Elisa - Dx 2010

[Johannes]

Johannes -
I am following your case, I started reading as my 9yr old daughter will be starting Cerdinib trial next week at NIH. Thanks so much for sharing your journey - it has helped me to read everything you have tried. It seems no ASPS case is the same, but a person can learn alot reading about experiences of others.
I totally understand being hesitate to remove the primary in your case, causing major life changing conditions. I think she had a lot of value in her ability to be active by having her leg in good condition, I think it is/was a wise choice at every stage you described.
I am interested in your next steps - it seems like geno type profiling and PDL theory are the major players for latest treatment options.
Sending positive energies your way - please take care -
MJ

Dear Marie Jo,

Thanks for your note, and all the best for the start of the treatment tomorrow! I think it makes sense in Prairie's case to try a systemic treatment, and I hope very much that Cediranib will shrink the mets and make at least some of them disappear. If you have any questions about Cediranib or Elisa's experience at the NIH, please feel free to send us a message!

All the best,
Johannes

[Johannes]

Dear all,

The latest news from our side is not overly encouraging. Elisa had to undergo an emergency operation two weeks ago. A mass had grown very quickly in the lower pelvic region and risked blocking the urethra. The surgery went well, and although Elisa only recovered quite slowly, she is much better now.

The surgeon confirmed today that the mass was resected with negative margins. We don’t have the final pathology report yet (“TFE3 pending”), but the pathologist excluded various other types of cancer and said that morphologically it did look like ASPS. I guess it’s better than another kind of cancer, but we are still quite shocked because this tumor grew very rapidly. It appeared in a matter of weeks and grew by about 20% over the last week before the surgery. By the way, nothing had been seen on any lung/abdomen/pelvis scans, but it seems that sometimes the images stop just above the region where the mass had grown.

On top of this, Elisa had just started stereotactic radiosurgery to one of the lung nodules, a lymph node that had clearly grown. All the other mets have, in fact, remained more or less stable over the past six months.

Regarding a systemic treatment, Elisa was taken off the MDM2-antagonist trial because she needed radiotherapy. We are still trying to get access to a PD-1 or PD-L1 drug. So far we haven’t had any luck, but we are pursuing a few options. She is now seeing another oncologist, who – unlike the “real” sarcoma specialist – is willing to do more research, contact colleagues, make special access requests, etc. So that’s encouraging.

We know it would be important to get rid of the primary in order to avoid new mets and to give a systemic treatment a better chance of working. But we are still hoping that a drug like MEDI4736 could shrink the tumor, which would make the surgery less invasive and give Elisa a good chance of maintaining some strength and function in her thigh.

I hope everyone else is doing well!

Best,
Johannes

[Johannes]

Hi,

The next update on Elisa is in the PD-1 / PD-L1 antibody section:

http://www.cureasps.org/forum/viewforum.php?f=76

Johannes

[Olga]

Is it a clinical trial or an off label/expanded access to Keytruda trough Merck Canada?

[Johannes]

Hi Olga,

It is indeed an off label use of Keytruda, i.e. not a clinical trial, which is why radiation was possible.

Johannes

[Olga]

Thank you for the clarification, I moved it to the systemic treatment, Keytruda topic ans edited the link you provided above. You are the first case we know of who got Keytruda off label/expanded access in Canada - thank you for letting us know, it is very important and creates a precedent.