Hello everyone,
I am a 23 year old who experienced a fleshy growth in my throat, near the Adam's apple, in June 2013, about the size of the tonsil itself. The growth, within a week, eventually came next to my tonsil and sat below the left tonsil. Time passed, and rather than checking it out sooner, I opted to let it stay. At the beginning of October 2013, I experienced the growth beginning to swell rapidly and it began to bleed slightly.
During this entire ordeal (June to October), I was asymptomatic. The moment the swelling happened, I rushed to the ER and the resident ENT suggested getting a CT scan. The initial scan showed a 22 x 15 x 19 millimeter soft tissue mass at the position of the left pharyngeal tonsil without extension into the adjacent parapharyngeal space. There was also a borderline enlarged left level II lymph node (12 millimeter). The differential diagnosis included infection and malignancy.
While at the ER, the doctors also prescribed antibiotics. The antibiotics actually helped in reducing the size of the tumor and at this point I was sent to follow up with an ENT. The ENT performed a routine exam and saw no issues other than the swollen growth. He then took a biopsy of the growth and asked me to follow up in 10 days. Time came and the results actually turned out to be a plasmacytoma.
It was then that the oncologist was brought in. Early December 2013, the oncologist ran the standard tests,
- serum protein electrophoresis came back negative with FLCs in normal range and ratio and no M-spike, although there was a high albumin level as well as calcium of 2.59, which I believe was because of being dehydrated when I took the test so early in the morning! The oncologist report suggests the mildly elevated total protein to be nonspecific and likely non pathological.
- bone marrow biopsy (flow cytometry) came back negative for lymphoma and, I believe, was negative for excess plasma cells (oncologist was happy with the report, so I assume all was well)
- 24 hour urine analysis showed trace proteinuria but nothing the doctors were concerned about since the electrophoresis was normal
- CT scan and skeletal survey showed no other lesions besides the left tonsil. The report mentions that "the hypodense left tonsillar mass was hypometabolic on the December scan" and that it had moderately decreased in size (because of the excisional biopsy). The hypodense part of the mass as seen on the first CT scan in October has completely disappeared and their is no findings to suggest multiple myeloma and a mildly enlarged, isointense left tonsil remains. One last point of note was a 12 x 14 millimeter, mildly enlarged left jugulodigastric node and borderline enlarged right jugulodigastric node was identified. All-in-all the oncologist, in the end, was happy with this report, too.
- PET scan showed a 2 x 1 cm of intense FDG uptake (SUVmax 10) at the tonsil with low-grade intake at the site of the bone marrow biopsy (which is slightly sore about 6 weeks later, but otherwise healed)
The final diagnosis came back to being a solitary extramedullary plasmacytoma of the left tonsil.
Treatment options were then discussed and the treatment recommended by the oncologist was radiotherapy, strictly, with the dose suggested for radiation therapy at 45 Grays over 25 fracs. The ENT had previously suggested surgery once the tests all returned negative. This has me stuck to which decision to make.
To my understanding, with the size of the tumor, the location and the tests results showing what they are, I felt that why subject myself to the adverse affects of radiotherapy when a resection is possible at the site (tonsillectomy). Options were also discussed to do a surgery followed by radiotherapy at the affected area with margins covering the salivary gland among others; however, if a complete resection is possible, I will make the call to stick with surgery only.
The literature I have read indicates that when complete resection is possible, the median survival rates are among those with radiation therapy or both and the progression rate to myeloma or other complications remain the same, regardless. The added issues of long term affects of radiation therapy have me worried.
The reason I am posting here today is mainly due to my age. I was advised to seek second opinions (third, fourth, etc) before subjecting myself to radiation therapy at all. If anyone has any advice on the case so far or any comments, please let me know. What decisions would you make? Currently, I am scheduled for surgery only and after pathology reports will make a decision on radiation therapy.
Sorry for a long-winded post. The dialogue on this particular topic is very limited, and I hoped to create more dialogue for this disease.
Thank you for your time.
Forums
Re: Extramedullary plasmacytoma of the tonsil
Hello,
I just wanted to wish you well in beating this thing. I don't feel qualified to give you any sort of suggestions, but I feel that you are definitely approaching this in a very good and methodical way.
Best wishes!
Alex
I just wanted to wish you well in beating this thing. I don't feel qualified to give you any sort of suggestions, but I feel that you are definitely approaching this in a very good and methodical way.
