Marion,
I have a few questions that I would like to have covered regarding the surgical management of cholangiocarcinoma.  One of the biggest questions and areas of controversy seems to relate to the eligibility for surgical resection.  Some patients are eligible for curative resection immediately upon diagnosis, some patients become eligible after successful chemotherapy or radiation treatment, and unfortunately, many patients are not eligible for surgery at all.  The criteria for surgery are far from clear cut.  Some surgeons and institutions are more aggressive than others, and many patients who seek out multiple opinions get vastly different responses.  In my wife Lisa’s case, we sought out six different opinions.  Three of the top experts (including the Mayo Clinic) said no to surgery and three said maybe, meaning that surgery could be possible with a robust response to chemotherapy.

In the case of ICC, the primary requirement for surgery is to have at least 20% remaining healthy liver, with no involvement of major vessels (portal vein, hepatic artery, etc.).  But from there the eligibility becomes very cloudy.  Some surgeons will disqualify a patient with tumors in both lobes, or tumors of some seemingly arbitrary size.  Some surgeons will disqualify patients with any extrahepatic spread.  However, other surgeons will still perform surgery as long as the spread is limited to certain lymph nodes or other organs that can also be treated surgically or through other means.

I guess my main question would be: is it possible to come up with more easily understandable guidelines for surgical eligibility?  I would also like to hear more about the concept of “tumor biology” and the role it plays into whether or not a patient is a candidate for a curative resection.

Thanks again for seeking our input.  I look forward to listening in on Thursday.

Jason,
So sorry to hear about the recent challenges that Andrea is facing.  Hopefully the folfirinox will help.  My wife Lisa had a robust response to folfirinox after her tumors progressed on gem-cis.  Her response to folfirinox was so great that she was able to have a resection back in January.  The surgical pathology report indicated that her largest mass (originally 15cm) was 70 percent necrotic due to the success of the neoadjuvant chemotherapy.  I just wanted to share our experience to let you know that there is hope.  Folfirinox is a very rough treatment protocol that is not easy to endure, but it also has had a dramatic treatment impact for many ICC patients.

I hope that Andrea gets some relief from the ascites very soon and that the roller coaster can begin to gain some upward momentum.  Your family continues to be in our thoughts and prayers.

-Matt

Dan,
My wife Lisa continues to deal with significant neuropathy as a result of several rounds of chemotherapy last year.  She has tried pretty much every treatment available and has been able to mitigate the symptoms somewhat through a combination of medications, physical therapy, and naturopathic remedies.  While her symptoms do not appear to be as severe as those experienced by your wife, Lisa continues to have significant numbness and lack of feeling in her feet.  This constant numbness definitely impacts her gait and balance, and she is still not able to drive a car or walk for long periods of time without frequent rest breaks.

The only drug that has worked for my wife is Lyrica.  She tried gabapentin and cymbalta to no avail.  The Lyrica does not cure the neuropathy by any means, but the symptoms seem to be much more manageable when she takes the maximum dose of 600 mg every day.  She was also prescribed a drug called Metanex, which is also taken daily.  We are not sure what impact, if any, the Metanex has on her neuropathy, but there are a number of anecdotal reports of patient improvements with this drug.

In addition to the Lyrica, Lisa has weekly physical therapy appointments with a physical therapist who specializes in chemotherapy induced peripheral neuropathy (CIPN).  She performs daily exercises recommended by the PT and has learned a number of coping strategies that have helped her quite a bit.  Lisa's physical therapist also recommended electro stimulation therapy (TENS device) which she uses every day in an attempt to help the nerves regenerate more quickly.  Again, we are not sure if this treatment is effective since there is little empirical data to support its efficacy, but Lisa is incredibly motivated and is willing to try anything to beat this neuropahty so she can get back to her normal life.

Lastly, Lisa has weekly accupuncture and massage treatments specifically focused on her feet and hands.  These treatments seem to help, but quite frankly, she is doing so many things to treat the neuropathy, we are not sure what is working and what is not working.  But at least we know that she is doing everything possible to manage her symptoms.

