Hi Jane,

Cholangiocarcinoma is usually found at a late stage because symptoms often only develop at a late stage.  However, if an extrahepatic tumor causes an early bile duct blockage, then that could contribute to finding it earlier. 

My understanding is that you are right in that surgery is the only procedure that is performed with "curative intent."  I don't think chemo + chemoradiation is generally thought to be curative.  However, I believe this is a pretty common approach, and is part of the protocol that the Mayo clinic uses as a preparation for a  liver transplant.  You could search for "Mayo transplant protocol" to learn some more about that.

I would also echo others in encouraging a second opinion.  So few doctors have CC experience, that it is a good idea, in my opinion, to try and find at least a couple of experienced teams and see what they recommend.

All the best,


It looks like they are starting up this trial at many of the major cancer centers.

The page on clinicaltrials.gov has the other centers (although everybody currently shows not yet recruiting.)


Thanks so much for letting us know how things are going.



(15 replies, posted in General Discussion)


Andrea and I had some issues with the process as well.  First, let me say I completely agree with your point...Don't over think it!  This is where many of our problems came up as well.  Anyway, I talked with Nasra (the RN at Mayo that is supporting this process) and I think I have the correct answers to my questions.  I thought I would pass along the things that I learned that were not obvious to me at least:

Where should I get the procedure done?

We tried to get the blood drawn at Andrea's cancer center (UCSF), but we could not get them to do it.  They didn't know how to bill the procedure for insurance purposes (more on that later), and the fact that the request came from a different institution (Mayo) did not help.  With effort, we probably could have gotten this to work, but we found it easier to just use a third party lab to do the blood draw.  We used Quest Diagnostics.

Does this cost me anything?

No, but...

The easiest way to get this procedure done is to pay for it yourself, and then send the receipt to Mayo (Nasra) for reimbursement.  I think this is the way most people end up doing it.  If this is a problem, your best bet is to get the lab in contact with Nasra.  Nasra can send over an invoice that should allow the lab to bill Mayo directly.

The problem here was really one of communication.  Andrea and I didn't realize we needed to pay and then get reimbursed.  We were thus stumped when the cancer center asked us "Who is paying for this procedure?"  Our answer should have been "it is patient paid," but we didn't know that...

When should I get the procedure done?

If you live in the U.S. or Canada, you need to plan on making an appointment between Monday and Thursday.  Mayo sends you a prepaid overnight Fed Ex box to send the sample back to them.  However, because the sample needs to arrive at Mayo on a business day (Mon-Fri), it needs to be given to Fed Ex on Mon-Thurs.  If you are not in the U.S. or Canada, be sure to talk with Nasra about when you should schedule your appointment.

Do I need to do anything before I go?

Yes.  You need to find the freezer pack that is in the package.  The freezer pack is under the sample collection box (both of which are inside the return Fed Ex box)  You should bring a frozen freezer pack to your appointment (at least 4 hours in the freezer is required)

What other issues can come up?

The clinic asked us if we had an "order" for the blood draw.  It turns out there is the equivalent of an order in the sample collection box.  If you are asked, say "yes, we do have an order!"

Let the clinic know that they need to give the materials to Fed Ex.  If you try to give the box to Fed Ex yourself, Fed Ex may not take it since it contains blood.  If Fed Ex doesn't normally pick up at your clinic, you or the clinic can call them and they will make a special stop.

Those are the things that tripped us up. 

If you tried participating in the Mayo study (successfully or unsuccessfully), please please please add any other things that you found confusing or challenging to this thread.  As I understand it, the plan is to take all of this feedback and use it to improve the Mayo process.  Part of that improvement will be a guide or FAQ on this website to help patients navigate potential problems.




(26 replies, posted in Members' Cafe)


I just wanted to say thank you. Thank you for all the time you have spent making hundreds of posts that have helped so many people.  Thank you also for the information and thoughts you have given me in person over the past year. 

The disease seems to ebb and flow, but the main arc is pretty consistent for all but a lucky few.  This does seem to be changing, but change is coming ever so slowly.

You have done so much for so many.  Be at peace my friend,



Any thoughts on the issue of forcing patients to terminate an active trial in order to qualify for hospice?



Andrea and I are also planning on attending.  We are not going to be able to stay overnight though.

Should be fun.  See you there Marion!



What an unfair and even cruel system!  Clinical trials generally make you wait until you have failed to respond to chemo in order to participate.  Then, when you do participate, you have to pick between a potentially helpful trial and the needed comfort offered by hospice?  That just does not seem right. 