Best wishes!
Alex
-
dnalex - Name: Alex N.
- Who do you know with myeloma?: mother
- When were you/they diagnosed?: 2007
- Age at diagnosis: 56
Re: Extramedullary plasmacytoma of the tonsil
Thank you for the kind words, Alex, it really means a lot! 
Re: Extramedullary plasmacytoma of the tonsil
Thanks, rocco24, for posting such detailed background information about your case. This is not only a great way to get feedback relevant to your situation, but it also will be a valuable resource for other people in the future who might find themselves in a similar situation.
If you have not seen it already, you may find this recent journal article helpful.
Extramedullary plasmacytoma of the nasopharynx: A case report and review of the literature (full text available at no cost)
The extramedullary plasmacytoma in this particular case study is not in exactly the same place as yours, but much of the rest of the discussion in the article is generally about solitary extramedullary plasmacytomas, so it should be helpful to you.
We're not physicians (although the Beacon's Medical Advisors, who comment from time to time here, are), so what we'll say next should be read with that in mind. But it seems like you and your physicians have done the right thing by making certain that there is no evidence of myeloma elsewhere in your body. That's a critical issue to resolve at this point.
If the plasmacytoma really is just a solitary occurrence, there are basically three things you can do in the immediate term, and they are not mutually exclusive:
1. Have the plasmacytoma removed surgically
2. Have radiation therapy
3. Have systemic therapy with an anti-myeloma agent
You've discussed (1) and (2), because they are the most common options discussed in this situation, but, for completeness, it is worth mentioning that (3) is also an option (although there may be insurance restrictions that limit how and/or whether you can pursue it).
It seems like you are leaning to option (1), but you don't seem too enamored of combining it with (2) due to potential longer-term side effects of radiation therapy.
We understand that concern. What have your doctors told you, or what have you read, about the likelihood and seriousness of such side effects?
Again, many thanks for sharing with us your situation and its background, and please keep us updated as you continue through this process. All of us here in the forum will be glad to hear more and provide whatever help we can.
If you have not seen it already, you may find this recent journal article helpful.
Extramedullary plasmacytoma of the nasopharynx: A case report and review of the literature (full text available at no cost)
Abstract: Plasmacytoma is an extremely rare and discrete solitary mass of neoplastic monoclonal plasma cells. Extramedullary plasmacytoma (EMP) tends to occur during the fifth and seventh decades of life and is rarely diagnosed in younger patients. Only four cases of EMP have been previously reported in relatively young patients. Here we report a 15-year-old patient presenting with long-term nasal obstruction, who was found to have EMP of the nasopharynx. The patient was treated with surgery followed by radiotherapy with a satisfactory outcome. To the best of our knowledge, this study describes the the youngest individual with nasopharyngeal EMP to be reported in the literature.
The extramedullary plasmacytoma in this particular case study is not in exactly the same place as yours, but much of the rest of the discussion in the article is generally about solitary extramedullary plasmacytomas, so it should be helpful to you.
We're not physicians (although the Beacon's Medical Advisors, who comment from time to time here, are), so what we'll say next should be read with that in mind. But it seems like you and your physicians have done the right thing by making certain that there is no evidence of myeloma elsewhere in your body. That's a critical issue to resolve at this point.
If the plasmacytoma really is just a solitary occurrence, there are basically three things you can do in the immediate term, and they are not mutually exclusive:
1. Have the plasmacytoma removed surgically
2. Have radiation therapy
3. Have systemic therapy with an anti-myeloma agent
You've discussed (1) and (2), because they are the most common options discussed in this situation, but, for completeness, it is worth mentioning that (3) is also an option (although there may be insurance restrictions that limit how and/or whether you can pursue it).
It seems like you are leaning to option (1), but you don't seem too enamored of combining it with (2) due to potential longer-term side effects of radiation therapy.
We understand that concern. What have your doctors told you, or what have you read, about the likelihood and seriousness of such side effects?
Again, many thanks for sharing with us your situation and its background, and please keep us updated as you continue through this process. All of us here in the forum will be glad to hear more and provide whatever help we can.
Re: Extramedullary plasmacytoma of the tonsil
Thanks for the information, Beacon Staff, it helps immensely.
