My husband has a very large tumour burst in his abdomen a year ago, it was removed but as it had burst the cells obviously spread and 6 months later he had 3 more appear in the abdominal cavity. It was originally called myofibroblastic sarcoma. He started chemo on doxirubicin and ifosfomide, of which the doxirubicin was dropped two months ago. The two of the tumours have shrunk and one disappeared completely, although another one appeared on his liver, this was contained. The last chemo session is scheduled for next Friday.
However, another tumour was shown during a routine scan on the outside abdominal wall, which was removed by simple surgery, with all margins clear. The biopsy has just come back and shows it is an aggressive synovial sarcoma and may need different treatment after the results of another scan next week.
We were blown out of the water by this news and everything had looked so good and positive. Reading about SS it seems that these sarcomas are usually in joints and in much younger people (my husband is 67), but having researched about the previous diagnosed sarcomas, I am not finding much to help me on the internet to explain this occurrence. Was the primary tumour misdiagnosed, how come a different type of sarcoma has occurred.... all these questions are running around in my mind. Now the tumour has metastasied is this likely to be more life threatening?
I would be so grateful if anyone out there could help please? Thanks so much.
(PS. we live in the UK)
Do you know what markers were used to conclude the tumor is synovial sarcoma. Did they find evidence of the translocation t(X;18)? In a case like this, probably getting a second opinion on the pathology would be wise. Synovial sarcoma in the abdomen is unusual but not impossible.
I am not at all sure what you mean. The original diagnosis of the first tumours took ages to come back from biopsy as it was originally thought to be GIST. This second biopsy went for genetic testing and the results were that it was SS. We are seeing the Professor in charge of my husband's case on Friday after the scan the previous day and I hope for more clarification then. What I hope is to gain enough information that I might ask informed questions of him. He did say it was very aggressive - and that has lowered our spirits.
Genetic testing probably means that they checked for the translocation t(X;18). It could be interesting to know which fusion gene he has (SYT-SSX1, SYT-SSX2 or another one). You may want to ask for a copy of the pathology report... Doxorubicin and ifosfamide chemo is standard for synovial sarcoma so his treatment was adequate even if the diagnosis was wrong. Surgery is usually necessary to get a complete remission. Is surgery not possible to remove the other tumors? By the way, there is a European clinical trial targeting synovial sarcoma in France that just started and may be worth looking into:
http://clinicaltrials.gov/ct2/show/NCT01469975?term=FZD-10&rank=1
Hello Bristol Girl,
I'm truly sorry to hear about your husband. My sister is now 44 and was diagnosed with synovial sarcoma 2 years ago, after 10 years of being diagnosed with desmoid tumors. She had a synovial sarcoma tumor burst last year. It had been attached to her lung. Tumors have appeared in several places and some are in areas which cannot be operated. She had one tumor grow so large that it cracked 2 ribs...they radiated that one, to hopefully shrink it. The pain of all these tumors and broken ribs has been unbearable so, she had a procedure to block 4 nerves and has a portable pain pump, to ease some of the pain.
Her prognosis is not good and she has given it an umbelievable fight. We just want her to be pain free, while she is still with us.
Best wishes.
Thank you for replying - I am afraid I am not good at the technical jargon although I do trawl the internet to find out everything I can about this wretched disease. I am now waiting to see the specialist on Friday with my husband, after the scan, to try to get a definitive prognosis and treatment plan.
I am so sorry to hear about your sister, I know this is a very rare cancer but that does not help in the fight against does it?
Best wishes
Wendy
Jessica Harvey said:
Hello Bristol Girl,
I'm truly sorry to hear about your husband. My sister is now 44 and was diagnosed with synovial sarcoma 2 years ago, after 10 years of being diagnosed with desmoid tumors. She had a synovial sarcoma tumor burst last year. It had been attached to her lung. Tumors have appeared in several places and some are in areas which cannot be operated. She had one tumor grow so large that it cracked 2 ribs...they radiated that one, to hopefully shrink it. The pain of all these tumors and broken ribs has been unbearable so, she had a procedure to block 4 nerves and has a portable pain pump, to ease some of the pain.
Her prognosis is not good and she has given it an umbelievable fight. We just want her to be pain free, while she is still with us.
Best wishes.
You are right, Wendy...the fight against this disease seems like a unwinnable battle. The rarer a disease is, the less information there is about it because there is not a lot of research on it.
