Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello everyone.
Our daughter was diagnosed with ASPS in the summer of 2003. Shortly after that she started an interferon alpha 2b treatment of 5 MIU (million units) daily. This dose dramatically reduced her white blood cells counts. She stopped the interferon treatment for one month in September 2003 and had an operation to remove her primary tumor that was located on her shoulder.
In October 2003 she resumed the interferon treatment at a dose of 3 MIU daily. We found that injection of the interferon before bedtime and drinking lots of water helped to reduce side effects of the treatment.
The interferon treatment was continued for 13 months. During that time we observed growth of 1-2 lung mets in the right lung.
The interferon treatment was stopped in December 2004, and in February 2005 our daughter went on a surgery to remove the lung mets. Fifteen tumors larger then 3mm in her right lung were resected.
The recent CT scans from July 2006 show no new growth in the right lung and one met in the left lung that doubled its size to 6mm from 3mm in January 2006. We are now considering an operation to the left lung to remove all the mets larger than 3 mm.

Best Wishes to all of you.

Hello everyone

Three and half years have passed since our daughter was diagnosed with ASPS. Her primary tumor was removed from the shoulder three months later. Then, two years ago, 15 tumors larger then 3 mm were removed from one of her lungs. Now we decided to clear also the other lung. In a lung surgery that was just recently done, 18 tumors larger then 3 mm were removed. We plan now to do lung CT scans once every 6 months, brain MRI and lower abdomen CT scans once a year and whole bone scan once every two years.

Best Wishes to all of you.

Hi, I have a question reg. the situation in the beginning - did she have these multiple lung metastases found at the same time as a primary was discovered or they have been found later. I am asking because there seems to be a very different approach to the treatment plan at the different institutions (and countries) - in some cases primary tumor is not getting radically resected when there are already distant mets, esp. multiple and only conservative treatment is offered. Was is a problem to get a decision to resect the primary?

Hi Olga,
The multiple lung mets were discovered at the same time when the primary was discovered. During our search for treatment options we asked the opinion of different specialists from different cancer centers. The majority of specialists recommended excising the primary out. One center recommended not doing any surgery at all. The reason had nothing to do with the multiple lung mets, but with the fact that alveolar soft part sarcoma is cancer that has no cure and the claim was that surgery if not proven to be fully curative is not necessary and reduces the patient’s quality of life. We strongly disagree with this approach of leaving the primary tumor in place. This tumor was growing and was about to cause more damage to the muscles and the bones around it. In addition this big and growing tumor continuously released tumor cells into the blood, increasing the chance of secondary tumors in other body organs as brain, liver or bones. Also, our conclusion from talking with other ASPS patients was that better outcome is usually seen for patients who went through multiple surgeries to remove the primary tumor, the lung tumors and metastatic tumors from other parts for the body.

Just a few years ago there was almost a rule that the new Dx patient with the stage 4 of the chemoresistant sarcoma would receive only a best supportive care or a palliative chemotherapy, now it is recognized that the simultaneous resection (or other local treatments) can significally prolong the patients lives with the preserving of the quality of life esp. when the sarcoma is slow growing. The new NCCN Practice Guidelines in Oncology – v.1.2007 - might be found here:

http://www.nccn.org/professionals/physi ... arcoma.pdf

and on the page 8 for the situation "Metastatic or unresectable disease" - for the single organ affected by the limited amount of the metastases (potentially resectable) they have an option "Consider metastasectomy" and for the situation "Disseminated metastases or unresectable" they still have an option of the "Palliative surgery" (but they also list there an "Observation, if asymptomatic" so if you are determined to have a resection then make it symptomatic).
Not all of the clinics are the NCCN members so technically they do not have to adhere to this guidelines, but it can be used as an argument as the NCCN Soft Tissue Sarcoma Panel Members are the most recognized sarcoma oncologist in North Am. (page 2 of this document).