The oncologist and surgeon have told us many times that none of the above treatments will cure the neuropathy.  The nerve endings take time to regenerate and apparently nothing can speed up that process.  Our goal is to manage the symptoms until her body can heal itself.

Please feel free reach out to me privately if you would like to talk or if we can provide additional information.  I can definitely relate to the pain and frustrations caused by severe neuropathy and hope that your wife finds relief very soon.

-Matt

Jason, thank you for your very informative and inspirational post.  I agree 100% that we have many reasons to be hopeful.  Our situations are quite similar in that my wife Lisa is around the same age as Andrea.  Lisa was diagnosed in May of 2013 and we also have small children.

You provided an excellent summary of the treatment options that offer tremendous hope for extended survival and possibly even a cure.

I do not believe that the published survival statistics are very applicable today because they are all based on retrospective studies from the past 10 or even 20 years.  The treatments employed today have definitely extended survival and many patients are living productive lives -- surgical and non-surgical candidates alike.  I think we will see a major shift in the statistics as new studies are published in the future that take the current patient population into account.

Thanks again for being such an excellent resource for this forum.  I personally have learned a lot from reading your posts.  Although this journey is quite challenging, with many highs and lows, I share your optimism about the future.  We will continue to keep your wife in our thoughts and prayers.

-Matt

Snowbird,
My wife Lisa is experiencing severe chemotherapy induced peripheral neuropathy as well as a result of Folfirinox infusions last fall prior to her resection in January of this year.  She has tried just about everything to treat her symptoms and so far, Lyrica has worked the best for her.  Cymbalta and Gabapentin were not effective.  Lyrica does not make her neuropathy go away, but it is much more bearable for her when she takes the maximum dose once per day.  My understanding is that everyone is different and the response to the various drugs is not predictable.

Aside from the pharmacological interventions, Lisa has weekly physical therpy sessions which has also helped her symptoms.  She also does accupuncture, and electrostimulation.

We have learned that the bottom line with neuropathy is that it takes time to resolve, and there is not really much you can do to speed up the process.  Some patients experience neuropathy for only a few weeks, while some may take several months or longer to improve.  The focus of Lisa's treatments is to find ways to alleviate the symptoms and to develop coping strategies until the nerves are able to regenerate.

Please feel free to e-mail me privately if you would like to talk or if I can provide more information.  I hope that Ron is able to get some relief soon.

-Matt

6

(14 replies, posted in Chemotherapy)

Porter,
I would echo Marion's suggestion to contact your oncologist.  As you know, my wife Lisa underwent 8 rounds of folfirinox prior to her resection surgery (preceded by 6 rounds of gem-cis).  The folfirinox caused neuropathy from the first round and progressively got worse with each infusion.  She definitely experienced the neuropathy during the treatments, although we do know for sure if the culprit was oxilaplatin or irinotecan.  Towards the end of her treatments, the neuropathy was so bad that she needed a wheelchair to get to our car, but fortunately that was only on treatment days and the symptoms gradually improved until her next dose.  I also remember that she experienced temporary jaw tightness and mouth sensitivity almost immediately after the oxilaplatin infusions were started.

Lisa still continues to deal with neuropathy and is so unsteady on her feet that she is unable to drive and needs to use a cane (grudgingly) while out in public.  In hindsight, her medical team believes that they may have pushed too hard and that stopping the treatments earlier or lowering the dose could have prevented the neuropathy that she is dealing with currently.  Although, on the other hand, if she did not complete the 8 rounds of folfirinox, she may not have been able to have a resection so it was definitely a tradeoff for her.  We are confident that Lisa's neuropathy will improve but we have been told that it could take several months or even longer.

Based on Lisa's experience, I would definitely stay in close communication with your oncologist so they can monitor the situation and hopefully prevent any long-term effects related to your adjuvant chemotherapy regimen.

