Curative intent resections for cholangiocarcinoma in primary sclerosing cholangitis (PSC) patients: the Mayo Clinic experience

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Presenter: Ron Pery MD | Mayo Clinic, Rochester Background: Primary Sclerosing Cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Although the course of PSC is variable, it is frequently progressive and can lead to life threatening complications such as Cholangiocarcinoma which is usually unresectable upon diagnosis and is often the cause of death in PSC patients. Although liver transplantation may offer the ideal treatment in selected non metastatic cases, that option is limited due to organ shortage and may be limited due to tumor type and/or presentation. Traditionally resection has been avoided due to concerns about progressive liver failure postoperatively. the short and long-term outcomes of PSC patients undergoing oncologic resections of the liver or pancreas are not well defined. Herein we describe our experience with 21 cases of PSC patients who underwent curative intent resection surgery for Cholangiocarcinoma. Methods: The Mayo Clinic clinical record was used to identify PSC patients who underwent curative intent resection of the liver or pancreas for Cholangiocarcinoma between 01/2010-12/2020. Clinicopathological characteristics, operative details and outcomes of patients were collected and retrospectively analyzed with specific emphasis on post-operative complications, recurrence and survival patterns. Results: Twenty-one patients with PSC underwent curative intent resection for Cholangiocarcinoma at Mayo clinic between 01/2010 and 12/2020. The mean age of patients was 58±16.7 and none of them had any clinical or biochemical evidence of cirrhosis or portal hypertension. Three patients underwent whipple operation, seven patients had major hepatectomy with bile duct resection and hepaticojejunostomy, six patients had major hepatectomy and five patients had minor hepatectomy defined as 3 segments or fewer. Five patients had major vascular involvement. Seven (29%) patients had severe post-operative complications (Clavien Dindo score ≥3) with no 90-day mortality. On pathology, 13 (62%) patients had nodal positive disease and 7 (33%) patients had positive resection margins. 13 (62%) patients had adjuvant chemotherapy. The cohort’s median overall survival (OS) was 17.2±2.4 months and the median disease-free survival (DFS) was 13.7±0.6 months. Node positive disease was associated with much worse median OS and DFS (14.2±3.3 versus 59.3±39.4 and 7.9±1.2 versus 17.5 months, respectively). Positive resection margins were associated with worse median OS and DFS (11.0±2.5 versus 31.6±10.0 and 6.7 versus 17.5±6.2 months, respectively). Major vascular involvement was associated with worse median OS and DFS (10.3±1.2 versus 17.7±8.0 and 4.1±1.0 versus 17.5±8.4 months, respectively). Conclusion: With the given limitations of a naturally small cohort, PSC patients tend to present with Cholangiocarcinoma at earlier age and are more likely to have nodal diseases at presentation when compared to sporadic historical controls. Nodal disease, positive resection margins and major vascular involvement are related to worse prognosis. The OS and DFS are comparable to sporadic historic controls and PSC patients who had node negative disease had exceptionally high median overall survival. Therefore, when other treatment options such as transplant are not possible, resection can be considered with acceptable risk and complication profiles.