So my father has, from what I can tell and what we've been repeatedly told, a very odd presentation of multiple myeloma. I'm going to try to keep this as short as possible but it will inevitably turn out to be quite long.
My father originally had a solitary plasmacytoma of his left sinus which was treated with radiation alone in 2005 when he was 42 years old. He has had high blood pressure since his 20's but is otherwise very healthy.
We live in Northwest Indiana and his treatment the entire time has been at the University of Chicago, which is about an hour and 45 minutes away.
Fast forward 10 years, starting in March of this year he had a loss of appetite and feeling of fullness. Blood work showed he was slightly anemic, so he had an endoscopy and colonoscopy done and was diagnosed with H. pylori and went on antibiotics. After 2 courses of antibiotics and nearly 4 months later, he had dropped from 165 lbs to 130 lbs and went to our family doctor. He originally was being seen by the clinic at his factory which is free of charge. Family doc immediately got a CT of his abdomen and found a large mass consuming his left kidney. Approximately 15 x 16 x 20 cm. He spent 2 weeks in the hospital because he was severely dehydrated and his other kidney function was very low. Luckily it seems to have fully recovered and his kidney markers are pretty good. He was scheduled for surgery as they thought it was renal carcinoma but because of his past plasmacytoma and an odd presentation on the CT they did a biopsy and found it was indeed another plasmacytoma.
He immediately started radiation on the tumor and after 7 weeks it had shrunk 85% and they felt the remaining mass was dead cells/fluid. He had a rather small IgA monoclonal spike in the beta region which slowly went down with radiation. After treatment was done his serum IgA was normal but still showed a small spike in the beta region. His oncologist felt he was was good to go and scheduled a CT for 3 months later. His radiation ended after the first week of September and a CT in October was clear. Additionally he had had a PET scan about 2 weeks into radiation treatment which was clear and his bone marrow has only 2-3% plasma cells. The cytogenetics and FISH also came back normal.
However by November he started to have pain under his left lower rib cage similar to the pain he felt from the mass. The mass never caused pain until about the last month before radiation. The doctors said his October scan was clean and the pain could be from a number of causes, including damage from the radiation. Toward the end of November he went to the ER near our home because the pain was getting worse and they did another scan. They had nothing to compare it to but the pathologist reported they could see the original mass but otherwise unremarkable. He was prescribed painkillers and sent home.
Mid-December he developed a lump on the top right side of his back. He went to our family doctor who did a biopsy and said he was almost positive it was another plasmacytoma. A few days later he was back in Chicago and had another CT, PET scan, and bone marrow biopsy. The CT and PET showed something like 9 new soft tissue masses which where all very active according to the PET scan. Including one on his left chest wall which near his spine. Bone marrow came back clean again with no myeloma cells, so doctor said there was no reason to do any genetic testing.
They started him on Kyprolis, Revlimid, and dexamethasone (KRD) by the first week of January, and that is what he has been on since. They are planning 4 cycles of KRD and then a single auto-transplant after that. His IgA spike continued to fall the entire time and his last blood test showed IgA, IgM, and IgG all below the range. Also, he no longer has the IgA spike in the beta region by electrophoresis. The lump on his back was about half the size after the first week and we couldn't feel it anymore by the 2nd week.
He's continued to work full time though the treatment and only has minimal side effects. Overall he feels pretty good. The pain is gone and he is back up to 155 lbs.
After a bit of nudging by both my mother and myself, we got him to agree to get a 2nd opinion. So just yesterday we went to Northwestern in Chicago and had a pretty long conversation with one of the doctors there. Not sure of the board rules as far as putting doctors names down, as I just joined, but this doctor did his fellowship at UAMS and seems to follow a similar strategy to "Total Therapy" (TT). He seemed like a great doctor and we all like him very much. He wants to hit my dad's cancer hard with VTD-PACE and a tandem transplant. He said we could do a PET scan after he finishes his second course of KRD next week and see how it has worked, which would tell us it's a good option for maintenance therapy. He assumes it's working well because the mass on his back shrunk so quickly, but he also said that's a factor in how fast it grows. If it shrinks fast then it grows fast. He had an MRI of his spine after the first month to make sure it wasn't something they needed to hit with radiation right away. The mass is still there but must be smaller because his pain in his side went away in the first week.