I have not posted for quite some time, so I wanted to provide a quick update on my wife Lisa.  Lisa was diagnosed with stage four ICC in May of last year with a very large mass (15cm) and several smaller masses in both lobes of her liver.  She also had positive retroperitoneal lymph nodes.  After consultations with our local team as well as second opinions at Mayo and Sloan Kettering, we learned that she was inoperable due to the tumor size, location, and extrahepatic spread.

Her tumors all grew slightly after 6 cycles of GEM/CIS and her CA19-9 continued to rise.  Needless to say, we were very concerned at this point.  The chemotherapy was switched to folfirinox, and fortunately, Lisa had a great response after 8 cycles of this second line treatment.  Against all odds, Lisa became operable and was able to have a resection surgery (trisegmentectomy, along with several other procedures) approximately one month ago.

The surgery was successful and all visible cancer was removed; however, the surgeon was not able to achieve negative margins along the hepatic vein.  Lisa will undergo six weeks of chemo-radiation (IMRT with Xeloda) starting in a few weeks to treat the R1 margin and potentially the lymph nodes as well.  Her CA19-9 from last week was 13 after a high of 1,880.  Needless to say, we are ecstatic over such a positive result.

As with all patients on this message board, the experience this past eight months has definitely been a roller coaster.  The folfirinox treatments were very rough on Lisa and she is still experiencing significant neuropathy which we hope will end soon.  The recovery from her surgery has also been very challenging, and she certainly is not looking forward to the radiation treatments.  However, we feel very blessed overall given the initial prognosis.

We have made numerous connections through this message board and Lisa is extremely thankful to all of those who have provided support over these last several months.  I hope to become a more frequent contributor to this site and will post additional updates going forward.

-Matt

Hi Chris,
My apologies for the delayed reply.  Life has been a little crazy the past week.  Our appointments at MSKCC went ok, but the news was a little disappointing.  Dr. Fong was not very encouraging about the future prospects for resection due to the advanced stage and lack of response to the first line chemotherapy.  He did not believe the HAI pump is advisable at this time but wants to reasses in a few months after four cycles of folfox.

Thanks to all who have responded for your continued support and encouragment.  Lisa and I have a very positive outlook in spite of recent setbacks and are pressing forward with the new treatment plan with great faith and determination.  I will do my best to keep you updated.

Kind Regards,

-Matt

Eric,
Thanks for your encouragement.  Who is your oncologist at MSKCC?  We are primarily making the trip to see Dr. Fong for an evaluation for a HAI pump as well as an opinion regarding surgical resection.  We will also be seeing Dr. Harding for a medical oncology consultation.  We have heard many great things about Dr. Fong, but have very little information on Dr. Harding.

Thanks again for your reply, and we hope that you continue to have success with your treatment.

Kind Regards,


-Matt

10

(25 replies, posted in General Discussion)

Kris,
Thanks so much for taking the time to talk to me and Lisa about your experiences with MSKCC, Dr. Fong, and the HAI pump.  It was great to get a first hand account of what Lisa would experience should she be a candidate for the pump protocol.

We are very inspired by your positive attitude and encouragement -- you are truly a gem.  Best of luck with your scan results on Monday, nothing but prayers and positive thoughts from Oregon. 

We will definitely follow up with you after our appointments in NYC on Monday.

Kind Regards,


Matt and Lisa

Porter, sorry to hear about the response you recieved from Mayo Phoenix.  It is totally unacceptable for them to not provide more details.  You deserve an explanation.  I have no idea why they did not believe that you would benefit from a consultation; however, it in no way means that you are a "lost cause" as you put it.  My guess is that the experts reviewed your images and reports and agreed with the current treatment plan -- meaning that they have nothing additional to offer at this time.

I like your attitude of fighting on despite this setback.  I am sure you will succeed in getting a second opinion.  Perhaps you could seek out a visit to another cancer center on your week off from chemotherapy in between cycles.

