Sona from Melbourne - Dx 2019 at 31 yo

[Shamsher Singh]

Hi Debbie

Thanks for the information. I’ll insist my SS to follow this schedule but at this stage I haven’t been asked to have MRI of brain even. It was initially scheduled and then cancelled. I have to enquire this too.
I’ll update the forum on Monday as well after the SS meeting.

Regards
Sona

[Shamsher Singh]

Update ; I had my first meeting with my sarcoma specialist today and he suggested me;

1. I should have MRI (brain) this week

2. Going to see radiational oncologist next week to discuss the radiation therapy ( have been recommended for 6 weeks , 5 days a week week )

3. Then after a break for 2 months and then another big surgery would be done to remove whole of my pectoral major and minor muscles.
And a reconstructive surgery by a plastic surgeon.

4. Then all of my scans ( PET, CT chest and MRI brain would be repeated).

5. My request for bone scan, CT pelvis and abdomen rejected again 😔

[Olga]

Do you have the surgical pathology report that discusses margins with the resection? Was the resection R0 or not - margins? The radiation treatment plan and the following resection/reconstruction appears to be very extensive and I am not sure why is it. You are going to have lots of issues/problems caused by this and you need to make sure the sarcoma oncologist is familiar with ASPS well and that this recommendation is based on the problems they see with the previous initial resection and not just on the general recommendation.
My son Ivan had the initial resection done by the local surgeon as well for the small tumor in his arm and then they had to redo it to make it more extensive so he lost one muscle and a part of the other. But the radiation was decided against as all the affected muscles were gone after the surgery. I am even not sure now if it was reasonable to remove all of that. Distant metastases are far more dangerous in ASPS. My son was training extensively after the surgery and the other muscles filled the space where the resected one was so visually it looks fine, he also kept all the functionality but I am not sure how it would work in case of the pectorals resected

[Shamsher Singh]

Hi Olga,
Thanks for the info. Just recapping that in my case my primary tumour was removed from my upper left chest without doing the biopsy by the plastic surgeon without knowing that it’s ASPS. Because they know it was partially resected and that it should have done with more wide margins they decided to do another big surgery. And they also want to use radiation therapy to kill any left over sarcoma cells. Whether or not it’s a right thing m not sure but that’s what I been told by my sarcoma surgeon.

What issues/ problems could I have you think after this surgery?

[Olga]

The incorrect initial resection is very common in ASPS due to its slow growth and surgeons assuming it is something benign...
But it is a very big difference, you should clarify - was it partially resected or without wide margins?
The problems would be loss of functionality if all the 4 pectorals are removed, plus possible irradiation damage for the surrounding muscles and lung tissue from the scattered radiation dose.

[D.ap]

Hi Sona
I agree with Olga 100%. And I will add that not more than 20 years ago , ASPS was treated just like all other sarcomas. First of all being total amputation, as it was assumed that was all we had as an option .
Now we know that with good margins being removed with a good knowledgable well informed surgeon , that limb sparing / non radical surgery’s can be sufficient to keep reoccurrence of the primary . And if that’s the case ,then the patient can be spared the exposure and experience that radiation can bring to the table .
But certainly talk with your oncologist .

Hope this helps .
Love ,

[Shamsher Singh]

Thanks 🙏🏻 but my SS Wants to do radiation therapy for first. Then surgery to obtain clear margins

I am depressed 😌

[Shamsher Singh]

Hi Olga

Could you please send me your email address please thanks 🙏🏻

[D.ap]

Sona
If you click “Olga’s “ name from a previous post , you can send a PM to Olga. 😊
Hope this helps
Love ,

[Olga]

Right now the long term scanning schedule is not that important, it can be discussed later. The initial staging was done ok.
The problem is if it makes sense to resect that extensively and whether or not to irradiate prior to resection.
There is a study in our Library link on the main page https://cureasps.org/bibliography/
Case studies of Alveolar Soft Part Sarcoma in MSKCC leads to the conclusion that practicing appropriate diagnostic techniques, aggressive surgical control of the primary tumor and long-term surveillance for metastases may result in long term survival (Kayton et al. 2006)
Sona, click on it - this is downloadable PDF file, read it. It is an older study and now the survival is improved due to the new immunotherapy drugs, but the study is still very useful.
They discuss In 2.6. Use of radiation therapy which was used in 25% of their patients.
the 5 receiving primary site XRT, it obviously did not help at all with the long term prognosis. And the re-operation did not help too although they also used a very aggressive surgical approach. Perhaps the less aggressive redo surgery and an immunotherapy treatment would be my choice although it is going to be a very hard to get one as you will not be eligible for the clinical trial as they need a measurable disease to survey during the treatment.

[D.ap]

Sona,

Olga has some very good points .
I’d like to add that the need for complete scans could be an aid in getting you to immune therapy, as it could see a measure able tumor for them to gage the affects .

[Olga]

Sona, I just wanted to add - your doctors behave like there are no advances in systemic ASPS treatment, but immunotherapy is showing the great results in our patients right now, and the treatment plan they offer is like they do not take it into consideration. ASPS is found to have such a great response to immunotherapy that it is widely expected now to change its long term outlook. In Canada and US some surgeons refuse to perform the extended surgeries that would inflict irreversible damages to the ASPS patients bodies because they know that now there is a good chance the immunotherapy would help if needed. Few leading cardiac surgeons refused to perform surgery for Ivan's cardiac tumor based on that, they demanded him to try the immunotherapy first.
And you did not answer if there were surgical margins negative for tumor cells or not. It is important and for me that would be an important factor when decide weather to have this extensive radiation and/or surgery

[Shamsher Singh]

**update** hey everyone
As you know that I had a major surgery back in February to remove my primary tumour from the chest, so repeated CT Chest was done and the results came back yesterday. The good news is that there is no evidence of pulmonary metastasis.🤞🏻
But I am still dealing with the same issue here in Australia with my sarcoma specialist, it’s so hard to convince him for extra scans like ct brain, repeated PET and all. I asked the clinic if I am entitled to get a second opinion, the did say yes but due to COVID-19, they said it won’t be a priority at this stage 😌

[Olga]

Hi Sona,
thank you for the update. It is indeed a very good news that there are no lung mets.
re. scans. Is your primary resection site going to be rescaned? Do you have a schedule for that? The brain mets usually come after the lung mets (although there are few known cases when they appeared without at the much later time like 10 years after). And the PET scan is not ASPS small mets sensitive. So overall, the primary resection site and chest CT is OK for now, to reduce the need to visit the hospitals.

[Shamsher Singh]

Thanks for your response. As you might remember that my primary tumour was in my left chest wall so yes they did re-scan the primary section site after the operation and it was all clear. They achieved negative margins. Plus didn’t have to remove whole of my major pectoral as previously planned, instead they removed half of my major pectoral didn’t remove pectoral minor. I have recovered completely from my major surgery. I am completely normal as I was like before, just not mentally I am the same person😌