I'm sure the "To TT or not to TT?" question is probably pretty popular on this site and I'll admit I haven't spent much time on the forums. The past few months I've researched many different studies trying to find cases similar to my fathers. I think in this case the question of whether or not to do the Total Therapy is a bit different because how his multiple myeloma has emerged. From what I can tell, the benefits touted by TT are mostly seen in the low-risk group with a median survival time reaching past 10 years. And the obvious cons of having to go through a much more harsh treatment plan.
Both doctors agree his multiple myeloma must be treated as high risk. Although no genetic testing has been done, we know the presence of extramedullary plasmacytomas gives a much worse prognosis. However it's hard to find much information about newly diagnosed multiple myeloma with extramedullary disease but no bone marrow involvement. I've found a couple studies showing that with high-dose treatment and an auto stem cell transplant there was no difference in overall survival. I'm not sure what "high does treatment" refers to exactly in this study. Also I'm not sure what the comparison of Total Therapy is with something like KRD when we are talking about high risk? I've seen a few graphs of Total Therapy which shows the much worse prognosis when extramedullary disease (EMD) is present before stem cell transplant but the sample groups seemed to be quite small (n=66?) compared to the "no EMD" group and they don't show if it's newly diagnosed or relapsed multiple myeloma with EMD. I'm kind of thinking the vast majority of them were relapsed extramedullary disease and probably most if not all with bone marrow involvement.
S-E Lee et al, "Impact of extramedullary plasmacytomas on outcomes according to treatment approach in newly diagnosed symptomatic multiple myeloma", Annals of Hematology, March 2015 (abstract)
P Wu et al, "The impact of extramedullary disease at presentation on the outcome of myeloma", Leukemia & Lymphoma, July 2009 (abstract)
So if he continues his current plan of treatment and gets his stem cell transplant, maybe his prognosis isn't much worse than the average person with multiple myeloma. However, there are other studies that show having extramedullary disease before treatment means a much shorter overall survival even with treatment like Total Therapy. However they don't split up those who had EMD after relapse and those who had EMD at diagnosis. And I've not found anything about those who have extramedullary diseae but no bone marrow involvement at diagnosis.
There is also the case of quality of life. With Total Therapy the VTD-PACE and tandem stem cell transplants will probably make him sick for the next 8 months or so. 2 courses VTD-PACE, stem cell harvest, stem cell transplant, (this doctor said ~ 3 months inbetween), and then a 2nd transplant. Plus maintenance therapy which I would hope wouldn't be too harsh on him. So if his prognosis isn't very good either way, this could mean a rather large amount of time that he could instead be spending feeling fairly healthy as he has been for the last 2 months on KRD.
So let's say hypothetically if his extramedullary disease at diagnosis with no bone marrow involvement doesn't necessarily give him a poorer prognosis by using at least 1 stem cell transplant. Then I guess were back at the original "To TT or not to TT?" question. And that maybe if he's able to get a great response from Total Therapy that he has a good chance at living 10+ years. Or maybe standard therapy that he's getting right now, will be enough? I've looked at the Msmart newly diagnosed treatment guidelines by Mayo which is basically what his doctor right now (high risk). However the only spot they mention EMD is under the relapsed multiple myeloma, in which the course of treatment is then VTD-PACE.
Total Therapy would mean he couldn't work anymore and we'd have to figure something out with insurance as he would eventually lose his. Also Northwestern is about 30 minutes further drive and downtown is so much more of a hassle. These are the things he's mentioning when in reality they shouldn't be much of a factor at all.
Obviously a very tough decision and really no way of knowing one way or the other. Sorry about this being so long, I knew I would do that, but I'll go ahead and stop here. I know no one can make this decision for us, and I can't make the decision for him, but if anyone has any info at all please send it my way. Studies, personal experiences, similar stories, general multiple myeloma knowledge that we should know, anything and everything. It would be helpful to find someone else who has a similar story although that might be tough.
Also ask away with any questions that might help. I'm sure I've left a lot out. Other than still being a bit anemic all his other tests are within range and he's still quite healthy. And thank you for any help you can give!
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Re: Extramedullary disease only at diagnosis
Hi Skip13!
I have a similar story. I had a solitary plasmacytoma of bone, left femur, in 2009. No M-spike and a clean bone marrow biopsy. Treated with surgery and radiation.
