My sister's progress has had an unexpected turn. She is waiting to have a stem cell transplant, which due to a shortage of hospital facilities is scheduled for January. In the meantime, she was getting maintenance chemo to keep her in remission. Stem cells have been collected successfully, enough for 1 transplant, but they will expire in February.
My sister's tests show full remission with zero M-spike following several rounds of chemo. She was diagnosed in December 2015 following removal of an extramedullary tumour in her breast. Grade 3 multiple myeloma was then confirmed with numerous bone lesions in all part of her body, but no more extramedullary tumours. She responded well to treatment.
We thought she was doing great and were looking forward to the transplant. Unfortunately, she now developed a painful lump on her head. The doctors first thought it was a benign cyst, but now are pretty sure it is extramedullary disease. She will have a biopsy soon to confirm this, but it is most likely to be cancer-related, especially considering she had a soft tissue tumour previously.
We are very worried about this development and the risk of not being able to have a transplant in January because of that. Also, how is it possible to have this growth when in full remission?
I have read the info on this website on extramedullary disease as well as the forum postings, but still do not understand whether she will be able to have a transplant and what it all means. The literature states it means a more aggressive disease. We are really scared.
Forums
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
I wonder if what is described in the article below is what my sister is dealing with. She has developed soft tissue lumps on her head, but has not had biopsy yet. It sounds bad. Any insight would be really appreciated.
Reference:
Requena, L, et al, "Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases," Arch Dermatol. 2003 Apr;139(4):475-86 (full text of article)
Abstract:
Background: Specific cutaneous involvement in patients with multiple myeloma is very uncommon. It usually occurs in late stages of multiple myeloma as a reflection of increased tumor cell burden. We studied 8 patients with cutaneous involvement of multiple myeloma without underlying bony lesions and reviewed the literature on this rare dermatologic manifestation.
Design: We were particularly interested in the clinical course of patients with multiple myeloma and cutaneous metastases, including survival once metastases were detected and the possible influence of various forms of therapy. Our goal was also to identify the immunoglobulin and the light-chain type in these cases, with emphasis on any possible association between a particular immunoglobulin class and cutaneous involvement, as well as the histopathologic, immunohistochemical, and cytogenetic features of the neoplastic plasma cells involving the skin.
Setting: University department of dermatology, university hospital, and private practice.
Patients: Medical records and biopsy specimens from 8 patients with multiple myeloma and specific cutaneous lesions were reviewed.
Results: Cutaneous lesions consisted of multiple erythematous or violaceous nodules or plaques with a wide anatomical distribution. Histopathologically, 2 different patterns were identified: nodular and diffuse interstitial. Neoplastic plasma cells showed atypical features, and in 1 case they displayed a spindle shape, giving a sarcomatoid appearance to the lesion. Immunohistochemical studies demonstrated that neoplastic plasma cells were strongly positive for CD79a, CD138, and epithelial membrane antigen, and variably positive for VS38c and CD43. In each case the immunoglobulin profile and the light-chain type expression of the neoplastic cells were the same as those identified in the serum of the patients: 5 cases were IgA lambda; 2 cases were IgG kappa; and 1 case was IgA kappa. In cases 2, 3, and 4, polymerase chain reaction investigations revealed monoclonal rearrangement for IgH genes, whereas the investigations for human herpesvirus 8 and Epstein-Barr virus yielded negative results. Fluorescent in situ hybridization investigations in these 3 cases demonstrated that the cutaneous neoplastic plasma cells showed the deletion of the rb-1 (retinoblastoma) gene. Despite aggressive chemotherapy, all 8 patients died a few months after the development of cutaneous involvement.
Conclusions: In our series, there was a perfect correlation of immunoglobulin and light-chain type between the serum electrophoresis and the cutaneous plasma cells. Patients with multiple myeloma showed a short survival once cutaneous metastases appeared independently of the therapy. The deletion the rb-1 gene may provide prognostically relevant information to identify a high-risk subset of patients with multiple myeloma.
