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 accessOne-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 SurgeryAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Desmoid disease.in: Familial adenomatous polyposis and other polyposis syndromes. Edward Arnold, London1994: 128-142
- The occurrence of desmoids in patients with familial polyposis coli.Br J Surg. 1970; 57: 618-631
- Desmoid tumors in familial polyposis coli.Ann Surg. 1986; 204: 94-97
- Desmoid tumors.J Am Coll Surg. 1996; 182: 369-377
- 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
- Desmoid tumors in patients with familial adenomatous polyposis.Cancer. 1994; 74: 1270-1274
- Mortality in patients with familial adenomatous polyposis.Dis Colon Rectum. 1990; 33: 639-642
- The desmoid (Reitamo) syndrome: etiology, manifestations, pathogenesis, and treatment.Curr Probl Surg. 1988; 25: 232-320
- Intestinal transplantation: 1997 report of the international registry.Transplantation. 1999; 67: 1061-1064
- Recurrence of desmoid tumor in a multivisceral transplant patient with Gardner's syndrome.Transplantation. 1999; 67: 1197-1199
- Successful treatment of a desmoid tumor with doxorubicin.Cancer. 1993; 71: 2242-2244
- Chemotherapy for desmoid tumors in association with familial adenomatous polyposis.Dis Colon Rectum. 1997; 40: 798-801
- The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis.Cancer. 1987; 60: 2863-2868
- Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors.Am J Surg. 1991; 161: 416-421
- Intestinal transplant for recurring mesenteric desmoid tumor [letter].Lancet. 1993; 342: 58-59
- Identical-twin small bowel transplant for desmoid tumour [letter].Lancet. 1995; 345: 1577-1578
- Multivisceral grafting for Gardner's syndrome.J Wound Care. 1995; 4: 214-216
- Intestinal transplantation: an early experience.Transplant Proc. 1996; 28: 2734-2735
- Living-related small bowel transplantation in adults: a report of two patients.Transplant Proc. 1997; 29: 1851-1852
- Outcome analysis of 71 clinical intestinal transplantations.Ann Surg. 1995; 222: 270-282
- The many faces of multivisceral transplantation.Surg Gynecol Obstet. 1991; 172: 335-344
- Intestinal transplantation in composite visceral grafts or alone.Ann Surg. 1992; 216: 223-234
- Small intestinal transplantation in humans with or without the colon.Transplantation. 1994; 57: 840-848
- Preliminary experience with intestinal transplantation in infants and children.Pediatrics. 1996; 97: 443-448
- Clinical intestinal transplantation: new perspectives and immunologic considerations.J Am Coll Surg. 1998; 186: 512-527
- Current results of intestinal transplantation. The International Intestinal Transplant Registry.Lancet. 1996; 347: 1801-1803
- Interleukin-2-receptor blockade with daclizumab to prevent acute rejection in renal transplantation.N Engl J Med. 1998; 338: 161-165
- 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
Article info
Publication history
Accepted:
August 6,
2000
Footnotes
☆Reprint requests: Andreas G. Tzakis, MD, Department of Surgery, Division of Liver and GI Transplant, University of Miami School of Medicine, Highland Professional Bldg, 6th Floor, 180 NW 9th St, Miami, FL 33136.
☆☆Surgery 2001;129:277-81.
Identification
Copyright
© 2001 Mosby, Inc. Published by Elsevier Inc. All rights reserved.