In 2011 I had another solitary plasmacytoma. This time vertebrae L1. Again, the bone marrow biopsy showed no signs of myeloma. I was treated surgically and with radiation.
In November 2013 I had some problems with the stomach. It turned out to be multiple extramedullary plasmacytomas. Small amount of monoclonal cells in bone marrow biopsy, hence the diagnosis multiple myeloma.
I did four rounds of induction therapy with Velcade, Cytoxan and dexamethasone. Then followed by high dose chemo and a stem cell transplant. After 100 days, two consolidation rounds of Velcade, Revlimid and dexamethasone. Also some radiation. I'm in stringent complete remission since two years.
I live in Sweden. I feel great and work full time. I wish your father the best. Best regards,
Mattias from Sweden
I have a similar story. I had a solitary plasmacytoma of bone, left femur, in 2009. No M-spike and a clean bone marrow biopsy. Treated with surgery and radiation.
In 2011 I had another solitary plasmacytoma. This time vertebrae L1. Again, the bone marrow biopsy showed no signs of myeloma. I was treated surgically and with radiation.
In November 2013 I had some problems with the stomach. It turned out to be multiple extramedullary plasmacytomas. Small amount of monoclonal cells in bone marrow biopsy, hence the diagnosis multiple myeloma.
I did four rounds of induction therapy with Velcade, Cytoxan and dexamethasone. Then followed by high dose chemo and a stem cell transplant. After 100 days, two consolidation rounds of Velcade, Revlimid and dexamethasone. Also some radiation. I'm in stringent complete remission since two years.
I live in Sweden. I feel great and work full time. I wish your father the best. Best regards,
Mattias from Sweden
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Mattias - Name: Mattias
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Solitary plasmacytoma 2009. Myeloma 2013
- Age at diagnosis: 39
Re: Extramedullary disease only at diagnosis
Thanks for your story, Mattias! Glad you're doing well and definitely nice to hear a similar story turn out positive. A bit different, but seems like quite an odd presentation of multiple myeloma as well. As far as I know, all of my father's tumors are soft tissue only.
It seems logical that EMD that shows up after chemo would result in a poorer prognosis because it must have been cells which survived the initial chemo and therefore are somewhat more resistant. My hope is that, since my Dad has only had radiation thus far, that it's very possible his plasmacytomas will respond well to treatment and we can get a complete response as well.
His kidney tumor was so large that they said it could have been growing for many years. But then 2 months after radiation the EMD blows up ... Just seems very odd. Makes me wonder if surgery would have been a better option. Or even surgery / chemo / radiation, since it was his 2nd plasmacytoma. But I know they have their protocols on what's best based on studies. It just doesn't always make sense.
Also I'm under the impression that bone marrow involvement isn't needed for a multiple myeloma diagnosis if you have multiple plasmacytomas? The diagnostic criteria I've seen really doesn't describe my father's situation. I know he had asked his doctor what "stage" he is, but really he doesn't apply to any of them because he doesn't have any abnormal results. He's slightly anemic but >11 and his kidney function is a little below average but only because he only has 1 kidney now.
Also, as far as I know, there's no standard protocol for EMD at diagnosis. Especially without bone marrow involvement. Even Mayo's guidelines don't mention it.
So you haven't had any other maintenance therapy?
It seems logical that EMD that shows up after chemo would result in a poorer prognosis because it must have been cells which survived the initial chemo and therefore are somewhat more resistant. My hope is that, since my Dad has only had radiation thus far, that it's very possible his plasmacytomas will respond well to treatment and we can get a complete response as well.
His kidney tumor was so large that they said it could have been growing for many years. But then 2 months after radiation the EMD blows up ... Just seems very odd. Makes me wonder if surgery would have been a better option. Or even surgery / chemo / radiation, since it was his 2nd plasmacytoma. But I know they have their protocols on what's best based on studies. It just doesn't always make sense.
Also I'm under the impression that bone marrow involvement isn't needed for a multiple myeloma diagnosis if you have multiple plasmacytomas? The diagnostic criteria I've seen really doesn't describe my father's situation. I know he had asked his doctor what "stage" he is, but really he doesn't apply to any of them because he doesn't have any abnormal results. He's slightly anemic but >11 and his kidney function is a little below average but only because he only has 1 kidney now.
