| 抄録 |
In the days when liver transplantation was being developed, a common indication for the procedure was unresectable cancer in the liver. Hilar cholangiocarcinoma (CCA) was among the diagnoses for which liver transplantation was performed in those early days, Due to dismal results, however, CCA soon became a contraindication to transplant. It is the nature of this tumor to spread relatively early in its clinical course to regional lymph nodes and within nerve sheaths in the porta hepatis; by the time the tumor presents clinically with jaundice, such spread is the rule. At the Mayo Clinic, where a large cadre of patients with primary sclerosing cholangitis (PSC) is followed, a program of endoscopic surveillance was developed that led to the diagnosis of a number of patients with early CCA who, based on their liver disease, were not candidates for resection. A protocol for transplantation of these patients was developed there incorporating preliminary exploration to rule out peritoneal or nodal spread followed by neoadjuvant chemoradiation, with initial results published in Liver Transplantation in the year 2000 comparable to those achieved with transplant for benign disease. Patients with unresectable CCA not arising in the setting of PSC have subsequently been included in the Mayo protocol, but the majority continue to be in the setting of PSC. Technical difficulties with vascular reconstruction after hilar radiation as well as logistical difficulties related to organ availability have hindered progress; nevertheless, based on Mayo Clinic data the United States transplant network decided to grant priority for transplant to patients with unresectable CCA who are treated within a protocol incorporating neoadjuvant therapy. Initial results of the more widespread application of transplant for CCA in this context were reported in Gastroenterology in 2012; intention to treat survival was only 53% at five years, with a 25% drop-outs and posttransplant recurrence-free survival of 65%, significantly worse than the earlier Mayo data (which were included in this analysis). The majority of patients transplanted had PSC; in the roughly 1/3 who did not, drop-out was significantly more likely, and recurrence-free survival after transplant was only 51%. It is becoming clear that, while transplant within the context of a multimodality protocol has an important role for patients with PSC who on surveillance are found to have early CCA, it is not the answer for the typical patient without underlying disease who presents with obstructive jaundice due to hilar CCA. |