Again, please let me know if I an help in any way.


Kind Regards,

Matt

12

(18 replies, posted in General Discussion)

Porter, sorry to hear the news about diagnosis, but now that you have more information you can at least formulate a game plan.

My wife Lisa got a second opinion at Mayo (Rochester) shortly after her first visit at OHSU.  You can definitely get a second opinion after you start chemotherapy, but some centers may want you to wait until after a few cycles are completed.  We were very impressed with the Mayo system, the doctors, and the overall efficiency of the 3-day visit.  Unfortunately, the Mayo team simply confirmed the opinion of the OHSU team that Lisa was inoperable and unlikely to ever be a candidate for surgery. 

However, since that time, the OHSU team has shifted their opinion a bit and now they believe that Lisa may be a candidate for surgery in the future.  Lisa's condition is quite similar to yours -- ICC with a large 15cm mass and several smaller masses.  The only difference is that Lisa is stage 4A due to local and distant lymph node metastases.  Dr. Billingsly is the liver surgeon at OHSU and he is one of the top surgeons on the west coast -- he trained at Sloan Kettering.  Dr. Billingsly reviewed my wife's scans at the tumor board and he believes that resection is possible with a good initial chemo response. 

We have also sought multiple other opinions from surgeons who essentially agree that resection could be possible in the future.  Some surgeons will review your scans and will provide a remote consultation without actually having to visit them.  Please let me know if you would like further information and I can provide some names and contact numbers.

The takeaway from my rambling is that second and third opinions are incredibly valuable.  Just because you have been told that surgery is unlikely does not mean that you will not become a surgical candidate in the future.  And like I stated previously, many of those who have been deemed permanently inoperable have had very good outcomes and live productive lives largely free of symptons.

Best of luck on your journey, and please let me know if I can help in any way.


Kind Regards,

-Matt

13

(25 replies, posted in Introductions!)

Porter,
For what it is worth, Lisa's primary tumor is nearly as large as yours (15x12x13).  The primary tumor has comprimised the portal vein and hepatic artery.  She also has 3 smaller tumors and positive lymph nodes near the aorta.  Despite the advanced stage, which I believe is somewhat similar to your case, we are very hopeful and optimistic.  Lisa has completed two cycles of chemotherapy (gem/cis) and has tolerated it quite well so far.  She will have her first post chemo scan in another two weeks.

There are many cases of patients with similar size masses that begin as unresectable and become resectable after chemotherapy and other targeted treatments.  We are very hopeful that resection will become an option in future.  Even for non-resectable patients, many have had success at shrinking the tumors, managing symptoms, and preventing further growth for several years. 

As for Dr. Vaccaro, I have heard many positive comments.  I believe that you are in very good hands at OHSU. 

I am sure you are still in shock after the diagnosis, and after almost two months the shock has still not worn off for us.  Please rely on the folks on this board for support, it is an unbelievable resource.  Don't give up the hope, and please let me know if I can help in any way.

Warm Regards,

Matt

14

(25 replies, posted in Introductions!)

Porter,
My wife Lisa was diagnosed with unresectable, stage 4 ICC on May 30, 2013.  We too have small children (ages 1 and 4), so I can definitely relate to the added anxiety and stress of battling this disease and raising young children.

We also live in the Portland area -- Lisa is being treated at OHSU as well.  We are very happy with our care at OHSU and our oncologist, Dr. Lopez.

Many on this site stress the importance of second opinions and I definitely concur with this advice.  We have obtained multiple opinions from some of top cancer centers in the country.  So far, all of the experts have concurred with the initial treatment plan outlined by the team at OHSU.

Please let me know if you have any questions.  I would be happy to share any information that I have regarding our journey as well as local resources that may be helpful for you.

Warm Regards,


Matt

15

(7 replies, posted in Good News / What's Working)

Carl,
Great news!  Thanks for sharing.  Since my wife Lisa has a very similar diagnosis, we are extremely encouraged.

