Familial Adenomatous Polyposis and Desmoid Tumor Treated with Multivisceral Transplantation and Kidney Autotransplantation: Case Report and Literature Review

Abstract

Introduction. Desmoid tumours (DT) are commonly associated with Gardener’s syndrome. Their surgical resection may be complicated by their close proximity to major vessels, multiple organ involvement, and frequent local recurrence. Multivisceral transplantation (MVTx) is an alternative treatment for patients with intestinal and liver failure. In patients with DT closely associated with renal structures but without end-stage kidney disease, concomitant excision of the patient’s own kidney, ex vivo tumour resection with nephron-sparing surgery, or autotransplantation has been proposed. Case Presentation. A 36-year-old Caucasian female weighing 60 kg with Gardener’s syndrome with a history of abdominal surgery was presented to our department with progressive abdominal distention associated with paroxysmal pain. With the use of CT, the patient was diagnosed with a mass arising from the mesenterial region. The patient had normal kidney function and nonalcoholic steatohepatitis. The patient was indicated for MVTx. Management and Outcome. After 16 months on the waiting list, the patient received a multivisceral graft from a deceased donor. Following the restoration of graft vascular flow, the patient’s right kidney was removed and the DT dissected ex vivo before autotransplantation into the right pelvic fossa. The patient received immunosuppressive, antithrombotic, and antibiotic treatment. There was no acute rejection, though the patient experienced pulmonary infection, dysphagia, and oesophageal reflux with fungal infection. The patient had required temporary dialysis for acute renal failure for 75 days. One year after the surgery, nausea and violent vomiting caused delayed gastric emptying caused by spastic pylorus. Clinical improvement was achieved using gastric peroral endoscopic myotomy (G-POEM). Conclusion. MVTx with kidney autotransplantation is a feasible treatment option in patients with familiar adenomatous polyposis complicated by an abdominal DT. Precise tumour dissection with nephron-sparing surgery was carried ex vivo. G-POEM was used to relieve MVTx-related gastroparesis. The patient had no disease reoccurrence after one-year follow-up.

Treatment of Complex Desmoid Tumors in Familial Adenomatous Polyposis Syndrome by Intestinal Transplantation

“…This has been described previously in both transplant and nontransplant settings and allows for the maximum preservation of renal function. 35,38 One patient (patient 2) in our series had renal failure with bilateral ureteric desmoid involvement and recurrent urosepsis. As a result, she underwent bilateral native nephrectomy with renal allotransplantation, in addition to a modified MVT.…”

“…This phenomenon has also been seen in most other series, with no intra-abdominal recurrences being reported. [21][22][23][24]35 The only exception was one report by the Miami group where a desmoid did recur in the abdomen but did not affect the transplanted intestine. 36 Although the exact reasons are unclear, the replacement with non-FAP genotype carrying transplant viscera, the ability to achieve radical disease clearance, and potential growth inhibition by some of the immunosuppressive agents may be contributing factors.…”

Update on Familial Adenomatous Polyposis-Associated Desmoid Tumors

“…Meanwhile, successful small bowel transplantation after total bowel resection has also been reported in patients with intra-abdominal DTs recently, 24 25 26 bringing hope to more patients.…”