I am not sure what to suggest.  Perhaps you could get a hospice "consult" to see what they would suggest without actually going on hospice?  You could also try to contact one of the other oncologists at UCSF.  Dr. Vinook was the name given to us as the backup for Dr. Kelley's patients when she was out.

You must be exhausted trying to support your sister and navigating an out of network clinical trial.  You are doing an amazing job.  Hang in there,


The whole pd-1/pd-l1 was very confusing to me, but I found this article helpful in understanding the pd1 / pd-L1 interaction:


If I read this right, MK-3475 is a pd-1 inhibitor.  There are also trials looking at pd-L1 inhibitors.  The Merck drug for anti pd-L1 is MSB0010718C.  A trial with this drug is described here:


Both approaches are trying to stop the pd-1/pd-L1 interaction (which turns off the immune response).  All very very exciting.

I hope your sister has a fantastic response.




(27 replies, posted in Clinical Trials)

Hi Melinda,

That is fantastic news.  Thank you for letting us know how it is going.  I can't tell you how helpful it is (to me anyway) hearing stories like yours. 

If I am reading you right, when you next go back to NIH, you will have been 12 months without treatment?  Amazing.  Congrats!

One question if you have a moment:  Do you know what the NIH is looking for when they look at the genetic sequencing?  If it is something exotic, people can try and make sure it is covered when they get a sequencing done.

All the best,



(14 replies, posted in Members' Cafe)

Today feels weird with no World Cup!

I guess I can go work on the patient registry smile


Thanks all for the ideas and the encouragement.  We are not at all thinking about giving up, so not to worry on that front.

The basic plan is to do chemo and then either radiation or ablation on the (hopefully) shrunken tumors.  It sounds like they would lean towards ablation given the previous radiation and the potential for overlap (they are checking on this).

They thought there was evidence that Xeloda kept the tumors in check since Jan, so the plan is to start there and monitor CA 19-9 for a 6 weeks or so.  If it keeps rising, we will have an early scan and change strategy (tougher chemo or local therapy).


I can't believe how long it has been (7 months!) since I officially updated this thread.

As I said in November, we had this plan:

"We have decided to continue on Gem/Cis at Stanford until January, and then switch to UCSF and do IMRT + Xeloda."

and that is pretty much what has happened. 

Andrea had her last Gem/Cis treatment Dec. 18.  In total, she was one treatment short of twelve cycles.  Overall, she handled the chemo really well.  A little tired, and a little hair thinning, but pretty manageable all things considered.  Towards the end of the treatment though her blood counts were starting to get low and not rebounding quite as quickly as earlier. 

The net result of chemo: 
-Main tumor 1/3 original size and PET inactive
-Two satellite "medium" tumors significantly small, necrotic looking and PET negative
-"Constellation" of tiny tumors no longer PET active or visible

Pretty good from where we started.

In late January (2014), Andrea started IMRT radiation therapy.  The plan was to irradiate the area around the main tumor.  The logic given was that this tumor would be the one most likely to cause problems in the future, and that all the shrinkage gave an opportunity to treat it within a manageable IMRT field.  The treatment regime consisted of 28 days of radiation (5 days a week for 5.5 weeks).

Radiation, it turned out, was much harder than chemo, both mentally and physically.  About halfway through treatment, Andrea was having difficulty keeping down any food or drink.  This lasted throughout the remaining weeks of treatment and didn't really start improving until 3 weeks post treatment.  During those weeks, Andrea lost 15-20 pounds and was very weak.  Mentally, the challenge was to get up every day and go to another treatment when you knew that was what was causing you to feel so lousy. 

Andrea had her last radiation treatment March 5.  I guess you never know how you will respond.  During chemo, the side-effects for Andrea seemed easier than advertised, but just the opposite was true for radiation.

Andrea also took Xeloda (aka capacitabine aka 5FU) during radiation, since that supposedly helps the effectiveness of the radiation treatment.  Since radiation was only treating the main tumor, the Xeloda also acted as systemic therapy for the other tumor areas.

Andrea got her first post radiation scan in late March.  Good news.  The treated areas looked as expected, and the untreated areas looked dormant (looking at the untreated areas was actually the purpose of the scan since they said it was too early to evaluate the radiation region).

At this point, the oncologist suggested a treatment break until June.  The idea was to let Andrea recover from the radiation, and then see what happens to the tumors.  If all remained quiet, the likely path would be localized treatment to each "medium" tumor.  If there was a recurrence, then that would dictate the treatment path.

By April 1 (Andrea calls it her "Canniversary"), Andrea was mostly recovered from radiation, and quickly getting her strength back.  When not knocked on her butt from radiation, Andrea likes to be active and involved.  Her new passion is fund raising for CCF...you will all be hearing more on that later!  smile

It is now mid-June, and the last two months without treatment have been fantastic.  Andrea feels great, and things seem almost normal.  We even got to spend a week in Paris.  Neither one of us had ever been to Paris, and it was fantastic.  Since the diagnosis last April, I think this was the best two months that we have had.

