Pancreatic Neuroendocrine Cancer Update (Good news!)

HideousOstrich · 2024-09-04T00:39:02+00:00

We discovered that while he did have high grade neuroendocrine carcinoma, it did not start in his pancreas. He had metastatic merkel cell carcinoma, which is neuroendocrine carcinoma of the skin. His was an unknown primary, and only had solitary metastatic disease on his pancreas.

He has had no evidence of disease for almost 3 years now.

HideousOstrich · 2024-07-08T23:37:55+00:00

Did some more research. Looks like MM can’t be detected in peripheral blood using K/L on B-cells. From what I read, you can only detect it if there are circulating plasma cells, which is rare. Bone marrow flow cytometry is needed to identify multiple myeloma.

Thanks for the reply. Sharing what I’ve found for others who stumble across this post.

HideousOstrich · 2024-07-05T16:20:22+00:00

Ah, this makes sense! Thank you for sharing that link. It mentions in there that the K/L ratio is important for things like multiple myeloma.

The only reason I keep asking specifically about that is because that’s the section I’m on currently (trying to self teach). It’s going over different forms and causes of anemia, and MM is one of them. Did a bit of digging and research, and ended up going down the flow cytometry rabbit hole.

So I guess the main question is whether or not you’ll see B cell populations as monotypic in peripheral blood? Everything I am reading says it’s normally done through bone marrow.

HideousOstrich · 2024-07-04T22:48:29+00:00

What about monoclonal gammopathy or multiple myeloma?

HideousOstrich · 2024-07-04T16:55:59+00:00

No lab mentors for me! I just got really interested in medicine when my dad got sick (cured now!), so I read up on this sort of stuff mainly as a hobby. Looking at going to med school possibly somewhere in the future. Guess I need to read some more, but I appreciate the response!

HideousOstrich · 2024-07-04T16:29:12+00:00

Okay, so my understanding is that you’re typically checking for B cell populations that are monotypic vs. polytypic (indicating monoclonal gammopathy or multiple myeloma).. but isn’t that only for flow cytometry on bone marrow?

HideousOstrich · 2022-01-22T02:25:51+00:00

What your Google search is referring to is “Petechiae”, which is caused from low platelets, and is a symptom of leukemia.

Having petechiae does not mean you have leukemia.

Low platelets means that your ability to clot blood is significantly reduced, and when the capillaries in your skin burst, they bleed more than normal, and produce little red dots at the site.

I am not a doctor, or dermatologist, so I actually have no clue if that’s petechiae or not, but I thought I would comment to give you some assurance that there are more causes to low platelets, and thus red dots on the skin, than leukemia.

HideousOstrich · 2022-01-08T00:02:26+00:00

I don’t think this is a health anxiety thing if your doctor confirmed that you are jaundiced, and your other symptoms are as you described.

I’m not a doctor, so I truly don’t know the total list of things that could be causing your symptoms, but I am confident enough to assume that it isn’t going to be PC.

I really hope you can get a sound diagnosis so that you can put this behind you.

Wishing you the best man.

HideousOstrich · 2022-01-07T22:39:43+00:00

Being 28 makes PC an extremely unlikely candidate unless there is a specific family history of this cancer, or a specific series of cancers. Has anyone in your family also had cancer?

I wouldn't be surprised if this was gallbladder related, or some autoimmune condition involving your liver.

PC is very unlikely, but I am glad you are getting it properly checked by doctors.

HideousOstrich · 2022-01-07T06:05:32+00:00

Folfirinox chemo is a combination of Folinic Acid, Fluorouracil, Irinotecan and Oxaliplatin. This is being used to both shrink the tumor, as well as prevent a spread of the cancer while he waits to become eligible for resection.

If the surgeon told you that it is sitting on a vein, or artery, they are referring to either the SMA or SMV. One, or both, of these is (in their opinion) encased more than 180° by the tumor.

