Sam from Florida - Dx 2007

[jcs2007]

Hello Olga, thanks for the info regarding brain mets and the need to up our MRI more often. We got a little complacent once Sam went away to college,which will not happen again. The brain met was removed encapsulated with clean margin but was 3x2cm in the cerebellum. The pancreas showed suspicious lesion in the tail but was inconclusive on MRI so we did a endoscopic biopsy which confirmed ASPS. Now we head to Miami this week to discuss treatment options, but I'm sure he will be taken off Arq due to progression even though lung mets have been pretty stable.
Hello Bonnie, the primary tumor was removed 10-2007 from left thigh and he started Arq197 3-2009. He had 1 lung met treat with Radiation in 2013.
I will update the board with our treatment option after we travel to Miami this week.
Thanks again for providing valuable information and caring thoughts!
Cindy

[Olga]

Cindy, hi, thank you for the clarification. I am not sure if you have seen our pancreatic met treatment story - if you have not it is here:
http://www.cureasps.org/forum/viewtopic.php?f=81&t=1260
we reviewed all the options available and decided to have it removed in Germany by Dr.Walz as it was minimally invasive trough the back with the greatest experience possible. There are few locations that could possible do it in US although this approach is rare (specifically retroperitoneoscopic - means VATS from the back) is mostly used for the adrenal metastases removal. It is a huge advantage versus other types of surgery from the front of abdomen as they do not need to unload all the organs first in order to get to that met - it is close to the spine. As the matter of fact our surgeon refused to perform a surgery here as he considered it to be to traumatic and not appropriate for the metastatic patient with lots of pain, pancreatic damage, potential loss of the panc function and a long recovery - bu after the surgery in Germany Ivan was up and completely normally functioning 2 weeks after this novel surgery.

[Bonni Hess]

Hello again dear Cindy and thank you for the additional information. I am grateful that Sam had his primary tumor removed because all of my ASPS anecdotal observations during the past 15+ years indicate that systemic treatments seem to be much less effective and ultimately unsuccessful if the primary tumor is not removed, a concern which I have repeatedly expressed to those patients/patient's families on this Board who have not had their primaries removed for various reasons. Unfortunately and inexplicably, doctors too often don't recommend/support the removal of the primary tumor taking more of a palliative approach towards fighting this very challenging disease.
Thank you for emphasizing the critical importance of not becoming complacent with having regular scans, even if this disease appears to be relatively stable as it did for Sam during his seven years of ARQ-197 treatment. This issue has been an ongoing battle for us during Brittany's thus far seven and a half years of sustained disease stability on Cediranib and her doctors/insurance companies/the Clinical Trial pharmaceutical company wanting us to extend the amount of time between scans, and our resisting the more infrequent scans knowing the unpredictability of this insidious disease coupled with the knowledge that Brittany could develop resistance to the Cediranib at any time followed by disease progression as heartbreakingly appears to have happened with dear Sam and his previously very successful ARQ-197 treatment. Unfortunately, there doesn't seem to currently be any permanent cure for ASPS, so pro-active and vigilant monitoring of the disease through regular and appropriate scans is essential.
I strongly encourage you to consider, explore, and discuss with the doctors in Miami Olga's recommendation of retroperitoneoscopic surgery for treatment of Sam's newly diagnosed pancreatic met as a possible treatment option. Resection of a pancreatic met can be an extremely difficult, physically traumatic, and dangerous procedure as we discovered when we were desperately seeking treatment options for Brittany's unresectable/untreatable met in the head of her pancreas in 2009. Through Olga's very extensive, dedicated, and resourceful international research she was able to find the minimally invasive retroperitoneoscopic procedure performed by Dr. Walz in Germany which thankfully appears to have been very successful for Ivan.
My most caring thoughts and very best wishes are with dear Sam, you, and your family for a positive, productive, and encouraging outcome to your meeting with the doctors in Miami this week, and I will be anxiously awaiting your update of the results of the meeting and the doctors' treatment recommendations when your time and the situation allow.
With deepest caring, healing wishes, much love and continued Hope,
Bonni

[jcs2007]

Hello, just wanted to post that Sam needs to heal from his brain surgery prior to starting any new treatment. We will have chest, abdomen, pelvic ct scan with contrast and a bone scan to evaluate for any changes especially in the lung mets and small lesion on the tail of the pancreas at the end of December. If needed, Sam may participate in the axi/pem trial in Miami. I appreciate all the information given on the board regarding scans, surgery, and systemic treatments since it's difficult to know what direction is best. Best wishes and happy thanksgiving to alll!! Cindy

[D.ap]

Cindy
Hope this day finds Sams speedy recovery!
He was able to have a tumor removed surgically on his brain ?