However, everyone says that this disease is a roller coaster, and I can see that more and more.  We just got the results from Andrea's June PET/CT scan, and the news was not good.  Two new tumors in the liver.  One is 1cm and one is 2.5 cm, both PET active. 

We don't have much in the way of details yet (we see the oncologist next Monday), but that seems like an awful lot of growth if nothing was showing up in March.  My theory is that since the March scan was not a PET scan, but only a CT scan, they missed the beginnings of these tumors.  I will find out more in a couple of days. 

I guess the one positive is that the tumors are still contained to the liver.  The report also indicated that no lymph nodes showed signs of involvement.  Still a major bummer.

So that is the latest.  Hopefully, Andrea can get back into the "good news / what's working" column soon.



(61 replies, posted in Suggestions)

Trying to register for the discussion board would take you to dead links.

It looks like the dead link issue has been fixed, but that has uncovered another problem.  Previously, a human would have to approve your registration request.  That step no longer seems to be happening, and now we are hip deep in bots!


Googled cholangiocarcinoma as well.


The 2nd Annual Paul J. Iwanowski Memorial Golf Tournament will be held next Monday, June 16 at the Whitney Farms Golf Course in Monroe, CT.  The event will benefit the Cholangiocarcinoma Foundation and the Boys and Girls Club of Stamford.

In addition to golf contests, the event will include a raffle, silent auction, cocktails and dinner.  Not a golfer?  Tickets for cocktails and dinner can be purchased separately. Event sponsorships are also still available.

Don’t miss the pre-event online bidding on 4 tickets to the June 18 Yankees – Blue Jays game.  You do not need to be present to win these tickets.

All the details can be found on the event website: http://www.birdeasepro.com/pauliwanowskimemorialgolf


(61 replies, posted in Suggestions)

My wife would like to create an account, but all of the "register" links appear to be broken.

That sounds like a big issue!



(61 replies, posted in Suggestions)

Dead link is fixed and website is faster.

Great work!



(61 replies, posted in Suggestions)

Dead link:

In the big banner ad, it says "The Cholangiocarinoma Foundation is in urgent need of Research Participants!"

Clicking on that text takes you to this link:


That is a dead link.

(The other links embedded in the banner ad work)



(16 replies, posted in Chemotherapy)


If you are stable or better, and handling the chemo really well, then I would be surprised if any changes are recommended.

Having said that, I still think it is important to have your case reviewed by a multi-disciplinary team at a major cancer center.  Even though they may not recommend changes, you never know what they will come up with.

Another advantage of visiting a major cancer center now is that you will have made future second opinions much easier (they will already have most of your records, you will have a patient ID, you will know an oncologist there, etc.)  If something changes with your condition, it will be much easier to get Mayo's quick take on the situation if you have already made contact with them.

Keep in mind that these are just some thoughts from a care-giver (and not a medical professional!)



(61 replies, posted in Suggestions)

It is slower for me as well Gavin.



(16 replies, posted in Chemotherapy)


In lieu of a biopsy, you could look for the IDH mutation via a blood test.  I have seen a number of papers linking the IDH1/2 mutation to 2-hydroxyglutarate (2HG) levels in the blood.  Here is one for example:


I do not know how difficult it is to get a blood test that looks for 2HG, but it might be something to track down if a sequencing is not possible.  The level of 2HG does seem to correlate with tumor burden, so that would be a consideration as well.



(61 replies, posted in Suggestions)

All the new content is fantastic. 

The only thing really negative I am experiencing is the floating menu bar (aka the Green Line).

There have been some posts from patients unable to find the 100 Q/A book and the Mayo blood registry, so I think we will need to go back and verify the links on the website (links on the new website differ from old website, and references are now dead links)

Overall though, the site is much more rich in content and very professional looking to me.


Genomic Profiling of Intrahepatic Cholangiocarcinoma: Refining Prognosis and Identifying Therapeutic Targets.


Interesting abstract (the paper is paywalled).  It reports the incidence of the IDH1 mutation in ICC as 15.5% based on 200 tumor samples.  It also said that patients with "bilateral" tumors, which I understand to mean tumors in both liver lobes, have 2.75 times the likelihood of having the IDH1 mutation.  That is starting to get reasonably likely for that type of patient.

Hopefully the IDH1 trial will fill and show a lot of promise.

Great news out of that trial would be fantastic.



(3 replies, posted in Introductions!)