Please keep us posted on your consultation with the team at MSK.

Kind Regards,

-Matt

16

(3 replies, posted in Introductions!)

Hi Sarah,
Sorry to hear of your father's diagnosis.

My wife Lisa was recently diagnosed with CC as well.  We also consulted with the team at Mayo and learned that her cancer is currently unresectable -- also due to portal vein involvement as well as some other factors. 

My understanding from my research and from reading posts on this site is that some patients are initally unresectable and later become surgical candidates due to positive response to chemo and other treatment modalities.  Perhaps surgery may be an option in the future?

Like others have stated, we have also learned the value of attaining multiple opinions.  It seems that Mayo may be more conservative in terms of surgical options, as I have read about some patients who were told that surgery is not an option at Mayo and subsequently underwent successfull resections at some other centers including Memorial Sloan Kettering and MD Anderson to name a few.

I wish your father the best of luck with the port placement and with chemo treatments.

-Matt

Wow.  Thanks so much to all of you for your kind words of support and encouragement as well as the very helpful tips and advice.  It is reassuring to know that my wife Lisa is not alone in this fight.  After a month of reading various posts to this board, it is nice to finally make personal connections. 
I am amazed at the level of medical knowledge and sophistication that you all possess.  My head is still spinning from my research into all of the various treatment options, facilities, doctors, etc.  Reading about your personal experiences and knowledge that you have gained certainly has helped to make some sense of this very complex labyrinth.

Jason – we have a lot in common.  Thank you for sharing your knowledge and experiences.  I was not aware that we could potentially purse the local and regional chemotherapy options concurrently.  I will definitely discuss the DEBIRI protocol with our oncologist.  I’m happy to hear that your wife Andrea seems to be responding to her initial treatments.  I believe we both have the same hope of becoming a candidate for resection at some point in the future. 

Carl – thank you for sharing your experiences thus far; our circumstances are quite similar as well.  It is reassuring to hear that we did the right thing by starting chemotherapy right away rather than delay treatment.  You stated that surgery is not an option for your wife Lynn.  Would you mind providing further details?  I will also be curious to hear about your experience at Memorial Sloan Kettering.  Dr. Fong at Sloan is so far the only surgeon who believes that my wife is a candidate for resection (the official tally is 3 no’s, 1 yes, and 1 maybe).

Susie – it is great to hear about another CC survivor right in our hometown.  We actually had an appointment with Dr. Vaccaro, but we chose to see Dr. Lopez at OHSU because he was able to see us a week sooner.  My wife was diagnosed during the ASCO meeting and just about every GI oncologist in the country that we contacted was in Chicago and not able to see her right away.  Do you know anything about Dr. Billingsley at OHSU?  If Lisa becomes a candidate for a resection, he would be the one to perform the surgery.

Lisa – thanks so much for providing your contact information and agreeing to speak with my wife – also named Lisa.  We visited your caringbridge site and were blown away by your amazing journey and your strength and determination.  Lisa would like to reach out to you directly in the near future.  Your story has become an inspiration.

Willow – you are an amazing advocate for your sister.  She is very lucky to have you in her corner.  Thank you for sharing your story.  I am sure your sister can relate our difficulties in managing this disease while raising young children.  It is comforting to know that there is also great hope for those who are not candidates for resection.

Marion – thanks for the tip regarding Dr. Schulick.  I previously lived in Denver and would not mind getting back there for a consultation.  As the biggest question mark for my wife is whether or not a resection is possible, I do not believe that you can have too many opinions from the top liver cancer surgeons.

My wife Lisa was diagnosed with Stage 4 intrahepatic cholangiocarcinoma on May 30, 2013.  Lisa is 43 years old with no risk factors and is in excellent health otherwise.  Obviously, the diagnosis came as a complete shock.  Her only symptoms have been moderate abdominal pain and general fatigue for the past several months.  She attributed the fatigue to the busy lifestyle of a mother with two small children (ages 1 and 4).

