Background: Pseudomyxoma peritoneii (PMP) arising from a low-grade appendix tumour can be cured by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. In those with small bowel involvement or recurrence, major tumour debulking can prolong life. However, inevitable disease progression results in nutritional failure from small bowel obstruction and often abdominal wall involvement with fistulation. This leads to poor quality of life and is eventually fatal. Radical cytoreduction and modified multi-visceral transplantation could prolong life in selected patients.

Methods: Between 2013-2016, 6 pmp patients underwent transplantation. All patients had prior surgery for PMP and further cytoreduction was unfeasible due to extensive bowel involvement. Patients had complete or intermittent bowel obstruction with intestinal failure ± fistulation. The procedures were performed jointly by peritoneal malignancy and transplant teams. 3 patients underwent radical debulking and 3 complete cytoreduction followed by transplantation. All were modified multivisceral grafts including stomach, Pancreaticoduodenal complex, small bowel, colon along with abdominal wall. Median operating time was 14 hours.

Results: Post-op stay on ITU average 12 days (range 2-45). Time on PN postoperatively: median 31 (range 19-51) Outcomes: 4 survived at time of review - 22, 18, 4 and 2 months following surgery; 2 died (Day 26 and day 64) the first from anastomotic leak, GVHD with associated fungal and bacterial chest sepsis, the other died of GI bleed and anastomotic leak. No episodes of acute rejection of intestinal graft seen but a single episode of grade 1 skin rejection of abdominal wall graft at day 68 treated with methylprednisolone. QOL data using EQ5D and SF36 showed significant improvements in QOL following transplantation with marked reduction in pain.

Conclusion: Cytoreductive surgery followed by modified multi-visceral  transplantation is technically feasible for end stage PMP and can prolong life, give independence from TPN with an excellent QOL. The long-term outcomes including recurrence will determine the effectiveness of this procedure. Surgery is complex and selecting fit patients and earlier referral may improve outcomes. This major intervention requires close collaboration of peritoneal malignancy & transplant teams.