As for the third option you list, I did not know about it and have not spoken about it with my physicians. I will try to bring it up and see their response. How does the therapy with an anti-myeloma agent work? Does it also concern itself with using an external radiation beam or is it through the use of medicine? The reason I ask is because I am pretty booked on doing the tonsillectomy, but if this is feasible for post-op treatment, I can definitely discuss it with the doctors.
As for the long-term effects of radiation therapy, this is strictly due to my age. If this were to happen later in life, there would be no question that I would choose radiation therapy. The doctors have informed me that they will be using IMRT and will try to spare most of the trouble areas associated with head and neck radiation (including avoiding most of the salivary glands) by using the ability to decrease dosage in certain areas with IMRT. The radiation oncologist completely understands the other risks I am concerned about specifically the general ones, including trismus, change in tastes, dental and other caries, secondary cancers, but with IMRT at a dose of only 45 Gray, feels that the side effects should only be temporary. Of course, he cannot guarantee they will only be temporary, but everything in life comes with a risk, I suppose. As for the probability of problems occurring, they increase, cumulatively, with age as with any radiation treatment.
Some literature I have read does state that when complete resection is possible (ie. in the case of a tonsil), that local control rates are the same as those with radiation therapy (http://www.cancer.gov/cancertopics/pdq/treatment/myeloma/healthprofessional/page8). My goal with the tonsillectomy is that the surgery goes well with margins that are very good and I feel that this should be enough. The oncologists feel that due to the nature of the tonsil tissue, it is not enough to say that *any* margin is safe and that radiation is still recommended after surgery. Their stance is that surgery might get rid of the visible section, but with imaging systems being limited to detection of 8mm, it is impossible to detect whether or not the cancerous cells remain in other parts of the tonsil tissue.
This is a view I completely understand, but then I go to the fact that current imaging shows that the neoplasm itself is (at most) 2x1 cm, an acceptable number to remove via resection. Also, the workup required for myeloma was negative.
Another thing I forgot to mention in my last post was the B2 microglobulin levels were 1.1, well in the normal range, according to the lab used.
In summary, I plan on going ahead with the surgery and doing the workup required before starting radiation therapy. Following surgery, I will assess the pathology reports and make a call on radiation therapy.
As always, any advice or comments are extremely helpful and thanks everyone for simply reading this thread, even if you don't respond. I hope I have raised more awareness for this extremely rare situation.
As for the third option you list, I did not know about it and have not spoken about it with my physicians. I will try to bring it up and see their response. How does the therapy with an anti-myeloma agent work? Does it also concern itself with using an external radiation beam or is it through the use of medicine? The reason I ask is because I am pretty booked on doing the tonsillectomy, but if this is feasible for post-op treatment, I can definitely discuss it with the doctors.
As for the long-term effects of radiation therapy, this is strictly due to my age. If this were to happen later in life, there would be no question that I would choose radiation therapy. The doctors have informed me that they will be using IMRT and will try to spare most of the trouble areas associated with head and neck radiation (including avoiding most of the salivary glands) by using the ability to decrease dosage in certain areas with IMRT. The radiation oncologist completely understands the other risks I am concerned about specifically the general ones, including trismus, change in tastes, dental and other caries, secondary cancers, but with IMRT at a dose of only 45 Gray, feels that the side effects should only be temporary. Of course, he cannot guarantee they will only be temporary, but everything in life comes with a risk, I suppose. As for the probability of problems occurring, they increase, cumulatively, with age as with any radiation treatment.
Some literature I have read does state that when complete resection is possible (ie. in the case of a tonsil), that local control rates are the same as those with radiation therapy (http://www.cancer.gov/cancertopics/pdq/treatment/myeloma/healthprofessional/page8). My goal with the tonsillectomy is that the surgery goes well with margins that are very good and I feel that this should be enough. The oncologists feel that due to the nature of the tonsil tissue, it is not enough to say that *any* margin is safe and that radiation is still recommended after surgery. Their stance is that surgery might get rid of the visible section, but with imaging systems being limited to detection of 8mm, it is impossible to detect whether or not the cancerous cells remain in other parts of the tonsil tissue.
This is a view I completely understand, but then I go to the fact that current imaging shows that the neoplasm itself is (at most) 2x1 cm, an acceptable number to remove via resection. Also, the workup required for myeloma was negative.
Another thing I forgot to mention in my last post was the B2 microglobulin levels were 1.1, well in the normal range, according to the lab used.
In summary, I plan on going ahead with the surgery and doing the workup required before starting radiation therapy. Following surgery, I will assess the pathology reports and make a call on radiation therapy.