With the website update, it looks like the book is now at a new link.

This one should work:

http://cholangiocarcinoma.org/news/foun … ring-form/

Let me know if you have any other problems.



(16 replies, posted in Chemotherapy)

I am so sorry to hear Lynn is not responding well to treatment.  I often worry about the day when Andrea’s treatment no longer works. I can’t imagine how difficult this is for you.
I keep a collection of thoughts and ideas for that time, and hopefully they help you.  There might be some more localized treatments you can consider, but if it needs a systemic approach, here are things I am thinking about that you might research and talk to the oncologist about:

Losartan is a drug that lowers blood pressure.  I mention it first because it is something you could consider right now.  It has been prescribed to millions of people, and has a good tolerance profile.
Here is an overview of the research:

http://www.cancerresearchuk.org/about-u … py-in-mice

Here is the main research article:

http://www.nature.com/ncomms/2013/13100 … s3516.html

Why am I interested in this?  During the stakeholders meeting, Christine Ferrone from MGH gave a lecture that claimed cholangiocarcinoma defends itself from the immune system by creating a barrier at the cell level.  The research paper indicates that Losartan seems to work by lowering cell collagen, increasing the ability of blood to penetrate, and ultimately allowing more chemo to be delivered.  They looked at pancreatic (which many have argued is similar to cholangio) and breast cancer cell lines in mice and noticed that the combination of losartan and chemo held the cancer in check much longer.  The results are mostly summarized in Figure 7 panel c and panel f for the pancreatic cell line.   For the pancreatic cell line, the chemotherapy they used in conjunction with Losartan was 5-FU (a component of FOLFOX).  I believe the article argues that Losartan can help chemo work again after the cancer stops reacting.  In any event, it has been shown to work in conjunction with a chemotherapy that Lynn is using, and has a reasonable tolerance profile, so based on that I think it is worth bringing up to your oncologist.


While you are waiting to see how FOLFOX does, I would definitely start looking into clinical trials.  Immunotherapy seems like a good place to look, especially if cholangio really does have an existing good immune response.  You already have identified the NIH trial (NCT01174121).  The other hot thing in immunotherapy right now seems to be PD-1 based trials.  There have been great results with other cancers for this type of drug, and reason to suspect it could work with cholangiocarcinoma given the immune response.  Merck is the furthest along with this drug, and the Merck drug got designated a “breakthrough” drug in March.  People seem to expect approval for the Merck drug (pembrolizumab aka lambrolizumab aka mk3475) later this year.  If it gets approved soon, you could try to use it off label.  If not, you could see if Lynn qualifies for a trial (e.g. NCT02054806).  Other companies are coming out with their own anti pd-1 and anti pd-L1 drugs, so I would search for other trials.  My rudimentary understanding is the tumors use PD-L1 to “turn off” nearby white blood cells and evade detection/destruction.  By suppressing PD-L1 in the body, your immune system is better able to find and kill the cancer.

Targeted therapy:

Since she was on the MET trial, I assume Lynn has gotten a genetic sequencing done on her tumor?  If not, I would definitely recommend getting it done.  There are a number of trials open based on having a particular genetic mutation (e.g. IDH, KRAS, MET, NOTCH, + lots more).  One I am focusing on is the IDH1/IDH2 mutation.  A trial was opened up in March for intrahepatic cholangiocarcinoma patients that have the IDH1 mutation:


The odds seem pretty decent for having this mutation.  If I recall correctly, the company running the trial (Agios), estimates that 20% of ICC patients carry the IDH1 mutation.  An earlier paper by Foundation Medicine indicated that IDH1/IDH2 was found in ~35% of cholangiocarcinoma patients (If you have survived a while with ICC, the odds may even be a little better of having IDH1 since this mutation seems to be associated with above average survival in other cancers).  The company had very good results with its initial trial of an IDH2 blocker and blood cancers.  The scientists seem to be pretty excited about IDH1/IDH2 blockers and cholangiocarcinoma, so that seems like one to look at.  For IDH1, my rudimentary understanding here is that cells with this mutation create IDH1 concentrations which causes immature cells to stop maturing and instead replicate (thus becoming cancerous).  By suppressing IDH1, the immature cells stop prematurely multiplying, and continue to develop into normal cells.

One thing I truly believe is that if you want a different result, you are going to have to do something different.  That is why I am a big fan of doing as many clinical trials as needed/possible to find something that works. Hopefully, some of these ideas help.

My heart goes out to you and Lynn,


PS - I think irinotecan is a common second line chemotherapy for cholangio.  Since Lynn is using FOLFOX rather than FOLFIRINOX, I assume she is not taking irinotecan?  Is there a reason for that?