Background: Pseudomyxoma peritoneii (PMP) arising from a low grade appendix tumour can be cured by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. However, 40% of patients devolop residual or recurrent disease. The inevitable disease progression eventually results in nutritional failure from small bowel obstruction and often abdominal wall failure with fistulation. This leads to poor quality of life and is eventually fatal. Median life expectancy is 10 months in patients requiring parenteral nutrition. Radical cytoreduction and modified multi-visceral transplantation could benefit selected patients.

Methods: Between 2013-2018, 13 pmp patients underwent transplantation. Patients had previous surgery for PMP and further cytoreduction was not feasible due to extensive bowel involvement. Patients had complete or intermittent bowel obstruction with intestinal failure ± fistulation. 11 of the 13 patients were on parenteral nutrition and 2 patients had impending gut failure. 4 patients underwent radical debulking and 7 complete cytoreduction. 6 Patients had isolated intestinal transplant and 7 received modified multi-visceral grafts. Median operating time was 13hours.

Results: Post-op stay on ITU average 4 days (range 1-45). Nine patients surviving at time of review (Follow up 1 month to 42 months following transplantation); 2 died (Day 26 and day 64) due to post operative complications, 1 due to GVHD at 5 months following transplantation and 1 due to recurrent pseudomyxoma at 40 months. Two patients had acute rejection and 1 patient had borderline rejection of intestinal graft treated with methylprednisolone. QOL data using EQ5D and SF36 showed significant improvements following transplantation with marked reduction in pain.

Conclusion: Cytoreductive surgery followed by modified multi-visceral transplantation is technically feasible for end stage PMP. It could potentially extend life giving independence from TPN with an excellent QOL. Our recommendation is to undertake the procedure in patients with low grade disease where complete cytoreduction is likely to be feasible and with impending or established intestinal failure.