As always, any advice or comments are extremely helpful and thanks everyone for simply reading this thread, even if you don't respond. I hope I have raised more awareness for this extremely rare situation.
Re: Extramedullary plasmacytoma of the tonsil
Rocco24-
Thank you for posting and supplying such a complete history of what's going on and what research you have done concerning your condition and the options that have been presented to you.
My gut reaction to your plan and what you are being told is that at this point I would go conservative and have the surgery. Hold off on radiation. See what happens post surgery. It may take care of the problem completely. If a plasmacytoma returns later, then you could add the radiation into the mix.
I know that the radiation that is used with plasmacytomas is much less than with other solid tumors, but in the neck area any radiation has the potential for major short and long term problems. If you decide to have radiation it doesn't have to be done immediately, you wouldn't be able to have it until the surgical scars heal anyway. You would still have time to research radiation oncologists and try to find one who has experience in doing radiation for plasmacytomas.
I commend you on how thorough you sound like you are being in researching your options and what long term effects each of them may have for you. Please don't wait so long to seek help when you experience unusual physical problems. This is now really important since you have been diagnosed with a plasmacytoma. You may never have any more problems with this again, but then again you might.
The best to you in what you decide to do and with your recovery.
Nancy in Phila
Thank you for posting and supplying such a complete history of what's going on and what research you have done concerning your condition and the options that have been presented to you.
My gut reaction to your plan and what you are being told is that at this point I would go conservative and have the surgery. Hold off on radiation. See what happens post surgery. It may take care of the problem completely. If a plasmacytoma returns later, then you could add the radiation into the mix.
I know that the radiation that is used with plasmacytomas is much less than with other solid tumors, but in the neck area any radiation has the potential for major short and long term problems. If you decide to have radiation it doesn't have to be done immediately, you wouldn't be able to have it until the surgical scars heal anyway. You would still have time to research radiation oncologists and try to find one who has experience in doing radiation for plasmacytomas.
I commend you on how thorough you sound like you are being in researching your options and what long term effects each of them may have for you. Please don't wait so long to seek help when you experience unusual physical problems. This is now really important since you have been diagnosed with a plasmacytoma. You may never have any more problems with this again, but then again you might.
The best to you in what you decide to do and with your recovery.
Nancy in Phila
-
NStewart - Name: Nancy Stewart
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 3/08
- Age at diagnosis: 60
Re: Extramedullary plasmacytoma of the tonsil
I am not as young as you, but I am quite young for multiple myeloma and was diagnosed when I was 32 (full blown stage 2) I had a stem cell transplant and chemo. I am now 4 years into this, and have had many extensive talks wit my doctors about the what ifs, and making plans for future treatments if and when the needs arise. (I'm in remission since my sct 
)
The general opinion in my case, which is mostly due to my age (36), is to hold off on some treatments and "save" them for future. Radiations is one of those!
There is no right or wrong it is what is right for you! You need to find a doctor who you trust to make the educated choice based on your case, your age, the potential need for future treatments etc.
) The general opinion in my case, which is mostly due to my age (36), is to hold off on some treatments and "save" them for future. Radiations is one of those!
There is no right or wrong it is what is right for you! You need to find a doctor who you trust to make the educated choice based on your case, your age, the potential need for future treatments etc.
-
lys2012 - Name: Alyssa
- When were you/they diagnosed?: 2010, Toronto, Canada
- Age at diagnosis: 32
Re: Extramedullary plasmacytoma of the tonsil
Thank you so much for your replies, Nancy and lys2012.
This opinion makes a lot of sense to me. I'm praying to be in the group which has a one-time thing such as this and for it to be solved by surgery. However, in the instance something else does happen in the future, I understand that having other treatment options open rather than exhausting them all now makes more sense.
I will be bringing up these topics with my physicians at my next meeting.
Thanks again!
This opinion makes a lot of sense to me. I'm praying to be in the group which has a one-time thing such as this and for it to be solved by surgery. However, in the instance something else does happen in the future, I understand that having other treatment options open rather than exhausting them all now makes more sense.
I will be bringing up these topics with my physicians at my next meeting.
Thanks again!
Re: Extramedullary plasmacytoma of the tonsil
Hello again,
I thought I would provide an update as to my decisions and my condition.
