Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear ASPS community members,
I wanted to share my experience with ASPS and as one who has overcome this disease. It was 34 years ago this spring that I was diagnosed with ASPS. I am now 47 years old and enjoying very good health.

The site of my primary tumor was my right thigh. I was stage IV at diagnosis with metastases in both lungs. (They actually didn’t discover the lungs mets until 4 -5 months after my diagnosis of ASPS. However, when they went back and reviewed my first scans they did see that lung mets had been there from the beginning). My primary tumor was removed surgically, followed by cobalt radiation treatments and aggressive chemotherapy. I was first treated with VAC (vincristine, atinomycin & cytoxan), then with adriamycin and finally cisplatinum. I also underwent two thoracotomies during this time to remove a total of 35 lung mets. Within a couple of months after the initial thoracotomies, approximately the same number of mets recurred in my lungs. I continued on chemo, however, my lesions were unresponsive. Treatment was discontinued and I was told to count each day as a blessing. Approximately 2 years went by with no improvement.

Then one day I went in for a routine CT scan and had remarkable results. All the lung metastases had disappeared; except for one. The largest lesion remained. For the next 8 years, this lesion was monitored and when it began to enlarge, I had my third thoracotomy. That was 22 years ago and I have been in complete remission ever since.

I truly believe in the beneficial effects of having a positive attitude and realize that this helped me considerably to get through such a difficult time in my life. I know how strong and courageous ASPS patients and their families are and my thoughts and prayers are with you all.

Best wishes,
Tammy

Dear Tammy, thank you for this extremely positive information. This is actually the second very similar case with the multiple ASPS lung mets I know about. The other one was Amanda Minderlein from Canada who had a resection of her big number of the lung metastases and then the recurrence, so she was give the second regimen of the chemo (experimental protocol of Celebrex+Vincristine) and lung mets stayed stable for 8 month and then she had to go off chemo due to the side effects and stayed on Celebrex for another year or so. Her lung metasases were followed by the CT scans and had regressed in number after significant period of time and then the second surgery was considered but surgeon was not convinced that it is needed. And consecutive scans have shown the regression of the remaining mets. She had a baby and is going to have another one - we all are happy for her! So my guess is that may be ASPS lung metastases are not sensitive to the chemo in the grown state when their vascular system and blood supply is already formed and we see when chemo is given for the existent lung mets it is almost uniformly ineffective. But when chemo is given after the surgery to remove the established lung mets for the recurrence - small just pop up mets - they may be more sensitive to the chemotherapy shortly after the lung surgery - ether is depends on the elevated VEGF as it si shown in other cancers that chemotherapy is more effective when is given shortly after the surgery - ether is depends on the samll size and different condition of the lung mets when they just appeared. They even look differently on the CT scans, I have read the report that small ASPS lung mets look non-specific on the CT scan but bigger ones - closer to 20 mm - are surrounded by the bunch of the dilated blood vessels. Also Dr.Nickerson has published his case with ASPS lung mets treated the same way you had - chemo therapy+surgery to remove multiple lung mets+ more chemo - the report is in the bibliography section of this web-site - and his patient is well and cancer free 10 years after the treatment.
So I wanted to thank you for this information and if you remember - after the surgery and lung mets recurrence when you continued on chemotherapy - did you have the growth or they were stable. My guess is that ASPS lung mets necrotic tissue dissolves slowly (as it grows slowly) and although the CT scan shows they are there - it may be the necrosis left after them. Also - do you continue to have a brain scans? There are the cases when brain mets were found decades after.

Dear Olga,
I am very happy to hear that Amanda Minderlein is doing so well! How wonderful that her remaining lung mets have regressed.

Thank you for your very interesting discussion regarding the sensitivity to chemo of established ASPS lung mets in their grown vascular state vs. small pop up mets.

As I recall, after the surgery, my lung mets that recurred were stable.

I had a brain MRI in 2003 that came back normal with no evidence of metastatic disease. I had wanted to have the brain MRI after I had read ASPS literature stating the there are cases when brain mets were found years later.

Best regards,
Tammy

Unfortunately the brain mets may be found after the very long period of the cancer free state, this is the article when primary was resected at the age 10 years, a lung metastasis at the age of 31 years, and brain and renal masses at the age of 43 year, the good part is that this patient is alive and well:

Alveolar soft part sarcoma: an unusually long interval between presentation and brain metastasis.
http://www.ncbi.nlm.nih.gov/entrez/quer ... ds=8253458

Dear Tammy,
Thank you so much for the encouraging and inspiring entry. I am sure that your personal case will give a lot of hope to our ASPS community.
I am not sure that one can deduce which treatments were beneficial for the cure. I agree that we should keep our eyes open on all the treatment options: chemotherapy, radical surgery and the combination of the two, and others. An unexplained regression of ASPS, could be due to a strong immune response against the tumor or to other unknown factors and I believe that as we learn more about the biology of ASPS we may come up with additional explanations and findings. The lesson from Tammy’s case is that patients need to be active, consider different treatment options including radical surgeries to make sure (as long as possible) that the tumor mass in their bodies is as low as possible. I join Tammy in her belief that a positive attitude is very important to get through this difficult time in a person’s life. Wishing you the best, Tammy.

Dear Group

Has anyone heard from Tammy or been following her story else where?
Just curios

Thanks in advance
Debbie

Dear ASPS community members,
I wanted to check in with an update on my status. I am currently 53 years old. I am in good health and remain symptom free.
Best regards,
Tammy

Hi Tammy, it is great to hear from you again and to find you in good health and symptom free, thank you so much for checking in and posting an update.

Hi Tammy,
Thank you so much for updating your condition. We're very encouraged by your good news. Keep on the good healthy and happy life

Hello Tammy

Very wonderful to hear from you. Your story is something that we all aspire to accomplish

When you get time could you get back to us how big your primary was that was removed
I apologize if I over looked that.

Thanks again for your time and input !
Sincerely
Debbie and family

Tammy ty for posting! I am so happy that you are doing well an it spreads hope an happiness when you post thank you <3

I have to comment here that although we do not know for sure what helped to Tammy to achieve this long survival, she underwent very aggressive chemotherapy with 2 the thoracotomies to remove the lung mets - one in between the chemotherapy courses and one years after. Probably some patients could have been treated the same way and have the same result despite the chemotherapy not being recommended to our patients now, I just do not know. I just do not see any other explanations here other that the chemotherapy worked and it just took time for most of the necrotic nodules to dissolve.

Olga,
have you ever wondered as i do if this *ASPS* is just the type for a wide range of ASPS variants?
I look at Ivan and I am others an i look as hard as I can an though its ASPS there are some strong differences..

Im just wondering if i am the only one that thinks its not just one or two types :/

Dear Group,
Thank you for your posts and comments, it's nice to be in touch again. In response to the question regarding the size of my primary tumor, I do not remember the precise measurements. I do remember that by the time it was surgically removed the mass had grown rather large. I first noticed the tumor when I was 12 yrs. old and felt a small lump about the size of a walnut on my right thigh. The mass was removed approx. 9 months later and by that time it had grown to the size of a small fist.
Best regards,
Tammy