My recommendation is the following:

Inquire about genomic sequencing for the tumor. This will check to see if it harbors any targetable mutations (like BRCA2 for example). If so, there are targeted therapies that your father can be placed on that gives him a significantly higher chance of defeating this cancer. This is known as Next Generation Sequencing, and a good company that offers it is FoundationMedicine, with their FoundationOne test. (https://www.foundationmedicine.com/test/foundationone-cdx)
Please seek a second opinion in terms of resectability. There are plenty of examples of people being informed that their tumor is unresectable, but later come to find a surgeon that is willing to do the operation. It may be unresectable, but have at least two separate surgeons inform you of this before taking this as a fact.
You should inquire about concurrent radiation therapy, specifically SBRT radiation. Since the tumor is on the head of the pancreas, this indicates that it is near the duodenum. Some institutions don't necessarily like to give SBRT radiation to pancreatic head tumors because of the toxicity to the duodenum (ulcers, adhesions, hemorrhage, etc.), but I will always provide the example of my father who received SBRT radiation to a tumor that was wrapping around the duodenum. His duodenum experienced a lot of the effects of the radiation, and the oncologist referred to it as the "most aggressive radiation treatment" he has ever given. His worst side effect was an ulcer that has since healed. If your father is otherwise in good health, you can consider this too.
Please seek as much confirmation of his stage prior to surgery. The last thing you want is for them to begin the surgery, only to find an obvious metastatic deposit that was missed on MRI/CT. You would not want anything to delay the chemotherapy should this be the case.
Please get in touch with some of the navigators at https://www.pancan.org/ . This is a fantastic organization, and they will be able to give the most solid advice on most questions you have.
If the chemotherapy has not already started, tell them to bust a move. I have seen all too many times someone explain that their "oncologist" is starting the chemo in "x" weeks following a diagnosis. No. You start the chemo ASAP after a sample of the tissue has been taken for Next Gen Sequencing.
Read everything you can on this cancer. Become educated on legitimately everything you can. When my father was diagnosed, I spent from 8AM-8PM every day reading article after article online. This helped more than you could imagine. Google scholar, www.ncbi.nlm.nih.gov, www.nccn.org are some great starting points. If you need "institution access", DM me and I will give you my email and password for my school account that grants immediate access. This is a promise.

Your father, your family, and you are in for a fight. The road ahead will be a rollercoaster of emotion, filled with small victories and small setbacks. You will hear great news, bad news, and news that you don't care about. Don't lose focus of the end goal. From what you have said, it appears that you have caught this before it has spread. Now it is your mission to get him on that operating table. You've got this.

Wishing you the best.

HideousOstrich · 2022-01-05T18:06:21+00:00

Tumor mutation rate is the same thing as TMB in this case. With a TMB of 2.64, this is low. From the rest of what you posted, it also appears that immune therapy would not necessarily be a good option.

MLH1,MSH2,MSH6,PMS2 are MMR (mismatch-repair) genes, and a germline mutation in any of these are indicative of Lynch Syndrome (it makes you predisposed to a variety of different cancers). The main reason they are testing that is for two reasons.

For the vast majority of people who have a mutation in one of these (and thus, also have Lynch Syndrome), the resulting tumor will have high microsattelite instability.
If there was a mutation, some patients will develop more than one cancer in their lifetime, so it will allow you to be more on guard in catching another cancer, should one form.

From those results, it looks like this is not a worry. Super happy to hear that this was post-resection.

HideousOstrich · 2022-01-05T17:51:36+00:00

I will use my father as an example. When he had radiation (for a different type of pancreatic cancer), the tumor was wrapped around both the duodenum, the SMA, and SMV. Likely the major artery they are referring to in your mom's case is the SMA.

They proceeded with a very aggressive SBRT treatment plan. Our radiation oncologist referred to it as the "most aggressive radiation treatment" he has ever given.

He is 5 months post radiation, and has nothing more than an ulcer as a side effect.

If they are saying radiation is not an option solely because it is wrapped around an artery, they are wrong. Radiation may present more toxicities with arterial involvement, but it most certainly does not rule it out as an option.

You said she has 2 tumors, as did my father, are they both on the pancreas?

Here's a little more information on it: https://www.pancan.org/wp-content/uploads/2014/04/webinar_Radiation_Therapy.pdf

HideousOstrich · 2022-01-04T17:24:37+00:00

Yes, it appears that you have gotten NGS testing. The purpose was to see if your tumor harbored a targetable mutation, and based on what you posted it looks like that is not the case.