Reference:
Requena, L, et al, "Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases," Arch Dermatol. 2003 Apr;139(4):475-86 (full text of article)
Abstract:
Background: Specific cutaneous involvement in patients with multiple myeloma is very uncommon. It usually occurs in late stages of multiple myeloma as a reflection of increased tumor cell burden. We studied 8 patients with cutaneous involvement of multiple myeloma without underlying bony lesions and reviewed the literature on this rare dermatologic manifestation.
Design: We were particularly interested in the clinical course of patients with multiple myeloma and cutaneous metastases, including survival once metastases were detected and the possible influence of various forms of therapy. Our goal was also to identify the immunoglobulin and the light-chain type in these cases, with emphasis on any possible association between a particular immunoglobulin class and cutaneous involvement, as well as the histopathologic, immunohistochemical, and cytogenetic features of the neoplastic plasma cells involving the skin.
Setting: University department of dermatology, university hospital, and private practice.
Patients: Medical records and biopsy specimens from 8 patients with multiple myeloma and specific cutaneous lesions were reviewed.
Results: Cutaneous lesions consisted of multiple erythematous or violaceous nodules or plaques with a wide anatomical distribution. Histopathologically, 2 different patterns were identified: nodular and diffuse interstitial. Neoplastic plasma cells showed atypical features, and in 1 case they displayed a spindle shape, giving a sarcomatoid appearance to the lesion. Immunohistochemical studies demonstrated that neoplastic plasma cells were strongly positive for CD79a, CD138, and epithelial membrane antigen, and variably positive for VS38c and CD43. In each case the immunoglobulin profile and the light-chain type expression of the neoplastic cells were the same as those identified in the serum of the patients: 5 cases were IgA lambda; 2 cases were IgG kappa; and 1 case was IgA kappa. In cases 2, 3, and 4, polymerase chain reaction investigations revealed monoclonal rearrangement for IgH genes, whereas the investigations for human herpesvirus 8 and Epstein-Barr virus yielded negative results. Fluorescent in situ hybridization investigations in these 3 cases demonstrated that the cutaneous neoplastic plasma cells showed the deletion of the rb-1 (retinoblastoma) gene. Despite aggressive chemotherapy, all 8 patients died a few months after the development of cutaneous involvement.
Conclusions: In our series, there was a perfect correlation of immunoglobulin and light-chain type between the serum electrophoresis and the cutaneous plasma cells. Patients with multiple myeloma showed a short survival once cutaneous metastases appeared independently of the therapy. The deletion the rb-1 gene may provide prognostically relevant information to identify a high-risk subset of patients with multiple myeloma.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
I hope no one will have to deal with this kind of development in the future, as it seems quite hopeless, but in case it happens to somebody else, my account may be useful. So, I'll keep updating this post.
My sister has just received her new blood results and they show a relapse. There were no signs of myeloma in October with full remission, no M-spike detected. Now M-spike is 7.7 and on top of that there are signs of liver failure.
I have read that cutaneous involvement does mean aggressive relapse and bad prognosis despite treatment. We are in total shock. I am thinking that if my sister received her transplant in September when her cells were harvested, she could have avoided this relapse and could live. Now I don't know. And I don't know what to tell her to support, as she is not ready to die yet and we are not ready to lose her. It has not yet been a year since her diagnosis. It's just too early, even for multiple myeloma.
My sister has just received her new blood results and they show a relapse. There were no signs of myeloma in October with full remission, no M-spike detected. Now M-spike is 7.7 and on top of that there are signs of liver failure.
I have read that cutaneous involvement does mean aggressive relapse and bad prognosis despite treatment. We are in total shock. I am thinking that if my sister received her transplant in September when her cells were harvested, she could have avoided this relapse and could live. Now I don't know. And I don't know what to tell her to support, as she is not ready to die yet and we are not ready to lose her. It has not yet been a year since her diagnosis. It's just too early, even for multiple myeloma.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
Hi,
I can't offer any explanations, but I wanted to reach out and tell you how sorry I am about what your sister and whole family is facing. I'm not educated enough in the topic to provide any guidance, but know that I really feel for you - and just be there for your sister as much as you can.