After an initial ultrasound identified multiple liver masses, a CT was ordered.  The CT revealed four distinct masses - a very large tumor occupying most of the right lobe and extending into the left lobe measuring 15x12x13, as well as three smaller tumors in the left lobe measuring 2, 2, and 5.5 cm respectively.  The right portal vein is completely encased by the large tumor and the right and middle hepatic arteries are also compromised.

A subsequent biopsy confirmed the diagnosis of cholangiocarcinoma.  Additional imaging (CT, MRI, EUS, and PET) revealed both regional and distant enlarged lymph nodes; however, no additional metastases have been identified.  A biopsy of the distant (aortacaval) lymph node was positive for cholangiocarcinoma. 

We were referred to a GI oncologist at our local university medical center in Portland (OHSU) for consultation.  We learned that transplant is not an option due to the intrahepatic nature of the cancer.  We also were informed that resection would be unlikely because of the size of the main tumor, the multifocal involvement, and distant lymph node metastasis.  The standard treatment of gemcitabine and cisplatin was recommended.  Our oncologist discussed the potential for future treatments depending upon the chemotherapy response, including ablation, embolization, and regional chemotherapy via an intrahepatic pump.

We scheduled a visit at the Mayo clinic in Rochester the following week for a second opinion.  The Mayo team (oncologists, hepatologist, and surgeon) agreed with the initial diagnosis and treatment plan.  While at Mayo, a second biopsy was taken for genome testing with the hope of developing some targeted therapies based upon the unique pathology of the tumors. 

We also had a phone consultation with an oncology surgeon at Sloan Kettering.  The surgeon at Sloan is confident that Lisa could be a candidate for resection if we are successful in shrinking the tumors.  The Sloan team recommend starting with regional chemotherapy instead of the systemic approach.  As we were scheduled to begin the standard systemic chemotherapy the next day, we chose to stick with our original plan and perhaps travel to NYC for further evaluation depending upon the response to the first few rounds of treatment.

Lisa has successfully completed her first two chemotherapy treatments and so far has tolerated them well.  Her symptoms have not changed much in the month since the initial diagnosis with the exception of increased fatigue 2-3 days after chemotherapy.  She still has a fair amount of pain from the installation of her port a few weeks ago.  We will have the first “post chemo” scan in another month.

Last week at our second visit with our oncologist, we learned that the local surgical team would be willing to consider Lisa for resection depending upon her response to the chemotherapy.  After initially being told that resection would not be an option due to the distant lymph node metastasis, the tumor board discussed her case and they concurred that one positive node would not render her unresectable.  We were also told that the lymph node could be removed during the surgery.

In addition to the opinions of the OHSU (Knight Cancer Institute) team, The Mayo Team, and the Sloan Kettering team, our medical reports and scans have been reviewed by a family friend who is an interventional radiologist at Loma Linda Medical Center.  Our friend reviewed our results with his colleagues as well as a prominent surgical oncologist.  The Loma Linda team assisted in the initial diagnosis and agreed with the findings above. 

Lastly, at the advice of a CC survivor and frequent poster to this site, we have sent our scans to Dr. Selby at USC for an additional (and technically the 5th) medical opinion.  We have heard great things about Dr. Selby and are anxious to hear his opinion and recommendations.

We had an appointment scheduled at MD Anderson immediately after our Mayo trip, but we cancelled the trip because Lisa was so exhausted after so many tests and procedures in such a short time. 

Obviously, we have learned a lot about this disease in the past month; however, after reading the posts on this site I realize that we still have much to learn.  I would welcome any thoughts, recommendations, words of wisdom, or treatment protocols that have worked for others with similar circumstances.  Lisa and I are very motivated and determined to fight this cancer.  My purpose in posting to this board is to leave no stone unturned.

Thanks for reading, and best of luck with all of your respective journeys.