I've just posted an article on the primary medical topic formum pertaining to the near future use of prem/keytruba on ASPS folks
Joshua's wife shared it with me

http://www.cureasps.org/forum/viewtopic.php?f=2&t=1292
Love
Debbie

[jcs2007]

Debbie, thanks for the info on the medicine since I want to know more about it just in case that is the recommended treatment plan after Jan. Best wishes to you all!

[Bonni Hess]

Dear Cindy,
I know that this must be a very unsettled time for dear Sam, you, and your family as you wait for Sam to heal from his craniotomy before moving forward with any treatment plans. I Hope that Sam's recovery is going well and that he is continuing to regain strength, energy, and function. My very best wishes and most positive thoughts will be with all of you for very good results from Sam's scans at the end of this month, and I will be anxiously awaiting your next update. I know that you have probably been following the posts from our ASPS Community patients with the very good and encouraging news of their very encouraging successful responses to the new Immunotherapy Checkpoint Inhibitors such as those that are being tested in Dr. Wilky's promising Axitinib/Pembrolizumab (Keytruda) Clinical Trial in Miami. Hopefully this will provide a possible treatment option for dear Sam if you decide to move forward with a systemic treatment to try to stabilize the progression of his disease and shrink/destroy his pancreatic and lung mets. In the meantime, I Hope that you are all able to enjoy a beautiful Holiday season together that is filled with Holiday joy and the Hope of healing for dear Sam. Take care dear Cindy and know how deeply I care.
With warm hugs, special caring thoughts, healing wishes, love, and continued Hope,
Bonni

[jcs2007]

Hey, thanks for your encouraging message. We are headed to Miami with ct and bone scan images to decide what is the next step so I'll post once we have decided on our next move. Sam has recovered quite amazingly from brain surgery so we had nice Christmas and new year's celebration. I want to encourage vigilant scans and also family and friends emotional support since ASPS is a rollercoaster for the patient and family members. Wishing you a great 2017 and although I don't always make comments on the forum, I have stayed up on the different personal stories and treatments. Prayers!

[D.ap]

Cindy ,Sam and family

Gods speed to you all !
Let us know when you get a moment

Love
Debbie

[jcs2007]

Just curious if anyone is aware of a lung/ adrenal met bleeding inside the lung? Sam started coughing up blood with pain in right lung which led to a hospital visit where they did a bronchial procedure which showed no obstructions, but a lesion bleeding so now we are planning to do trial anlotinib to hopefully shrink lung met and pancreas met. Then, we look at surgery for pancreas met. Thanks to everyone who is posting their journey with asps!

[D.ap]

HI Cindy
I'm sure sorry to hear of Sams trip to hospital .
Is the offending lung tumor on right side /lung ?
How big is it?

How big is the pancreas tumor?
Cindy so there is an adrenal tumor as well?

Bleeding sure isn't unusual with repeated coughing
If it were myself ,I'd be a little concerned of especially the pancreas tumor as well as the adrenal tumor ?

[Olga]

Cindy - it is a puzzling plan to get into a clinical trial to stop a bleeding. It is an acute situation that needs to be addressed ASAP and the cryoablation could take care of it immediately, or some angioprocedure to block the bleeding blood vessels. ASPS metastases can bleed, yes, and we had the cases of bleeding. Needs to be stoped before doing anything else as can cause the collapsed lung. Is there any blood collecting inside of the lung (at is base)? Was the thoracocentesis done to evacuate the blood, sometimes they leave it in hope it gets absorbed if the amount is small, like less than 50 mil.

[arojussi]

Icompletely agree with Olga. Anlotinib is tki medication as far as I can remember, so it can make bleeding worse. So dealing with bleeding met first would be savest route.

[D.ap]

I concur all

[D.ap]

http://ascopubs.org/doi/abs/10.1200/JCO ... ppl.e17071