Also, as far as I know, there's no standard protocol for EMD at diagnosis. Especially without bone marrow involvement. Even Mayo's guidelines don't mention it.
So you haven't had any other maintenance therapy?
Re: Extramedullary disease only at diagnosis
Hi again!
Before I had my myeloma diagnosis in 2013, I was treated for solitary plasmacytomas in 2009-10 and 2011 with radiation and surgery only. The first solitary plasmacytoma resulted in a pathological fracture. I had a titanium rod inserted in my left femur. The surgery in itself was not intended to remove the plasmacytoma. Radiation took care of that. The majority of plasmacytomas are really sensitive to radiation therapy and that is the first line of therapy.
My second surgery splinted five vertebrae with two parallel titanium rods fixed with screws. Of course the orthopedic surgeons removed what they could of the plasmacytoma in L1, but once again the radiation did the trick.
I believe that almost 75 percent of patients experiencing solitary plasmacytoma of bone eventually progress to multiple myeloma, if not all ... if you live long enough ...
When you start out with a solitary extramedullary plasmacytoma, the prognosis is far better when it comes to cure and not progressing to multiple myeloma. Unfortunately, this seems not to be the case with your father.
I´m not on any kind of maintenance therapy. I even stopped taking IV bisphosponates (pamidronate) in July 2015 because all my numbers are ok and I have no bone involvement. The bisphosphonates have effects on the cell turnover in bone for many years after stopping treatment.
I´m also IgA like your father. Every third month I go to check up. The usual tests with immunofixation, electrophoresis (detecting and quantifying m-spike) and free light chains. Total blood count etc...
Of course it´s a bit disturbing to know that ones subtype of myeloma is very uncommon and it´s hard or impossible to find information of how to treat with the best possible outcome.
The Myeloma Beacon is a really good site and specially the different forums. It´s been a great source of information for me and also inspiring to take part of other people's experíences and stories.
Before I had my myeloma diagnosis in 2013, I was treated for solitary plasmacytomas in 2009-10 and 2011 with radiation and surgery only. The first solitary plasmacytoma resulted in a pathological fracture. I had a titanium rod inserted in my left femur. The surgery in itself was not intended to remove the plasmacytoma. Radiation took care of that. The majority of plasmacytomas are really sensitive to radiation therapy and that is the first line of therapy.
My second surgery splinted five vertebrae with two parallel titanium rods fixed with screws. Of course the orthopedic surgeons removed what they could of the plasmacytoma in L1, but once again the radiation did the trick.
I believe that almost 75 percent of patients experiencing solitary plasmacytoma of bone eventually progress to multiple myeloma, if not all ... if you live long enough ...
When you start out with a solitary extramedullary plasmacytoma, the prognosis is far better when it comes to cure and not progressing to multiple myeloma. Unfortunately, this seems not to be the case with your father.
I´m not on any kind of maintenance therapy. I even stopped taking IV bisphosponates (pamidronate) in July 2015 because all my numbers are ok and I have no bone involvement. The bisphosphonates have effects on the cell turnover in bone for many years after stopping treatment.
I´m also IgA like your father. Every third month I go to check up. The usual tests with immunofixation, electrophoresis (detecting and quantifying m-spike) and free light chains. Total blood count etc...
Of course it´s a bit disturbing to know that ones subtype of myeloma is very uncommon and it´s hard or impossible to find information of how to treat with the best possible outcome.
The Myeloma Beacon is a really good site and specially the different forums. It´s been a great source of information for me and also inspiring to take part of other people's experíences and stories.
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Mattias - Name: Mattias
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Solitary plasmacytoma 2009. Myeloma 2013
- Age at diagnosis: 39
Re: Extramedullary disease only at diagnosis
Skip-
Has your father considered maybe going for a 3rd opinion? Although he might get a 3rd suggestion for treatment, he might also get a consensus with one of the 2 opinions that he's received so far. Maybe going to the Mayo Clinic would be a good idea.
Your father certainly is in a small category of people. I wish him all of the best in making a good decision as to how he wants to progress.
Nancy in Phila
Has your father considered maybe going for a 3rd opinion? Although he might get a 3rd suggestion for treatment, he might also get a consensus with one of the 2 opinions that he's received so far. Maybe going to the Mayo Clinic would be a good idea.
Your father certainly is in a small category of people. I wish him all of the best in making a good decision as to how he wants to progress.
