arvanitis ML
        • Jagelman DG
        • Fazio VW
        • Lavery IC
        • McGannon E.
        Mortality in patients with familial adenomatous polyposis.
        Dis Colon Rectum. 1990; 33: 639-642
        • Häyry P
        • Scheinin TM
        The desmoid (Reitamo) syndrome: etiology, manifestations, pathogenesis, and treatment.
        Curr Probl Surg. 1988; 25: 232-320
        • Grant D.
        Intestinal transplantation: 1997 report of the international registry.
        Transplantation. 1999; 67: 1061-1064
        • Misiakos EP
        • Pinna A
        • Kato T
        • Rodriguez MG
        • Francavilla A
        • Mazzaferro V
        • et al.
        Recurrence of desmoid tumor in a multivisceral transplant patient with Gardner's syndrome.
        Transplantation. 1999; 67: 1197-1199
        • Seiter K
        • Kemeny N.
        Successful treatment of a desmoid tumor with doxorubicin.
        Cancer. 1993; 71: 2242-2244
        • Schnitzler M
        • Cohen Z
        • Blackstein M
        • Berk T
        • Gallinger S
        • Mablensky L
        • et al.
        Chemotherapy for desmoid tumors in association with familial adenomatous polyposis.
        Dis Colon Rectum. 1997; 40: 798-801
        • Klein WA
        • Millie HH
        • Anderson M
        • DeCosse JJ.
        The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis.
        Cancer. 1987; 60: 2863-2868
        • Waddel WR
        • Kirsch WM.
        Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors.
        Am J Surg. 1991; 161: 416-421
        • Calne RY
        • Pollard SG
        • Jamieson NV
        • Friend PJ
        • Rasmussen A
        • Neale G
        • et al.
        Intestinal transplant for recurring mesenteric desmoid tumor [letter].
        Lancet. 1993; 342: 58-59
        • Morris JA
        • Johnson DL
        • Rimmer JAP
        • Kuo PC
        • Alfrey EJ
        • Bastidas JA
        • et al.
        Identical-twin small bowel transplant for desmoid tumour [letter].
        Lancet. 1995; 345: 1577-1578
        • Chan WK
        • Friend PJ
        • Jamieson NV
        • Caine RY
        Multivisceral grafting for Gardner's syndrome.
        J Wound Care. 1995; 4: 214-216
        • Baliga P
        • Reuben A
        • Baker R
        • Baker S
        • Cofer J
        • Rajagopalan P.
        Intestinal transplantation: an early experience.
        Transplant Proc. 1996; 28: 2734-2735
        • Jaffe BM
        • Beck R
        • Flint L
        • Gutnisky G
        • Haque S
        • Lambiase L
        • et al.
        Living-related small bowel transplantation in adults: a report of two patients.
        Transplant Proc. 1997; 29: 1851-1852
        • Todo S
        • Reyes J
        • Furukawa H
        • Abu-Elmagd K
        • Randal GL
        • Tzakis A
        • et al.
        Outcome analysis of 71 clinical intestinal transplantations.
        Ann Surg. 1995; 222: 270-282
        • Starzl TE
        • Todo S
        • Tzakis A
        • Alessiani M
        • Casavilla A
        • Abu-Elmagd K
        • et al.
        The many faces of multivisceral transplantation.
        Surg Gynecol Obstet. 1991; 172: 335-344
        • Todo
        • Tzakis AG
        • Abu-Elmagd K
        • Reyes J
        • Nakamura K
        • Casavilla A
        • et al.
        Intestinal transplantation in composite visceral grafts or alone.
        Ann Surg. 1992; 216: 223-234
        • Todo S
        • Tzakis A
        • Reyes J
        • Abu-Elmagd K
        • Furukawa H
        • Nour B
        • et al.
        Small intestinal transplantation in humans with or without the colon.
        Transplantation. 1994; 57: 840-848
        • Langnas AN
        • Shaw Jr, BW
        • Antonson DL
        • Kaufman SS
        • Mack DR
        • Heffron TG
        • et al.
        Preliminary experience with intestinal transplantation in infants and children.
        Pediatrics. 1996; 97: 443-448
        • Abu-Elmagd K
        • Reyes J
        • Todo S
        • Rao A
        • Lee R
        • Irish W
        • et al.
        Clinical intestinal transplantation: new perspectives and immunologic considerations.
        J Am Coll Surg. 1998; 186: 512-527
        • Grant D.
        Current results of intestinal transplantation. The International Intestinal Transplant Registry.
        Lancet. 1996; 347: 1801-1803
        • Vincenti F
        • Kirkman R
        • Light S
        • Blumgardner G
        • Pescovitz M
        • Halloran P
        • et al.
        Interleukin-2-receptor blockade with daclizumab to prevent acute rejection in renal transplantation.
        N Engl J Med. 1998; 338: 161-165
        • Kato T
        • O'Brien CB
        • Nishida S
        • Hope H
        • Gusser M
        • Berho M
        • et al.
        The first case report of the use of a zoom videoendoscope for the evaluation of small bowel graft mucosa in a human after intestinal transplantation.
        Gastrointest Endosc. 1999; 50: 257-261