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Cholangiocarcinoma

Cholangiocarcinoma. Part of the problem with cholangiocarcinoma is finding it. Ferreting it out in patients with primary sclerosing cholangitis cholangiocarcinoma or bile duct cancer. In this paper that Chuck Rosen published in 1991 pointed out that these patients.

Patients who are sick from PSC can’t be distinguished from patients with primary cholangiocarcinoma with regard to most of the symptoms that most of these patients present with. So a few years ago we sought to find a tumor marker that might help in this regard, and we settled on the CA-19/9. In 37 patients with PSC, nine of whom had cholangiocarcinoma, a value of over 100 units per milliliter gave us an 89% sensitivity for the presence of cholangiocarcinoma.

We continued to rely heavily on this modality for trying to determine who has cholangiocarcinoma in this very difficult group. Liver transplantation for cholangiocarcinoma has been abysmal in this country, including a number of studies that have shown a zero survival only one or two years out from transplantation. So again, at Mayo, we have tried to develop a protocol which would allow us to transplant patients with cholangiocarcinoma and do something for patients with this abysmal disease, many of whom are young men with PSC who have young families, for whom we certainly want to try to pull out all the stops.

The schema incorporates external beam radiation and brachytherapy, followed by 5-FU and as the waiting times lengthen for patients on the list it becomes a bit more difficult to administer this protocol. Suffice it to say that if after this therapy and on a mini-laparotomy the patient does not have evidence of extrahepatic disease, then we’ll transplant them.

I’m going to blow through the inclusion and exclusion criteria. Most of them are somewhat intuitive I think. This is a bit of a moving target, by the way, but at the time this slide was made 16 patients had been enrolled. Several of those had been excluded, either at the time of laparotomy or because of obvious extrahepatic disease, but about half had been transplanted and so far with a median survival of over 16 months, nine are alive with no tumor recurrence.

Well, I think most importantly, with regard to cholangiocarcinoma, we have to continue to talk about surgical resection and I’m going to show you the results of one study which I think is fairly representative of all the studies in the literature over recent years, with regard to resection of this disease. David _ and colleagues presented these data.

There are a number of other primary malignancies, including epithelioid hemangioendothelioma that represent good candidates for transplant. I would only make a plea to you that if you find a patient with even advanced EHE, this is a patient who can still be considered for transplant and they should not be ruled out. Even patients with metastatic disease may do well for a period of 4-5 years and they should be given a chance.