Introduction: Intra-abdominal desmoid tumors are one of the leading causes of death in patints with Gardner's syndrome. Surgical resection of the desmoid tumor with or without intestinal resection is the treatment of choice for growing symptomatic intraabdominal desmoid tumor. Visceral transplantation is indicated for patients with short-gut syndrome after curative desmoid tumor resection with enterectomy. However, there has been a limited number of reports on outcomes of visceral transplantation for desmoid tumor. Here we report the first large series of visceral transplantations for desmoid tumor with long-term follow up at a single-center.
Objective: To Measure the outcome of visceral transplantation for desmoid tumor and recurrence after transplantation.
Methods: Between March 1992 and March 2012, a total of 24 intraabdominal desmoid tumor patients underwent 27 visceral transplantations with irreversible intestinal failure after tumor resection with enterectomy. With a total mean age of 35.6 ± 8.9 years, all patients were adult with female to male ratio of 1.6: 1. The average size of desmoid tumor on resection was 18.5 ± 8.9 cm. 2 (8.3 %) patients had prior chemotherapy. All donors were cadaveric and ABO-identical. Type of primary allograft was dictated by extent of tumor and polyposis in gastrointestinal tract. Of 24 primary allografts 19 (79.2%) were liver-free with 10 (41.7 %) isolated intesine, and 9 (37.5 %) modified multivisceral that contained stomach, duodenum, pancreas, and intestine. The remaining 5 (20.8 %) were liver-containing with 3 multivisceral grafts (12.5 %), and 2 liver/ intestine/ pancreas grafts (8.3 %).
Results: With a mean follow-up of 49.0 ± 39.3 months, 18 (72%) patients are currently alive. The overall actuarial patient survival was 87.3 % at 1 year, 73.2 % at 3 years, and 73.2 % at 5 years. The respective graft survival was 77.1 %, 64.5 %, and 64.5 %. The intraabdominal desmoid tumor recurred in 7 (29%) patients. 1 (4%) patient developed extraabdominal desmoid tumors. 8 (33%) had resection for recurrent or extraabdominal desmoid tumor.
Conclusion: With improving survival outcomes, intestinal and multivisceral transplantation is a valuable therapeutic modality for patients with advanced intraabdominal desmoid tumor. Early referral to a transplant center is essential for complex intraabdominal desmoid tumor.
To cite this abstract in AMA style:Sogawa H, Stein W, Koritzky D, Soltys K, Bond G, Sindhi R, Mazariegos G, Humar A, Costa G. Visceral Transplantation for Intraabdominal Desmoid Tumors: Survival Outcome and Tumor Recurrence [abstract]. Am J Transplant. 2013; 13 (suppl 5). https://atcmeetingabstracts.com/abstract/visceral-transplantation-for-intraabdominal-desmoid-tumors-survival-outcome-and-tumor-recurrence/. Accessed October 30, 2020.
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