Thymic Tumors

Posted by anotherfinemass @anotherfinemass, Jan 16, 2019

As the owner of a fairly large thymic tumor, I have been trying to and understand the condition, currently acceptable treatments, and the various risks associated with those treatments (and non-treatment). The basics of my initial understanding are:

(1) Thymic tumors are relatively rare.
(2) Except in the case of thymic hyperplasia, thymic tumors are cancerous.
(3) Thymic hyperplasia is extremely rare in adults.
(4) The cancerous thymic tumors are thymomas, thymic carcinomas, and neuroendocrine tumors.
(5) The most dangerous and least understood of those are neuroendocrine tumors.
(6) In almost all instances, surgical removal of the thymic tumor is the standard treatment.
(7) The most common and most desired goal of surgery is a full resection of the tumor.
(8) Full resection of the tumor is important to achieve satisfactory long-term survival.
(9) Biopsies of thymic tumors are usually performed at the time the tumor is being surgically removed.

Some of the most common conditions that are associated with thymic tumors and the operations conducted to remove them are:

(1) Phrenic Nerve damage;
(2) Sympathetic Chain damage;
(3) Sternal wound infections;
(4) Damage to the superior vena cava and/or other nearby veins;
(5) Recurrent laryngeal Nerve damage;
(6) Horner Syndrome, and;
(7) Myasthenia gravis.

Of greatest concern in considering the above is that just as the tumors are relatively rare, so are surgeons that specialize in resecting the tumors. And not being independently wealthy, resources for consults and second or third opinions are limited. This has given rise to the question of what is the proper or commonly recognized protocol to engage surgeons.

More specifically, many thoracic surgeons do not work with thymic tumors. That being the case, I have tried to get answers to the following questions before making an appointment for a consult:

(1) How many thymic tumors (approximately) have you removed or debulked in your career?
(2) Of those, in how many cases was the tumor completely removed and how many debulked?
(3) Of all those procedures how many (approximately) were for which of the four types of tumor?
(4) What were your best and worst results?
(5) The mass in my chest now measures ## cm x ## cm x ##.# cm in craniocaudal, mediolateral and anteroposterior dimensions. Have you ever operated on a mediastinal mass this size?
(6) Have any of the several conditions listed above have been common (occurring in more than 50%) in your patients, and if so, which conditions?

My limited experience in this pursuit of these answers is that the surgeon's assistant says I need to schedule an appointment to discuss those matters with the surgeon. However, that leaves you in a "chicken or the egg" situation where you need a referral to see a surgeon, but without the answers, you aren't sure if you want to use the referral for that surgeon! And sending unsolicited emails has not been productive.

Has anyone else dealt with this? What was your process? What's the process at Mayo? (I am not located near a Mayo Clinic.) Also, please feel free to supplement the information above or correct any misinformation (provided unknowingly).

Thanks in advance.

Interested in more discussions like this? Go to the Cancer Support Group.

@hopeful33250, Thank you for your welcome and the various resources you've suggested. I have spent a great deal of time on the National Institutes of Health and U.S. National Library of Medicine websites. And yes, with thymic tumors having an incidence of only 0.15 per 100,000 population that comes out to about, what, 1 in 700,000 people having such tumors? An especially unique group, for sure.

From the medical professionals (a PA, an Emergency Room doc, a family practice doc) I have spoken with on a personal level (friends, relatives, and friends of relatives), it seems that the deeply involved patient is a pretty rare thing itself. I'm sure some of that can be attributed to a fuzzy familiarity with the diagnosis. I mean, if your doctor tells you "We need to remove your appendix" you may well know someone that has already been through it. And with an incidence rate of 1.1 cases per 1000 people per year and a roughly 99% percent success rate for appendectomies, people don't bother getting into the weeds on the details of what they're about to go through.

I imagine it's frustrating for doctors too, trying to help people when there's so little science and data to guide them. So far, it seems to me that all you can do is try to connect with the doctor you find that has the most experience (which probably is less than you would like), cross your fingers, and climb up on the table. And as you can see, I'm still at the "connect with the doctor you find that has the most experience" phase of my journey.