Hang in there...Jessica
Hi Britsol girl,
My husband is now 51 and has synovial sarcoma tumors in his abdominal cavity also. His case is very similar to yours. The first one ( 5cm) actually burst also in May of 2009. The diaganosis was GIST. He took Gleevec from June through October of 2009. His original tumor was sent off for chromosone testing, because they want to see if he would qualify for a clinical trail to take Gleevec longer than FDA approved. Well....the same day that those test results came back - which showed that is was X:18 instead of X:9 or 11 that they were looking for - and then changed his diagnosis to synovial sarcoma - they discovered a large tumor developed in his pelvis (10cm x 15 cm). That tumor was removed, along with 2 other small (about 2cm) they found. He was scheduled to start chemo with Doxorubicin and ifosfamide chemo when he recovered from surgery. When he was finally healthy enough to start chemo (Dec of 2009) a ct scan showed 5 more tumors in his abdomin. They started the chemo and it did work. By the summer of 2010, the tumors were gone. A ct scan in Nov of 2010, then showed 4 more tumors in his abdomin. To make a long story shorter.....his chemo plan needed to be modified because of his intollerance for the full regimen, so the oncologist administered Doxorubicin first in 3 rounds, and then was going to administer ifosfamide after that. After the Dox, the ct scan showed that one of the tumors grew very large (it was originally about 3 cm and it grew to 12 x 15 cm) so they started Ifosfamide immediately, and he has been on Ifosfamide treatments since Jan 2011. As of now he is still getting Ifosfamide every 4 weeks, and he is down to 2 tumors that are both about 2 cm each. His oncologist is encouraged but I know he know he is a little surprised that Ifosfamide alone is keeping this disease under control because that is not what the normal is. We have discussed briefly with him about other treatment plans, but we have not yet gone very far with it. We will stay with the Ifosfamide until it no longer works.
I know this is a long explaination, and not very technical, but if I can help you in any way, let me know.
My husband is receiving treatment at Siteman Cancer Center/Washington University School of Medicine in St. Louis, Missouri.
Thought I might share this with you. These are the clinical trials for sarcomas at Washington Universtiy School of Medicine. http://www.siteman.wustl.edu/ProtResult.aspx?id=625&mid=70
I don't understand all the medical language, but if you may be able to use this information.
Thank you so much for your reply Debbie - we saw the oncologist yesterday and he confirmed that the original 3 secondary tumours are still shrinking so he gave the goahead for the final of the 6 chemo sessions, which in November were reduced from doxirubicin and ifosfomide to just ifosfomide. But in the abdominal cavity there was another tumour which has grown from 9mm to 14mm in a month and some (maybe 3) very small nodules which have appeared and are cause for concern.
Therefore he has said that after the chemo ends on 9 March my husband is to have two months off treatment and have a full body CT scan in the middle of April and see the Professor two days later for the results and make a decision then depending on the results. He said they are totally perplexed by the non-reaction of the new secondaries to the chemo but there are two drugs he can think of he may use: trabectedin or another which is still in the research state. which is a pill, it began with an 'r' but I cannot remember exactly the name. He favours the latter course again depending on the scan results. You husband's history sounds very similar to my husband's and if you have no objection may I pass on the details to the Professor in the hope there may be some cross-Atlantic discussions which may help both the men.
Thank you again for taking the time and maybe we can stay in touch.
Your husband's case is very similar. The only thing that concerns me is that they want to wait to see how big the tumor will get. When Kevin's tumor started growing, it cause severe pain and I know his doctor was concerened as to how fast it grew and was somewhat pleased that the ifosomide slowed the growth and then has continued to decrease the size of the tumor.
You can share this information with your doctor. If your oncologist would like to contact Kevin's doctor, his name is Dr. Douglas Adkins. He is with the Washington University School of Medicine in St. Louis, Missouri. The cancer part of this school is called Siteman Cancer Center. So you may google it to find the link. I will try to post a link. This is a research facility, so I see no reason why they would not share information about standards of care. Kevin's doctor, Dr. Adkins, is not longer accepting new sarcoma patients, although his partner, whom he trained, Dr. Brian Van Tine is taking all the new sarcoma patients. So now all their information on the web has Dr. Van Tine's name on it.
this is the link for the sarcoma clinical trials being administered by Dr. Van Tine.
http://www.siteman.wustl.edu/ProtResult.aspx?id=625&mid=70
this is the link for Siteman Cancer Center
http://www.siteman.wustl.edu/
I had the opportunity to meet him at an awareness event for rare sarcomas a few weeks ago. He mention to me that one of these trial just opened up and I believe that the drug that they are finding good results with starts with a "d" ....so looking at this link, it might be Dacarbazine . We actually talked at a restaurant for about 20 min. I had mentioned that we really don't know what we could do if the ifosomide quit working, and he was very postitve and said that there definitely were more options for treatment.
I wish you and your husband the best in this fight. If I can help you in any way, please let me know. I would like to keep in touch. This fight is hard feeling like you are the only one out there.
Thank you for your reply - yes I too would like to keep in touch. This can be a lonely world sometimes, especially as a carer watching someone we love go through all this stuff. When this chemo round is finished on March 9 it will be a waiting game, which is not good!! I have made a note of Dacarbazine and will talk to the doctors about it when I get to see them again.
I hope all goes well with you and your husband.