I can't offer any explanations, but I wanted to reach out and tell you how sorry I am about what your sister and whole family is facing. I'm not educated enough in the topic to provide any guidance, but know that I really feel for you - and just be there for your sister as much as you can.
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torifrog - Name: Tori
- Who do you know with myeloma?: Myself - MGUS
- When were you/they diagnosed?: Sept 1 2016
- Age at diagnosis: 51
Re: Extramedullary disease on the head / skull
Thank you, Tori, it means a lot.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
I'm sympathetic to you and your sister and this seemingly aggressive relapse. 
I hope you can find some answers and hope in discussing her case with her doctors. The internet is a vault of knowledge, but unfortunately (or maybe fortunately) in the case of multiple myeloma, a lot of the info is out of date! I noted the article you posted is from 2003! A lot has happened since then!
Many of the newer therapies are better at treating cases such as your sister. Don't give up just yet, she can probably still have her transplant!
I hope you can find some answers and hope in discussing her case with her doctors. The internet is a vault of knowledge, but unfortunately (or maybe fortunately) in the case of multiple myeloma, a lot of the info is out of date! I noted the article you posted is from 2003! A lot has happened since then!
Many of the newer therapies are better at treating cases such as your sister. Don't give up just yet, she can probably still have her transplant!
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lys2012 - Name: Alyssa
- When were you/they diagnosed?: 2010, Toronto, Canada
- Age at diagnosis: 32
Re: Extramedullary disease on the head / skull
Hi Alyssa,
Thank you very much for your support. I do hope that with the advancements in treatment things can still be done to help my sister and maybe even to go ahead with transplant. Unfortunately, I cannot find any recent information on this particular issue. I have found a couple of people on this forum posting similar accounts, but then leaving the forum without reporting a follow-up, so they are either doing very well or not well at all. I hope for the former.
In the meantime, my sister has been evaluated by a panel of doctors yesterday.They are puzzled why she had such a development while in treatment. She is having a biopsy on a lesion on her chest on Thursday. She now has numerous lesions mainly on her head, but those on the head are bigger and the docs do not want to biopsy them due to high risk of bleeding or infection. They believe all the lesion are of the same nature. She also has a different looking lump of her arm, but they still think it is all the same thing. I don't know.
They are saying it is not a typical development for myeloma. There are three possibilities according to the doctors: cutaneous plasmacytomas (myeloma), another cancer, or skin reaction to treatment. It is most probably myeloma, as her M-spike has now re-appeared. However, we will only know for sure after the biopsy, so we are trying not to dwell too much on it now or play a guessing game.
I'll keep updating this post. Thanks again for your support. We do still have hope.
Thank you very much for your support. I do hope that with the advancements in treatment things can still be done to help my sister and maybe even to go ahead with transplant. Unfortunately, I cannot find any recent information on this particular issue. I have found a couple of people on this forum posting similar accounts, but then leaving the forum without reporting a follow-up, so they are either doing very well or not well at all. I hope for the former.
In the meantime, my sister has been evaluated by a panel of doctors yesterday.They are puzzled why she had such a development while in treatment. She is having a biopsy on a lesion on her chest on Thursday. She now has numerous lesions mainly on her head, but those on the head are bigger and the docs do not want to biopsy them due to high risk of bleeding or infection. They believe all the lesion are of the same nature. She also has a different looking lump of her arm, but they still think it is all the same thing. I don't know.
They are saying it is not a typical development for myeloma. There are three possibilities according to the doctors: cutaneous plasmacytomas (myeloma), another cancer, or skin reaction to treatment. It is most probably myeloma, as her M-spike has now re-appeared. However, we will only know for sure after the biopsy, so we are trying not to dwell too much on it now or play a guessing game.