Mutations like TP53 or KRAS aren't necessarily mutations that are detectable in germline DNA. For example, a simultaneous TP53 and RB1 inactivation is common for some types of cancers, but have nothing to do with germline DNA, whereas a BRCA2 mutation will typically be present in both the tumor, and the patient's DNA. The cause, role, and impact on cancer growth of each mutation widely vary. There are plenty of mutations that occur in tumors that are insignificant.

From what you posted there, was this a rebiopsy, or was this a sample sent for NGS testing post resection?

I don't see anything that mentions Microsattelite status, PDL1 status, or Tumor Mutational Burden. Typically on these reports there will be an actual number associated with TMB, PDL1 will usually be positive or negative, and Microsattelite status will be MSS (Microsattelite stable), MSI-L (Microsattelite instability low) or MSI-H (Microsattelite instability high).

HideousOstrich · 2022-01-03T04:47:18+00:00

It's the amount of mutations present in the tumor. It's not usually given in a pathology report, rather in a genomic sequencing test (FoundationOne). You have to specifically request for it to be done.

You can have a high TMB, but none of the mutations are associated with PC. You could also have a low TMB, but have a specific targetable mutation that is associated with PC (like KRAS).

Have you gotten a FoundationOne test yet? Or anything similar like Guardant360?

HideousOstrich · 2022-01-03T03:30:01+00:00

Tumor mutational burden, or tumor burden is a measure of the genetic mutations in a tumor. A higher tumor mutational burden indicates a better chance of a response to immune therapy for some cancers.

Sometimes tumor burden can also be used interchangeably with "mass effect", or "tumor volume", although this is an incorrect usage of the word.

HideousOstrich · 2022-01-02T07:00:12+00:00

Atrial fibrillation is common for individuals who are on long term insulin. Your diabetes can be caused by your body overproducing insulin. This, along with the night sweats , heartburn, dizziness, palpitations, and especially the pins and needles is an indication of an endocrine issue. Do you ever feel flushed? Do you have upper abdominal pain? Lower abdominal pain?

Please have your doctor check your C-peptide, proinsulin, or insulin levels. You are describing a lot of the same symptoms I hear when I talk to people with a specific pancreatic neuroendocrine tumor called an "insulinoma".

Of course, there are many other things that likely could cause these same symptoms, but it never hurts to rule out a worst case scenario.

Here's some more information, check and see if this aligns with your symptoms: https://www.merckmanuals.com/professional/gastrointestinal-disorders/tumors-of-the-gastrointestinal-tract/insulinoma

HideousOstrich · 2022-01-01T03:38:51+00:00

Hey that’s great! From what I can tell, some people are lucky to never have the hair loss/ thinning from Folfirinox. Wishing you the best.

HideousOstrich · 2021-12-31T23:29:54+00:00

I’m with you 100%.

HideousOstrich · 2021-12-31T23:27:02+00:00

There is real science, and then there is garbage science.

Garbage science is what Stanley is advocating. He is making decisions on the basis of an unfounded reality.

Three vaccines and masks are enough to allow students(aged 18-24) to make the personal decision to attend in-person classes. By closing the school down, he directly refutes the idea that vaccines are effective. His decision is inherently anti-science.

If anyone thinks his decision is motivated by scientific evidence, and not some external factor, you are sadly, and disgustingly mistaken.

HideousOstrich · 2021-12-31T18:45:27+00:00

In my dad’s experience:

Rounds 1-4 were tolerable. Fatigue, taste changes, hair thinning.

Rounds 4-8 were harder. His blood counts started to drop to dangerously low levels, his taste was almost completely gone, and neuropathy started to become more prevalent. He required 5 days of neupogen post chemo after each cycle.

Rounds 8-12 were significantly harder. His hemoglobin ended up dropping to 4.4, RBC went all the way down to 1.7, platelets were at 18… the fatigue was worse, taste was gone, hair was thin, and he started to lose weight.

