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Extramedullary plasmacytoma

by PeterSydney on Mon Oct 12, 2015 11:45 pm

I was diagnosed in 2011 with a fairly standard IgG myeloma with the genetic mutations identified being in the “standard risk” category. Following CTD (cyclyphosphamide, thalido­mide, and dexamethasone) chemotherapy and an autologous stem cell transplant, I had a complete remission and a couple of years of clear blood tests. Earlier this year my paraprotein levels started to increase (although the level of plasma cells in my bone marrow was still quite low) and I went onto a CyBorD (cyclophosphamide, Velcade, dexamethasone) chemotherapy regimen with a view to getting the numbers down.

However, about six weeks ago, following extreme pain in my back / ribs, a lump about the size of a golf ball was found in my right lung, specifically between the lung and pleural wall, which was identified as a soft tissue plasmacytoma (as opposed to one on a bone).

Since then, it has grown fairly aggressively – spreading across the pleural wall. I’ve now started on a more aggressive DL-PACE (L=lenalidomide/Revlimid) chemotherapy regimen with a view to another autologous stem cell transplant in the next few months and probably an allo­geneic transplant as well. Initially we were looking at radiotherapy, but with the spread of the tumour it is becoming too hard to target and there was some concern of damage to the lung, which not be ideal heading into a stem cell transplant. So this has been put on hold.

Has anyone else had experience with extramedullary plasmacytomas?

I have read a few research papers and some of the things I have found:

  1. The behaviour of soft-tissue plasmacytomas is not well understood and the treatments are usually the same as for myeloma generally (with the exception of the use of radiotherapy)
  2. Generally the prognosis is not good (even worse than relapse with a bone-related plasmacytoma)
  3. There seems to have been an increase in the rate of soft-tissue plasmacytomas over the last ten years or so. There is some speculation this could be linked to the use of novel agents, which started being used around the same time, but there is no clear evidence to support this and it may just be that detection has improved with better scanning methods available.
  4. Some researchers have postulated a link between soft-issue plasmacytomas and scar tissue from previous surgery, although no broad study has been undertaken. This particularly interests me as I had a pleurodesis when I was 14 to overcome collapsed lungs. The procedure involved scarring of the pleural wall in my lungs.

PeterSydney
Name: Peter Mansfield
Who do you know with myeloma?: myself
When were you/they diagnosed?: 2011
Age at diagnosis: 45

Re: Extramedullary plasmacytoma

by JinMingDao on Tue Oct 13, 2015 11:38 pm

I have an extra medullary plasmacytoma in my jaw. I was initially on an Revlimid, Velcade, dexamethasone (RVD) regimen, and had an auto transplant last December. My cancer's returned already though, and today I got a CyBorD (cyclophosphamide, Velcade, and dexamethasone) treatment plan that I'll start next week, along with Zometa shots.

My pain is in my hips and legs, and it hurts to sit or stand too long. I'm just focused on getting through the chemo, and taking it easy at the moment.

Best of luck to you.

JinMingDao
Name: Kim
Who do you know with myeloma?: Self
When were you/they diagnosed?: April 2014
Age at diagnosis: 46

Re: Extramedullary plasmacytoma

by Cheryl G on Wed Oct 14, 2015 9:50 am

Hi Peter,

Sorry to hear about your extramedullary plasmacytoma. You seem to have done your homework, though, and your doctors also seem to be on top of things, in that they are treating the plasmacytoma aggressively with multi-drug combination therapy, which is the usual recommendation for what to do in such cases.

You may have done it already, but if you haven't, you may want to check some of the previous discussions here in the forum about extramedullary diseases. This link will take you to a list of some of them.

There are some comments from some of the Beacon's medical advisors that you may find helpful in this thread,

"Extramedullary plasmacytomas in eyes, spinal nerves" (started March 30, 2015)

and also in this one:

"Mom just diagnosed with extramedullary myeloma" (started March 1, 2013)

Some of the newest myeloma agents, such as Pomalyst and Kyprolis, have shown some success in patients with extramedullary disease. I am not sure if either of those agents are available yet in Australia (maybe Pomalyst is?), but there also have been trials that combine the two agents, and maybe one is still ongoing in Australia; here's a list of open myeloma trials in Australia, which you can check.

Another option, of course, would be taking part in a trial of one of the new immunotherapies, such as daratumumab. I saw in the list of trials that there are some involving daratumumab that are ongoing in Australia. However, daratumumab as a single agent may not be enough for you, but it's worth discussing with your doctors.

Of course, in a situation such as yours, it's particularly important to be under the care of a myeloma specialist. I suspect you are, but if that's not the case, now is definitely the time to see one.

Good luck, and please let us know how things go and if you have any more questions.

Cheryl G

Re: Extramedullary plasmacytoma

by JinMingDao on Wed Dec 02, 2015 8:12 am

Hello Peter,

Was thinking about you, and praying that things are going well. If you feel well enough, please share your progress. God bless.

Kim

JinMingDao
Name: Kim
Who do you know with myeloma?: Self
When were you/they diagnosed?: April 2014
Age at diagnosis: 46


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