Okay then. Thanks again and be well.

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@anotherfinemass

@hopeful33250, Thank you for your welcome and the various resources you've suggested. I have spent a great deal of time on the National Institutes of Health and U.S. National Library of Medicine websites. And yes, with thymic tumors having an incidence of only 0.15 per 100,000 population that comes out to about, what, 1 in 700,000 people having such tumors? An especially unique group, for sure.

From the medical professionals (a PA, an Emergency Room doc, a family practice doc) I have spoken with on a personal level (friends, relatives, and friends of relatives), it seems that the deeply involved patient is a pretty rare thing itself. I'm sure some of that can be attributed to a fuzzy familiarity with the diagnosis. I mean, if your doctor tells you "We need to remove your appendix" you may well know someone that has already been through it. And with an incidence rate of 1.1 cases per 1000 people per year and a roughly 99% percent success rate for appendectomies, people don't bother getting into the weeds on the details of what they're about to go through.

I imagine it's frustrating for doctors too, trying to help people when there's so little science and data to guide them. So far, it seems to me that all you can do is try to connect with the doctor you find that has the most experience (which probably is less than you would like), cross your fingers, and climb up on the table. And as you can see, I'm still at the "connect with the doctor you find that has the most experience" phase of my journey.

Okay then. Thanks again and be well.

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All you say is so true, @anotherfinemass. The one frustrating thing for all of us with rare or orphan disorders is the lack of research. Drug companies are less likely to spend money developing treatments for rare disorders that affect only a small percentage of people, because it is not that profitable for them. The frustrations for these disorders are too long to describe. It seems you have a good handle on what you need to do and who you need to find. I certainly wish you well.

Will you be going to Mayo for a consult?

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Hello @anotherfinemass, I'm not sure if you are familiar with Google Scholar (https://scholar.google.com/) but thought I would mention it in case you are not familiar with it. I use it a lot trying to find the newest research information including medical treatments. It lets you sort the sources/articles it finds by year to get the newest first.

Hoping you find some answers...

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@anotherfinemass

Hello @shilo14, Thanks for the welcome and the FB referral. This is all very new to me, but it was my understanding that the outlook for survival with thymomas was extremely positive - more a matter of what survival looks like. As an example, from the limited data I'm finding, it looks like phrenic nerve damage occurs in about a third of patients. Apparently, there is very little room in the mediastinum area for surgeons to work and collateral damage is an unfortunate reality of that. If I had it all to do over, I would have monitored my tumor a lot better and caught it before it became so large. But when you don't have any symptoms from it, it's easy to just put it out of your mind in the hustle and bustle of the day-to-day. Anyway, thanks again and be well.

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I'm wondering if you already saw this discussion:
- Phrenic Nerve damage https://connect.mayoclinic.org/discussion/phoenix-nerve-damage/

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@hopeful33250

All you say is so true, @anotherfinemass. The one frustrating thing for all of us with rare or orphan disorders is the lack of research. Drug companies are less likely to spend money developing treatments for rare disorders that affect only a small percentage of people, because it is not that profitable for them. The frustrations for these disorders are too long to describe. It seems you have a good handle on what you need to do and who you need to find. I certainly wish you well.

Will you be going to Mayo for a consult?

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@hopeful33250 - Well they say money won't buy happiness, but it will pay the salaries of a large research staff to study the problem. đŸ™‚

As for coming to Mayo, that would be ideal, but given that I am several states away, there are many moving parts and I would have to be more organized than I am this minute. At this stage, I'm mostly just digesting the realities and trying to sort out my priorities. And as it turns out, that's a fairly large order to fill.

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@johnbishop - You're a man after my own heart. I absolutely use Google Scholar. The Internet is such an amazing tool that it's almost a miracle. Just 30 years ago even doctors didn't have the volume of information readily available that the average person can access today. You have to be careful though; some of the papers you find on there are so focused on one case that if you don't read a ton of them you could easily lose context. Anyway, thanks for checkin' and be well.