I'll keep updating this post. Thanks again for your support. We do still have hope.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
Today my sister had a biopsy of one of her cutaneous lesions. They decided not to biopsy the ones on her head, and as she also had one on her chest they decided to biopsy that one to avoid having a wound on her head due to higher risk of infection or heavy bleeding. In the meantime we sent a photo of her lesions to a good friend who is a dermatologist and she said that the lesions did not look like any skin condition, but rather like tumors. So, it's most likely cancer.
My sister said that the biopsy procedure was very painful and she was glad they did not do it on her head. They took the whole lesion with the surrounding tissue. The doctor stated that the cutaneous lesions do indicate a more aggressive form of the disease. Considering that my sister was diagnosed when she developed a plasmacytoma in her breast following a removal of what seemed to be a benign cyst (I later read that soft tissue plasmacytomas often grow close to scar tissue), the doctor said it was to be expected that she might develop more plasmocytomas as her disease progresses. She, however, reassured my sister that they still hope to find a chemotherapy regimen to fight the tumors and, considering her previous excellent response, they are hoping to be able to still go ahead with the transplant.
The worry remains that the stem cells that have been collected may be destroyed if there is a delay, due to lack of storage facility. We are now looking at finding another facility where we could move her cells for storage. At the moment my sister is not paying for the storage, but if we have to move the cells it may not be affordable. It is a very worrying time for us. In Russia doctors tend to reassure patients even if the prognosis is not good. It does help my sister's morale, but leaves us in the unknown, always wondering if they really mean what they say.
My sister said that the biopsy procedure was very painful and she was glad they did not do it on her head. They took the whole lesion with the surrounding tissue. The doctor stated that the cutaneous lesions do indicate a more aggressive form of the disease. Considering that my sister was diagnosed when she developed a plasmacytoma in her breast following a removal of what seemed to be a benign cyst (I later read that soft tissue plasmacytomas often grow close to scar tissue), the doctor said it was to be expected that she might develop more plasmocytomas as her disease progresses. She, however, reassured my sister that they still hope to find a chemotherapy regimen to fight the tumors and, considering her previous excellent response, they are hoping to be able to still go ahead with the transplant.
The worry remains that the stem cells that have been collected may be destroyed if there is a delay, due to lack of storage facility. We are now looking at finding another facility where we could move her cells for storage. At the moment my sister is not paying for the storage, but if we have to move the cells it may not be affordable. It is a very worrying time for us. In Russia doctors tend to reassure patients even if the prognosis is not good. It does help my sister's morale, but leaves us in the unknown, always wondering if they really mean what they say.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
My sister's biopsy results are back. It confirmed aggressive proliferation and it is now clear that all those lesions are plasmacytomas. Besides numerous lesions on her head, she has one on her chest, one on her arm, and one on her back. They could not take a bone marrow biopsy from her chest bone, as it was too soft and virtually disappearing (falling through) at contact. It was the case when she was first diagnosed, but then with treatment and Zometa was strengthened really well. Now we are back at square one. So they had to drill into her hip bone.
She was immediately admitted and today they are doing some 'conditioning' treatments (IV saline solution, eye drops, and numerous tablets to prepare her organs for aggressive chemo). They are starting new second-line regimen tomorrow. My sister does not know yet what agents will be used and for how long.
She has been a real trooper up to now, being very positive and determined to beat the disease. Now she is very depressed and scared. She has been crying all day.
The doctors are still reassuring her, but said 'you have a big battle ahead of you'.
She was immediately admitted and today they are doing some 'conditioning' treatments (IV saline solution, eye drops, and numerous tablets to prepare her organs for aggressive chemo). They are starting new second-line regimen tomorrow. My sister does not know yet what agents will be used and for how long.
She has been a real trooper up to now, being very positive and determined to beat the disease. Now she is very depressed and scared. She has been crying all day.
The doctors are still reassuring her, but said 'you have a big battle ahead of you'.
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Gala - Name: Gala
- Who do you know with myeloma?: sister, LgA-k
- When were you/they diagnosed?: December 2015
- Age at diagnosis: 48
Re: Extramedullary disease on the head / skull
Have they talked about radiation for the plasmacytomas?That is what they did for my daughter.
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