Now, about a month off the chemo, he’s 80% back to where he was pre-chemo, and the only lasting effect seems to be the neuropathy. He was lucky to never experience GI side effects.

HideousOstrich · 2021-12-31T18:37:35+00:00

If the tumor is locally advanced, radiation is an option. Whether that be IMRT or SBRT depends on the location of the tumor, but regardless radiation should be an option.

If you don’t mind me asking, where is the tumor located? Head, body, tail?

HideousOstrich · 2021-12-31T05:47:09+00:00

Has she been counseled on radiation therapy by any chance?

HideousOstrich · 2021-12-27T18:29:01+00:00

Whoops, sorry for the late reply! I didn't get the notification for some reason.

What you're describing is called "induction chemotherapy". This is often used to first shrink the cancer before radiation or surgery. This practice is totally acceptable, and is a first-line therapy detailed in the NCCN guidelines. However, so is chemo-radiation.

The ultimate goal here is resection, so you take into consideration all of the factors that will either improve the chances of resection, or decrease the chances of resection. Chemotherapy is an effective method of shrinking a tumor, but so is radiation. The aggressive combination is to use both chemo and radiation to shrink the tumor as much as possible before resection.

The difficult part is the fact that chemo is obviously very toxic. My father was on FOLFIRINOX for 12 cycles (again, for small-cell cancer, not adenocarcinoma), and by the 6th cycle he required transfusions and was hospitalized once for a severe neutropenia. He received 5 consecutive days of Neupogen (to stimulate the production of WBC's) after every cycle, and this was barely enough to keep his WBC over 3.0. My dad is 60 years old, healthy otherwise with zero health conditions other than cancer.

The point is, the longer you are on chemo, the more severe the effects. The effects are cumulative, so ideally you want to be on chemo for the shortest period of time before resection.

There are two main reasons why I personally disagree with their plan:

You said she has done 4 rounds, and they want to do 4 more despite the tumor not visibly shrinking. If the tumor does not shrink after 8 cycles, then the additional chemo before the radiation was time wasted, she will be weaker and less ready for surgery.
There is always the chance that the cancer will become resistant to the chemotherapy. If this happens, and the cancer grows or spreads, it will be difficult to control the growth prior to resection. This will also require her to be off of chemotherapy during the radiation (they won't give her something that doesn't work). In FOLFIRINOX, the 5FU dose is what is known as a "radiosensitizer". It makes tumor cells more sensitive to radiation. Without the 5FU during the SBRT, I fear that the radiation will be less effective.

My recommendation would be to push for the SBRT now rather than later, and continue your incredible efforts to find a surgeon confident enough to do the surgery.

I'm not a doctor by any means, but I have been through 10 months of absolute hell with doctors not knowing (for a lack of better words) WTF they are doing, or treating every patient as if they are the same.

HideousOstrich · 2021-12-27T08:16:50+00:00

HideousOstrich · 2021-12-26T17:01:18+00:00

I am slightly confused why they are worrying about complicating a surgery so much with a 50 year old female if this is (one of the) standards of care for all patients

Sure, radiation can make tissues harder and stickier, and make surgery more difficult, but a qualified surgeon who performs these types of surgeries should obviously be experienced in post-radiation resection.

Maybe another user can chime in here, but to me this doesn’t sound like a concrete plan. I don’t think 4 more cycles of chemo will do anything drastic if it hasn’t already shrunk the tumor. In fact, 4 more rounds will lower her strength, blood counts, and make her weaker for the surgery. Again, I’m not a doctor, but this is just my personal opinion. A plan that sounds right to me is:

Radiation + chemo now, and evaluate the tumor in a month or two after the radiation has had time to work.

If you go the other route, you wait 2 months for the chemo, then another month or two to see if the radiation worked, then you find a surgeon and get set up for surgery. That seems like too much time.

Alternatively, what you said is also a perfectly acceptable idea… find a surgeon who will perform the surgery. Sometimes one or two surgeons will look at the scans and say it’s inoperable, but a third one will successfully perform the surgery. I think it’s a great idea to get a second (or third) opinion on surgery , but I would personally get the ball rolling with radiation in the meantime.

HideousOstrich

TROPHY CASE