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@anotherfinemass

@hopeful33250 - Well they say money won't buy happiness, but it will pay the salaries of a large research staff to study the problem. đŸ™‚

As for coming to Mayo, that would be ideal, but given that I am several states away, there are many moving parts and I would have to be more organized than I am this minute. At this stage, I'm mostly just digesting the realities and trying to sort out my priorities. And as it turns out, that's a fairly large order to fill.

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@anotherfinemass
Yes, take it one step at a time. Just wrapping your mind around a diagnosis like this is as you said, "a fairly large order to fill."

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@colleenyoung

I'm wondering if you already saw this discussion:
- Phrenic Nerve damage https://connect.mayoclinic.org/discussion/phoenix-nerve-damage/

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@colleenyoung - No I had not seen that thread, but I have read similar accounts. Anyway, thanks for all your help and be well!

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@anotherfinemass

Hello @johnbishop and thanks for the welcome!

I actually saw the post you refer to and felt it had a more personal theme than my post, which I intended to be a discussion pertaining to process. Obviously, people's personal accounts can be valuable to the similarly situated, but in the context of what is currently working for those entering the process today, I'm not sure that thread, which is fairly long, is as useful since @allisonsnow began her journey a decade ago in 2009.

Additionally, it seems that the rareness of these tumors has caused more than a bit of confusion about the facts. For instance, other than thymic hypoplasia, all thymic tumors are considered cancerous. However, it is more recently stated that thymomas "have the potential to behave like cancer" and spread beyond the thymus. Yet many thymomas appear to behave in a benign fashion and are noninvasive. I believe this type of doublespeak is a result of the lack of scientific understanding of thymic tumors and that it contributes to confusion for those trying to understand their condition.

It was my hope that this thread would focus more on sharing the most current understanding of this condition's informational deficit and how to address medical professionals that may not be experienced in this evolving area of medicine. Similarly, to your point that the surgeon is the only one who may be able to answer questions such as those I have posed, I believe it is inappropriate that patients should have to spend valuable resources just to get a clear picture of a doctor's experience with a particular procedure. Indeed, it is my opinion that if the information available for doctors was anything near the volume of information available for NFL players, patients would be in a drastically better position.

Finally, pertaining to the post edit policy, 5 minutes seems rather fast to shut off editing for a post - particularly when there has been no response to the post. This forces a user to actually create a comment to their original post in order to correct an error. Of course, readers may not get to that correction and instead leave with the incorrect information. The more current trend is to allow editing for the life of the post but to offer "edit history" to readers. Likewise, it seems inappropriate to disallow the deletion of one's own post. This (at least on my part) dampens dynamic discussion and creates a reluctance to post what is in effect a permanent record - particularly when folks may be discussing personal issues.

Regards -

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Hi there and welcome,

My dad was diagnosed with a mediastinal mass 6.5cm X 5.8cm on August 7, 2018. Like you, I was feeling overwhelmed at the lack of current research and the research that existed lumped thymomas and thymic carcinomas together (to get a bigger sample size) added to my frustration and fears. I was hoping it was a thymoma, but learned it was squamous cell thymic carcinoma. This path was sent off for further analysis which took another 3-4 weeks to result, but was able to tell us how receptive it would be to immunotherapy. The chemo regimen of carbosplatin and taxol had just fair results.

What I learned through my dad's surgeon, at least in our case, was that de-bulking was not an option with his type of tumor. What we were told by the surgeon was that he would not even attempt to remove the tumor unless he was sure he could safely get all of it. This being an aggressive tumor, he didn't want to leave any of it behind. To determine proximity, a cardiac MRI was ordered to try to "see" if it had invaded or nested too close to vital vessels, like the aorta. The surgeon was hopeful he could do the surgery, but after reviewing the cardiac MRI he and his team felt the tumor was too close to my dad's pulmonary artery and innominate vein for him to attempt to safely remove it. His fear was to open my dad's chest via sternotomy only to realize he could not do anything or worse attempt surgery and have a poor outcome. A poor surgical attempt would simply delay treatment for 5 weeks and that's barring any more time due to surgical complications.