I was very lucky in that I found an ENT that I trust very much and can discuss everything without them rolling their eyes at even the "dumbest" questions. I chose to undergo surgery (tonsillectomy) with this ENT and have been recovering for the last 2 weeks since. The tonsillectomy recovery was up and down, but that's a whole different topic and on day 14, I'm doing much better than I was on day 5.
Now, as for the results of the surgery, the ENT was happy with the results. He was able to get a 0.8 cm and 0.6 cm border around the plasmacytoma. He mentioned that after these results are formally out, my local tumor board *should* reconvene and discuss the next steps and whether radiation therapy is still required. My ENT really has gone the extra mile and contacted leading professionals across North America to voice their opinions. There is no consensus. Many do say, however, surgery alone should be enough, with radiation for anything in the future.
My next followup with the oncologist is early March. I have blood work to do before then and then will report back for the results of those results.
As far as the surgery go, what do people think about the margins achieved? If anyone has had an extramedullary plasmacytoma in the past (not necessarily on the tonsil, but anywhere), what margins were you able to achieve with surgery?
I am still hoping that the oncologist will look over the results and say yes, the results from the surgery are great and should be enough.
Thanks for your time.
I thought I would provide an update as to my decisions and my condition.
I was very lucky in that I found an ENT that I trust very much and can discuss everything without them rolling their eyes at even the "dumbest" questions. I chose to undergo surgery (tonsillectomy) with this ENT and have been recovering for the last 2 weeks since. The tonsillectomy recovery was up and down, but that's a whole different topic and on day 14, I'm doing much better than I was on day 5.
Now, as for the results of the surgery, the ENT was happy with the results. He was able to get a 0.8 cm and 0.6 cm border around the plasmacytoma. He mentioned that after these results are formally out, my local tumor board *should* reconvene and discuss the next steps and whether radiation therapy is still required. My ENT really has gone the extra mile and contacted leading professionals across North America to voice their opinions. There is no consensus. Many do say, however, surgery alone should be enough, with radiation for anything in the future.
My next followup with the oncologist is early March. I have blood work to do before then and then will report back for the results of those results.
As far as the surgery go, what do people think about the margins achieved? If anyone has had an extramedullary plasmacytoma in the past (not necessarily on the tonsil, but anywhere), what margins were you able to achieve with surgery?
I am still hoping that the oncologist will look over the results and say yes, the results from the surgery are great and should be enough.
Thanks for your time.
Re: Extramedullary plasmacytoma of the tonsil
Hey Rocco,
I saw your story and it reminded me of my case. I am 34 years old and had surgery for localized amyloid deposit on the back of my tongue, when the biopsy came back it was amyloid with plasma cell infilitrate. I went through the same rigorous tests you went through, and multiple myeloma was ruled out, but they diagnosed me with extramedullary plasmacytoma of the base of tongue. I did a PET scan and they found uptick next to where they did the surgery to remove the amyloid.
I just finished my 23 appointments two weeks ago. Side effects aren't too bad, because it was low dose compared to traditional cancers, but i haven't taken another pet scan yet to see if it is gone.
I would of rather did the surgery as well, but my ENT and the oncologist both recommended the radiation. After meeting with all these doctors no one can tell me what the danger of leaving the plasma cells without treatment will do to me. For example if I didn't have the amyloid build up, and needed surgery to remove it on the right side of my tongue, I would have never known that I had plasma cells on my tongue.
Anyway lets keep in touch since we have such a rare condition, and we have it so young.
Thanks
I saw your story and it reminded me of my case. I am 34 years old and had surgery for localized amyloid deposit on the back of my tongue, when the biopsy came back it was amyloid with plasma cell infilitrate. I went through the same rigorous tests you went through, and multiple myeloma was ruled out, but they diagnosed me with extramedullary plasmacytoma of the base of tongue. I did a PET scan and they found uptick next to where they did the surgery to remove the amyloid.
I just finished my 23 appointments two weeks ago. Side effects aren't too bad, because it was low dose compared to traditional cancers, but i haven't taken another pet scan yet to see if it is gone.
I would of rather did the surgery as well, but my ENT and the oncologist both recommended the radiation. After meeting with all these doctors no one can tell me what the danger of leaving the plasma cells without treatment will do to me. For example if I didn't have the amyloid build up, and needed surgery to remove it on the right side of my tongue, I would have never known that I had plasma cells on my tongue.
Anyway lets keep in touch since we have such a rare condition, and we have it so young.
Thanks
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