UC is a well-recognized complication of PSC. The current comorbidity rate of UC for patients with PSC in Japan ranges from approximately 20% to 55%, which is lower than the range of 30% to 80% for Western cohorts [1,2,3,4]. Although UC is also recognized as a risk factor for the development of dysplasia or cancer [5,6,7,8], we rarely encounter a patient with PSC-related UC and concurrent colon cancer requiring both LT and colectomy, as in the current case. Fukuhara et al. reported a case of proctocolectomy for colon cancer with PSC-related UC that was detected after LDLT [13]. However, there are no reports of concurrent cancer successfully treated with both LDLT and proctocolectomy.
The treatment of this case presented several challenges. The first involved the timing of proctocolectomy. Proctocolectomy could not be independently performed without LT because of severe liver failure. Simultaneous proctocolectomy with LDLT without a stoma could have resulted in anastomotic leakage as a potentially fatal complication. Even with the stoma, we were concerned that it might be difficult to control the fluid balance due to the possible massive fluid loss, not only from the ascites, but also from the intestine. Poritz et al. reported that patients with PSC who require colectomy can undergo simultaneous orthotopic LT and total abdominal colectomy; thereafter, they can be candidates for subsequent ileal pouch–anal anastomosis reconstruction when the liver function has improved [14]. However, unlike whole LT, small-for-size syndrome with massive ascites is a specific and significant complication of LDLT [15]. The patient developed small-for-size syndrome with a peak total bilirubin level of 20 mg/dl and massive ascites. However, if we had performed LDLT first without proctocolectomy, then bacterial translocation might have been a concern, especially with immunosuppression, because inflammatory bowel diseases, including UC, are known to cause a high risk of bacterial translocation [16,17,18,19]. Eventually, we planned to perform two-stage surgery with LDLT first because UC was expected to be controlled with immunosuppression. Fortunately, the posttransplant course was uneventful, and we were able to safely perform proctocolectomy after the patient had fully recovered at 3 months after LDLT.
The second challenge was the indication of LT with concurrent nonhepatic malignancy. Kosai-Fujimoto et al. reported that there was no significant difference in survival after LDLT for patients with and without nonhepatic malignancy, including nine concurrent cases [20]. They concluded that elective resection was successful if the tumors were at an exceedingly early stage. The UC-associated colorectal cancer patients had a prognosis that was similar to that of sporadic colorectal cancer at an early stage [9,10,11]. In our case, colon cancer was pathologically diagnosed as TisN0M0 stage 0; therefore, the prognosis was expected to be similar to that of sporadic colon cancer, which has a chance of recurrence-free survival without additional treatment of > 90% according to the Japanese Society for Cancer of the Colon and Rectum [12]. With UC, the incidence of poorly differentiated cancer with multiple developments is reportedly higher than that of cases without UC; this is one of the reasons why proctocolectomy is recommended [9,10,11, 13]. In the current case, because we did not find any evidence of poor differentiation characteristics in the colon tumor, we performed careful patient follow-up with minimal immunosuppression and planned to perform proctocolectomy as soon as possible after LDLT. We selected ileal pouch–anal anastomosis, which has been proven to be a safe approach after LT, as the bowel resection procedure [14].
PSC recurrence after LT, which contributes to poor patient and graft survival, especially after LDLT, has been well-recognized. Egawa et al. showed that the risk of re-transplantation for graft loss due to recurrence of PSC after LDLT is high, and that the risk factors for recurrence are high MELD scores (> 24), first-degree relative donors, postoperative cytomegalovirus infection, and early biliary anastomotic complications [21]. These did not occur in this case. Concomitant IBD was not associated with recurrence in their study [21]. We have not observed any signs of PSC recurrence in this case; however, regular liver biopsies and careful follow-up must be performed to monitor for recurrence.