Since thymic carcinoma is rare AND aggressive form of cancer, he consulted with cardiologist reading MRI, oncologist, radiation oncologist and his surgical partners to make the safest decision and determined the best course of action for my dad. We ended up not having surgery, but did have chemo and radiation. My dad's tumor has not spread and after radiation was notably smaller. He has since been started on Keytruda every 3 weeks. The disease is currently stable and we could not ask for more.

I would definitely find a surgeon who was not eager to cut, but one that was thoughtful and understood this type of tumor, meaning since rare they will need to review and learn about this very rare cancer. Time is of the essence. I never appreciated all the considerations that have to made until we were knee deep. We were fixated on surgery, but know we went down the right path.

Please feel free to ask any questions. You are not alone and treatment options are getting better with options in immunotherapy.

Gail
Greenville, SC

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@gailkattouf

Hi there and welcome,

My dad was diagnosed with a mediastinal mass 6.5cm X 5.8cm on August 7, 2018. Like you, I was feeling overwhelmed at the lack of current research and the research that existed lumped thymomas and thymic carcinomas together (to get a bigger sample size) added to my frustration and fears. I was hoping it was a thymoma, but learned it was squamous cell thymic carcinoma. This path was sent off for further analysis which took another 3-4 weeks to result, but was able to tell us how receptive it would be to immunotherapy. The chemo regimen of carbosplatin and taxol had just fair results.

What I learned through my dad's surgeon, at least in our case, was that de-bulking was not an option with his type of tumor. What we were told by the surgeon was that he would not even attempt to remove the tumor unless he was sure he could safely get all of it. This being an aggressive tumor, he didn't want to leave any of it behind. To determine proximity, a cardiac MRI was ordered to try to "see" if it had invaded or nested too close to vital vessels, like the aorta. The surgeon was hopeful he could do the surgery, but after reviewing the cardiac MRI he and his team felt the tumor was too close to my dad's pulmonary artery and innominate vein for him to attempt to safely remove it. His fear was to open my dad's chest via sternotomy only to realize he could not do anything or worse attempt surgery and have a poor outcome. A poor surgical attempt would simply delay treatment for 5 weeks and that's barring any more time due to surgical complications.

Since thymic carcinoma is rare AND aggressive form of cancer, he consulted with cardiologist reading MRI, oncologist, radiation oncologist and his surgical partners to make the safest decision and determined the best course of action for my dad. We ended up not having surgery, but did have chemo and radiation. My dad's tumor has not spread and after radiation was notably smaller. He has since been started on Keytruda every 3 weeks. The disease is currently stable and we could not ask for more.

I would definitely find a surgeon who was not eager to cut, but one that was thoughtful and understood this type of tumor, meaning since rare they will need to review and learn about this very rare cancer. Time is of the essence. I never appreciated all the considerations that have to made until we were knee deep. We were fixated on surgery, but know we went down the right path.

Please feel free to ask any questions. You are not alone and treatment options are getting better with options in immunotherapy.

Gail
Greenville, SC

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@gailkattouf - First of all, thank you for your generous offer of consultation; those having an up-close experience with thymic tumors are almost as rare as the tumors themselves. I was also pleasantly surprised to hear of your "so far, so good" results, given your initial description of the situation. In my case, the tumor has grown very slowly and has been doing so over several years. This generally rules out more aggressive tumors. Additionally, my mass is approaching twice the size of your dad's mass and it has begun to cause more serious problems that are basically related to the size of it. It doesn't appear to be leaching or remote; it's just crowding everything and needs to come out. I'm on the right path I believe... but it's hard to tell when it's covered with so much red tape. đŸ™‚ Be well and please do expect me to take advantage of your offer as the need may arise.

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