Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello lovely people,

Some of you might have seen a couple of posts in the guest book a couple of months ago about my dear friend HM in Sweden. I thought I should move over to this bit now.

She had the primary tumour removed from her left arm 7 years ago, and after apparently being clear for all these years had another tumour removed in August. This one was small (2cm) near the lymph nodes on her right side. After a bit of difficulty she has finally been referred to the Karolinska Institute Sarcoma Centre in Stockholm where she had more scans last week. Very sadly they found two mets - one or her lung, one on her pelvis.

This is what she said:

"The PET-CT scan showed a 6 mm large met on the right lung as well as a 1cm large one in the pelvic bone. They haven't finished discussing treatment options yet: he initially mentioned radiation or chemo, but after me reminding him it didn't respond to well on either, he seemed to think it worth while checking with thorax if they thought it the lung met was removable. He also mentioned using precision radiation therapy for the met in the bone."

I've been reading and reading through all the amazing posts you've put up about treatment for mets. It looks to me as if laser resection or cryoablation are the best options for the lung met, and cryoablation is best for the one on her pelvis. Does that sound right?

Is a PET-CT scan enough or should she have an MRI of the brain, and a fully body bone scan as well?

Sending thanks and best thoughts to you all,

miller
x

Hi,
thanks for an update, sorry to hear about more troubles for your friend, but it is typical for ASPS.
Lets talk about the mets.
The lung met - they probably want to have the tissue to confirm that the lung met is an ASPS met, so they might want to remove it by VATS surgery - not the full size open surgery but the one when the tools are inserted trough the small incision and a tiny camera trough another one. It is a low traumatic surgery and they can see around if there are more mets - the small ones that might be not seen on the CT scan. Cryo is easier but the experience of the performing doctor is the key - the same with the VATS but it is much more common. So the question is if their surgeon is more comfortable with VATS? The laser assisted surgery is very advantageous with the multiple small mets, not so with the single met. Try to find out if there is a good interventional radiologist in the area that is skilled with cryo to get his opinion on the local treatment.

The pelvic met. I suspect that by the precision radiation therapy for the met in the bone they mean the radiosurgery - very high dose of the radiation given with the narrow ray using some dedicated unit like Novalis or CyberKnife etc.. It is probably a good choice for the size and the location - if the bone is thin in that area it may break from the cryo, and I would hurry up with that one, if this met grows, this option might be off the table or the probability of the treatment to be complete and definite gets lower.

And yes, should she have an MRI of the brain given her history of the lung mets (brain mets are noted to always be preceded by the lung mets), and a fully body bone scan as well (as she already have a bone met, and also the fact that smaller ASPS mets are not highly metabolic and do not attract enough glucose tracer to be lit on the PET/CT scan - you can remind that to her docs).

Dear Miller,
I am so very sorry about your dear friend's disease progression, but am grateful that she has your special friendship and strengthening support, and that you wrote to the Board to update her situation and to reach out for information, input, and advice.
I agree with Olga's advice to you regarding the various treatments for the new mets, and the need to also have a brain MRI and a full body bone scan. PET-CT scans can be erroneous, and the only definitive scan for accurately diagnosing/ruling out brain mets is a brain MRI.
Regarding the pelvic bone met, depending on the location, Radiofrequency ablation (RFA) or Cryo might be good treatment options, but as Olga said, there is urgency in pursuing immediate treatment for this one since it is already one centimeter, and the smaller the tumor, the better the chance for a successful response. Also, with RFA and Cryo, there are size limits for the tumor due to the need to have good margins from adjacent organs since the burn of the RFA or the freeze of the Cryo can't be precisely controlled.
Please give your friend a gentle hug from me and tell her that my special thoughts and very best wishes are with her. Take care and keep the Board updated as you are able.
With special caring thoughts, healing wishes for your friend, and continued Hope,
Bonni Hess, mother of 30 year old Brittany diagnosed at age 19 in July 2001

Dear Olga and Bonnie and everyone else,

Once again I'm overwhelmed by your kindness and the depth of your knowledge about this awful disease: thank you. It is so difficult to stay strong in the face of it but your support makes an enormous difference: it felt like a big weight off my shoulders to get your replies. I wish none of us had to know anything about it, but life's not like that, so thank you once again. I guess the most positive thing we can do is share what we learn to help anyone else who's faced with it.

I'll do my very best to help H get an MRI brain scan and a full body bone scan: it seems hard to imagine in the circumstances that they would refuse but you never know. It sounds like they're not as experienced/knowledgeable about ASPS at this sarcoma centre as I had hoped.

For the mets they've already found, it sounds as if they should treat the pelvic bone one first? I'd hoped that 1cm was still quite small but I can see that the smaller the better for treatment. I suppose the size limits depend on exactly where it is, and how close to other organs. I'm actually quite amazed that a tumour can grow inside a bone, and that it can be treated at all.

I didn't know anything about VATS surgery so it's really helpful to hear about that and the comparison with cryo and laser-assisted surgery. So we will try and find out what experience the surgeon and radiologist have - that's such helpful advice.

There's one other heartbreaking thing I'd really welcome any advice on. H has two gorgeous children, aged 5.5 and 1.5. The older one knows H has been poorly (from the anxiety rather than the ASPS itself) but they haven't talked about her diagnosis. H sees that it would be better to do so because that would get it out in the open and allow the 5.5-year-old (who is very mature for her age) to express her fears and anxieties; but how on earth does anyone have that conversation? What do you say? How do you start it? When is ever going to be the right time?

Sending best loving thoughts to all of you, and all those you support -

miller
x





I wish I could give H a hug, more than anything, but we're not even in the same country which is very hard. But I shall tell her that your'e all thinking of her and wishing for the best.

Dear Miller,
I'm so sorry about your friend's latest diagnosis and I do hope that she can be treated effectively and overcome this latest hurdle.... like so many others whom we read about here in this forum.
As for the children and how or how much to tell especially the older child.... Well, that's a tough question to answer because it depends on the child and on how the child is being raised. One of my daughter's closest friends since childhood came from abroad to spend Patti's final 6 weeks with her and brought her 5-year old daughter with her. They were at her bedside daily both in the hospital and finally at home under hospice care. The little girl watched her Auntie Patti become sicker as the days wore on, but because her mother explained what was going on very well, calmly and adding reassurance and faith, the child was not affected in a negative way. To the contrary, she was able to show love and compassion to her Auntie Patti, and even now - back home in her own environment - she will say that she misses Auntie Patti but she is happy she is no longer sick. Again, this was an experience that this particular little 5-year old girl was able to handle well, but it may not have been OK for another child. But a parent should know their child and know what the child is able to cope with. Most children do very well with the truth, actually, in my opinion. If mommy isn't feeling very well, it's impossible to hide that fact for very long from the child.
All the best to your friend. I, too, wish you could hug your friend. She's probably in need of some good hugs and support. Not feeling well and dealing with two small children is certainly very difficult. May she find peace of mind and, of course, the best and most effective treatment available.... and lots of help with the children until she's back on her feet again.
Trixi