Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi,

My daughter(15 Years) was diagnosed with 5cm Alveolar soft part sarcoma on the upper part of left thigh and it was removed by surgery on 07-20-2007.

Before the surgery as per the MRI they were under the impression of, it would be Haemangioma. They did the needle biopsy at the time of surgery and in the frozen section they found that this is Alveolar soft part sarcoma.

She had the second surgery on 09-27-2007 at the same spot, as in the post surgery MRI they realized that there are some positive margins in her thigh. After that there is no evidence of disease in her body and we totally forgot about those dark days.

She went for CT scan on Apr’02 2008. Now we came to know she had 3 metastasis tumors in one lung and 2 metastasis tumors on the other lung. Their sizes are in the range of 1mm to 4mm.

I am in contact with Yosef from starting. He suggested me to post the whole case history in our site then you will get right suggestions for further steps.

Please suggest me what is the best procedure to remove the tumors in this stage. I am in Illinois, USA. I am ready to go to any where. Please suggest me.

Thanks..Rani.

Hi Rani,

Sharing our experiences are so important. I think the best thing is to be your own advocate. It seems prompt surgery is very effective (from my son's history). There are medical publications and expert doctors identified throughout this invaluable website to help guide you to make the best decision for your situation. Also, if surgical removal is done, then the tumor can be potentially used for further research or a specific vaccine. Deciding on the best course of treatment is hard work, but so important. Best wishes.

Elaine Anderson

Thnaks for your suggestion Elaine.

Dear Rani,
I am so deeply sorry for your daughter's heartbreaking ASPS diagnosis and the recent scan results which showed metastasis to her lungs. I am very grateful that you found your way to our Web site and that you have reached out for information and support from the ASPS Community. As Elaine said, it is very important to be your own advocate. You need to be very proactive and to be as knowledgeable and well informed as possible through active research and networking with other ASPS patients before making any decisions about the best course of treatment. Because your daughter's lung mets are thankfully relatively few and small there are several possible options which you should discuss with the oncologist including a thoracotomy, a less invasive thoracoscopy or laser assisted surgery, or a minimally invasive Radiofrequency ablation or Cryoablation procedure. Information on these procedures is presented and discussed on this Web site. I would be glad to share information with you and try to answer any questions that you might have about thoracoscopy, RFA, and Cryo, all of which we have experience with since our daughter Brittany has had all three of those procedures to remove or destroy her lung mets. Other ASPS Community members on this Forum can provide you with information about the thoracotomy and laser assisted surgery based on their experiences. Location of the mets will be an important factor in the treatment decision since if the tumor is too deep in the lung, too much lung tissue might be lost with a thoracoscopy or thoracotomy, and RFA and Cryo cannot be done if the tumor is too close to a vessel or organ. The most important thing is to be very knowledgeable about all of the options and to thoroughly discuss each option with the oncologist before making the final treatment decision. Know that you are not alone in this challenging battle and that I and others on this Web site are here to help with information sharing and strenghening support and encouragement. Please take care dear Rani and keep this Forum updated as your time and situation allow.
With special caring thoughts and continued Hope,
Bonni Hess

Feel free to contact us off list, as well. Our email is drseide "at" neurolearning.com

There are a variety of options re: what to do with the lung nodules. Dana Farber peds onc didn't want to take them out (they want to watch them and only operate if they grew into a dangerous area). UCLA likes a trial of a neoadjuvant prior to removing them because it tells you about possible efficacy of the medication. ASPS can spread after many years, so it can be an advantage to know if a medication may have benefit if they return, or to treat possible microscopic disease. In addition, there may be some therapeutic benefit to taking an effective neoadjuvant before a sarcoma is removed. Existing chemotherapeutic drugs are not known to be helpful, though.

Knowing what I know now, I would insist on a CT sarcoma surgeon to remove the lung nodules. Because your daughter is a child (as ours is), it is also important how you go about looking for a surgeon. As others have said, you also want a surgeon who can do VATS. But whether your daughter is a candidate for VATS depends on the location and size of the nodules. If you look through a pediatrician or peds onc, then they will refer to a ped surgeon. At Seattle Childrens, a general surgeon (with no particular expertise in sarcoma) removes all the lung nodules / mets.

We chose to have our daughter see an adult CT (cardiothoracic) sarcoma surgeon at UCLA. We were thankful afterwards with this choice because the surgeon was able to spare more lung by doing a segmentectomy - something that the general surgeon did not do. One of 'K''s nodules rapidly enlarged and was near her heart - and this was another reason to feel better with our choice of this CT doc over the general one.

As far as I know, the largest sarcoma surgery centers in the U.S. are Sloan Kettering, M.D. Anderson, and UCLA. We thought the most experienced surgeons would be the ones removing sarcomas all the time from the lung.

Dear Rani,

I am very sorry to hear about your daughter suffering from asps.
My boyfriend Sina (he has already posted on the personal info board about his story) has also experienced a similar situation.
Having had a ~ 5 cm primary tumor removed, lung mets were discovered about two years after the surgery - I must apologize I
forgot the exact amount but about 2 or 3 in each lung, between 2-4 mm.
After having received this shocking news we immediately decided for as complete removal as possible.
We contacted the leading lung surgeon (no ped surgeon because we felt that for the surgery it had to be a specialist for this area of the
body rather than for this type of tumor) at the clinic in Innsbruck, Austria to discuss further actions.
Luckily the lung mets were in areas of the lungs, where they could be removed with negative margins so we had
two options:
- minimal invasive surgery with removal of only the mets which we could see on the CT
- open surgery on lungs
I have to be honest, despite the fear of immense pain after the surgery I am happy we have decided for the open surgery on lungs.
This is on the one hand the more dangerous and invasive option, but on the other hand, Prof. Schmid (that's the doctors name)
found not only the few mets screened on the CT but about double the amount in smaller versions, which we did not see on the CT.
Totally, 8 mets were resected - more than expected.

So the advice I can give you: if your daughter has not gone through lung surgery yet, you may prevent remaining mets in her lung by
deciding for an open lung surgery to make sure everything is removed. I can only say this out of own experience!
I hope very much that I could help you and good luck!
Greetings and all the best,
Laura