Thank you Olga,
I read the relevant pages in the "National Comprehensive Cancer Network" (NCCN) Guidlines in Oncology for soft tissue sarcoma. It is very encouraging to see that some of the Treatment options that benefit ASPS patients are finally in this list: Thoracotomy and Video-assisted thoracic surgery (VATS), Tumor ablation by radiofrequency [RFA] or cryotherapy. Let me then add that from the link that you sent me I learned that The National Comprehensive Cancer Network (NCCN) is a not-for-profit alliance of 20 of the world’s leading cancer centers and it is dedicated to improving the quality and effectiveness of care provided to patients with cancer. For patients who are seeking advice regarding treatment options for ASPS, you can find more information about the NCCN and the location of those centers by visiting: http://www.nccn.org/about/default.asp

Best Wishes to all of you.

Dear All,

Long time since I updated on the condition of our daughter. She was diagnosed with ASPS in the summer of 2003. She used interferon alpha 2b for a year and had two surgeries during that time to remove her primary tumor, which was located on her shoulder and one surgery to remove metastatic ASPS from one lung. In 2007 she had another thoracotomy to remove mets from the second lung. Since then her ASPS is stable. No change was detected in annual CT scans.

Best wishes to all of you

Dear Cure,
It is allways wonderful to see that someone has done so well
I am just starting my journey down this asps road and reading the post made me feel there is hope!

Thank you again and a *huge cheer* for your daughter!

Amanda R

Great news. I am really happy for her.

It is already almost one year since I updated you on the condition of our daughter. I am very glad to report that there is no change in the size of her lung mets. This stabilization is seen now for almost three year. Our daughter was diagnosed with ASPS in the summer of 2003. She used interferon alpha 2b for a year. Then she had two surgeries. One to remove her primary tumor, which was located on her shoulder and second surgery to remove metastatic ASPS from one lung. In 2007 she had another thoracotomy to remove mets from the second lung. Since February 2007 her ASPS is stable. No changes were detected since then in annual CT scans. No one understands why ASPS tumors grow and why would they stop proliferation. I wonder if the fact that she used Interferon has anything to do with the current condition.

Happy New Year and Best wishes to all of you

IvanAndreevich wrote:Great news. I am really happy for her.

Once again

Dear CureSearch,
Thank you for writing to update about your daughter's status and to share the wonderful news of her continued disease stability. This is a great victory, and truly a source of great encouragement and Hope for all of us who share this challenging battle with this very unpredictable and difficult to understand disease. It is interesting that your daughter has had disease stabilization following Interferon treatment and subsequent resection of her primary tumor and thoracotomies for her lung mets, while other ASPS patients who underwent Interferon treatment unfortunately did not have as successful a response and outcome. I wonder if any studies have been done regarding the difference in treatment response between patients receiving Interferon treatment prior to resection of their primary tumors and lung mets, and those who underwent Interferon treatment after primary tumor and lung met resection. This disease remains extremely difficult/impossible to try to figure out, but Hopefully someday an answer will be found which will lead to a successful treatment outcome and cure for all ASPS patients. In the meantime, please know how very happy I am for your daughter and your family, and how deeply appreciative I am of your keeping this Board updated. Have a wonderful and most happy New Year blessed with continued good health and healing for your dear daughter.
Sharing the joy of your continued good news with special caring thoughts and continued Hope,
Bonni

Dear All,

It is now 7.5 years since my daughter was diagnosed having ASPS. She had just done her annual scans. I am very glad to report that we continue to see stabilization in her lung mets. This stabilization is seen now for 4 years. Our daughter was diagnosed with ASPS in the summer of 2003. She used interferon alpha 2b injections for a year. Then she had three surgeries. One to remove her primary tumor from the shoulder, second surgery to remove metastatic ASPS from one lung and third surgery in 2007 to remove mets from the second lung. These two thoracotomies removed all the growing metes as well as all the mets above 3 millimeter in size. Since February 2007 her ASPS is stable. No changes were detected since then in the annual CT scans. I wonder if the fact that she used Interferon has anything to do with the current condition. If any of you ASPS patients is using interferon alfa 2b, please write and tell us your experience and its effects on your tumors.
Best wishes to all of you

Wonderful news!
I am so happy hearing this news!
Happy holidays to your family!

Thanks for this update curesearch. Good to hear that your daughter is doing well.
When I searched for interferon and ASPS on the internet, I found only one report published in 2003 where interferon was successful. Your update reinforces the fact that interferon might have an activity in ASPS.

Have a wonderful thanksgiving.