Nancy in Phila
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NStewart - Name: Nancy Stewart
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 3/08
- Age at diagnosis: 60
Re: Extramedullary disease only at diagnosis
Just wanted to update this thread in case anyone happens to find this thread in the future and want to know what happen.
My dad decided to stay with his doctor at the University of Chicago and they followed the M-smart protocol from Mayo. He did Kyprolis, Revlimid, and dexamethasone (KRD) until his auto stem cell transplant in June 2016. Had a hard time getting enough stem cells, but eventually he got enough. Stem cell transplant went off without a hitch. He's currently on maintenance therapy with Revlimid. He had a rash for a little while that eventually went away. His WBC is still a bit low and he has gotten sick a few times, but nothing serious. Also he's still anemic but feels well.
Had a PET scan in December and has monthly blood work that has all come back normal. As far as we can tell so far so good. He came back from the stem cell transplant pretty quickly and it's hard to think he'd still be feeling awful had he done the Total Therapy. We'll never really know which way would have ended better I guess, but it seems like this protocol is working well for now and we know they're hard at work looking for better treatments, only a matter of time before they find a cure.
Thanks everyone so much for the support.
My dad decided to stay with his doctor at the University of Chicago and they followed the M-smart protocol from Mayo. He did Kyprolis, Revlimid, and dexamethasone (KRD) until his auto stem cell transplant in June 2016. Had a hard time getting enough stem cells, but eventually he got enough. Stem cell transplant went off without a hitch. He's currently on maintenance therapy with Revlimid. He had a rash for a little while that eventually went away. His WBC is still a bit low and he has gotten sick a few times, but nothing serious. Also he's still anemic but feels well.
Had a PET scan in December and has monthly blood work that has all come back normal. As far as we can tell so far so good. He came back from the stem cell transplant pretty quickly and it's hard to think he'd still be feeling awful had he done the Total Therapy. We'll never really know which way would have ended better I guess, but it seems like this protocol is working well for now and we know they're hard at work looking for better treatments, only a matter of time before they find a cure.
Thanks everyone so much for the support.
Re: Extramedullary disease only at diagnosis
Hi Skip13,
I have just read about your father's experience and his very unusual presentation and wanted to share my experience to offer some confidence going forward.
I presented in a very unusual way in June 2013 with an extramedullary plasmacytoma in my lymph nodes in my left collar bone area and in my chest left side. I am being treated in the UK and many of the specialists here had never treated a patient with such a presentation. I had very little involvement in the bone marrow, some, but less than 10%. I was aged 33 at the time.
I was treated with two cycles of DT-PACE and then had an autologous stem cell transplant in October 2013. Those two treatments wiped cleared the bone marrow and took out 90% of the plasmacytoma. I was the put on weekly Velcade, thalidomide, and dexamethasone (VTD) for 9 months and then had radiotherapy on all the affected lymph nodes. This finally pushed me into remission in October 2014. I remained on weekly VD (thalidomide was stopped for blood clot reasons in my lungs) for maintenance. This has since been changed to fortnightly and I just started cycle 25. I remain in remission and work almost full time.
At the moment I am very optimistic and do not fear the myeloma. I hope things go well for your father.
Good luck.
Doug.
I have just read about your father's experience and his very unusual presentation and wanted to share my experience to offer some confidence going forward.
I presented in a very unusual way in June 2013 with an extramedullary plasmacytoma in my lymph nodes in my left collar bone area and in my chest left side. I am being treated in the UK and many of the specialists here had never treated a patient with such a presentation. I had very little involvement in the bone marrow, some, but less than 10%. I was aged 33 at the time.
I was treated with two cycles of DT-PACE and then had an autologous stem cell transplant in October 2013. Those two treatments wiped cleared the bone marrow and took out 90% of the plasmacytoma. I was the put on weekly Velcade, thalidomide, and dexamethasone (VTD) for 9 months and then had radiotherapy on all the affected lymph nodes. This finally pushed me into remission in October 2014. I remained on weekly VD (thalidomide was stopped for blood clot reasons in my lungs) for maintenance. This has since been changed to fortnightly and I just started cycle 25. I remain in remission and work almost full time.
At the moment I am very optimistic and do not fear the myeloma. I hope things go well for your father.
Good luck.
Doug.
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Beetlejuice54321
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