Pam experience on Axitinib and Pembrolizumab (Keytruda) trial in Miami

[khp]

Hello Olga,
My name is Karl, my wife Pam was diagnosed with ASPS in November 2011 and is currently in the Clinical Trial NCT 02636725 in Miami. We are from Louisiana and Pam is currently 65 years old.

Without going through Pam's timeline of events prior to going to Miami, we wanted to share what is happening with her due to this Trial. The Results speak for themselves.

Pam started in the trial June 16th, 2016.

First Scan Results.

June 13 Scan

Metastatic lesion involving the mid sternum with soft tissue component measuring approximately 2.9 x 3.0 x 3.0 cm with associated subacute to chronic pathologic fracture.
Metastatic focus in the right posterior C7 vertebral body, right rudimentary rib at C7 and right posterior elements at T1 with soft tissue extension into the right neural foramen and anterior and right lateral central canal causing mass effect on the
thecal sac.
Metastatic sclerotic lesion of the proximal posterior left seventh rib and left posterior elements of T7 with associated soft tissue component which extends into the left paraspinal region and into the left epidural central canal causing mass effect on
the thecal sac. Sclerotic lesion also involving the left posterior elements of T8.
Subacute pathologic fracture of the proximal left posterior 10th rib with associated lytic lesion.
Lytic lesion involving the right posterior elements of T11.
Metastatic lesion involving the left anterior fifth rib with associated soft tissue component and pathologic fracture.
No significant axillary, mediastinal, or hilar lymphadenopathy
7 mm nodule in the left lung apex. 6.5 mm nodule in the anterior left upper lobe, image 16.
5 mm nodule in the posterior left lower lobe, image 31 and 4 mm nodule in the medial left lower lobe also visualized on image 31.
4.5 mm nodule in the right upper lobe, image 16. Numerous nodules in the right middle lobe, the largest is pleural-based and measures 6 mm, image 31. Somewhat spiculated pleural-based 7 mm nodule in the right middle lobe, image 119/270 There are also
numerous nodules in the right lower lobe, the largest is in the medial costophrenic recess and measures 8 mm
Numerous subcentimeter nodules are visualized bilaterally, most consistent with metastatic disease.
Subsegmental atelectasis in the left lower lobe and right upper lobe.
Liver measures 22 cm in length.
Numerous enhancing lesions throughout the liver consistent with metastatic disease, there is a large conglomerate of masses in segments 3, 4, 8 and segment 6.
Largest conglomerate of masses in segments 4 and 8 and measures approximately 11.8 x 9.0 x 8.7 cm There is a another conglomerate of masses in the inferior aspects of segments 5 and 6.
There are also multiple fluid density lesions in the liver with no evidence of enhancement and may reflect cysts.
Main portal vein measures 1.4 cm in diameter. 
Peritoneal metastatic implants are visualized in the right lower quadrant, the largest nodules measure 1.3 x 1.3 cm on image 84 and 1.7 x 1.5 cm on image 87. There are also small peritoneal implants posterior to the inferior right abdominal rectus
muscle. Shotty lymph nodes and fat haziness in the inferior anterior pelvis.
Schmorl's node in the superior endplate of L1 with acute appearing compression deformity of the superior endplate.
Large sclerotic lesion involving the L4 vertebral body. Mixed lytic and sclerotic lesion involving the bilateral sacroiliac, greater on the left.
Small central sclerotic focus at S4.
Mixed sclerotic and lytic lesions visualized in the bilateral iliac bones and proximal right femur
.
September 16 Scan

Numerous previously noted pulmonary nodules have either resolved or significantly decreased in size. 3 mm nodule in the posterior lateral right upper lobe, image 15 compared with 7 mm on prior study.
Peripheral interstitial thickening in the anterior right middle lobe is again noted is cystic with chronic changes. Previously noted 7 mm nodule in the left lung apex, pleural-based nodule in the lateral right middle lobe spiculated nodule in the right
middle lobe have resolved.
Previously noted 8 mm nodule in the right medial costophrenic recess now measures 2 mm.
Stable biapical pleural thickening/scarring.
Mixed sclerotic/lytic lesion of the mid sternum is again visualized, however now demonstrates pathologic fracture, age indeterminate. Previously noted soft tissue component is no longer visualized.
Stable sclerotic lesions involving the posterior elements of T1 and posterior vertebral body and rudimentary rib on the right of C7.
Previously noted soft tissue density extending into the right neural foramen and extra-axial space in the central canal at C7-T1 is no longer visualized. Irregularity of the cortex of the proximal left second rib suspicious for pathologic fracture of
indeterminate age.
Blastic lesion in the proximal left posterior seventh rib appears mildly improved with resolution of soft tissue component which extended into the paraspinal region and left epidural central canal. Adjacent atelectasis in the left lower lobe is again
visualized.
Sclerotic lesions involving the left posterior elements of T7 and T8 are grossly stable. Evidence of interval healing and pathologic fracture of the proximal left 10th rib. Grossly stable sclerotic lesion in the left anterior fourth rib and interval
healing of pathologic fracture and the left anterior fifth rib with near complete resolution of soft tissue component.

Abdomen and pelvis:
Interval decrease in size and enhancement of metastatic lesions throughout the liver. Target lesion in the dome of the liver on image 12 measures 2.7 x 2.1 cm compared with 3.9 x 3.2 cm on prior study. Second target lesion in segment 8 of the liver
measures 2.2 x 1.8 cm, compared with 3.2 x 3.0 cm on prior study. Central lesion in segment 4/8 measures 2.7 x 2.1 cm compared with 3.7 x 3.0 cm on prior study.
Liver is decreased in size measuring 15 cm in length, compared with 22 cm in length on prior study. Stable fluid density lesions in the liver which likely reflects cysts.
Stable distention of the gallbladder with mild pericholecystic fluid.
Stable mild intrahepatic and extra hepatic biliary duct dilatation.
Portal veins, splenic vein, and SMV are patent.
Spleen, pancreas, and adrenal glands appear unremarkable.
Multiple surgical clips in the retroperitoneum are again visualized.
Small left renal cyst is again visualized.
Kidneys enhance and excrete contrast symmetrically.
Bladder is well distended and appears unremarkable.
Wall thickening of the distal gastric body is again visualized. Gastric antrum appears unremarkable.
No evidence of bowel obstruction.
Significant stool noted throughout the colon. Rectum is decompressed.
Peritoneal metastatic implants in the right lower quadrant have either resolved or significantly decreased in size. Previously noted peritoneal implant in the right lower quadrant on image 87 is not visualized on today's study.
Slightly more superior on image 83, peritoneal implant measures 1.0 x 0.6 cm, compared with 1.3 x 1.3 cm.
Previously noted tiny implants posterior to the inferior right abdominal rectus muscle are no longer visualized. Previously noted fat haziness and tiny lymph nodes in the anterior pelvis are not visualized on today's study.
Abdominal aorta is normal in caliber.
No significant retroperitoneal or external iliac chain lymphadenopathy.
Stable nonspecific bilateral inguinal lymph nodes.
No evidence of free intraperitoneal air or fluid.
Stable appearance of compression deformity of the L1 superior endplate sclerosis and large Schmorl's node.
Large sclerotic lesion involving the L4 vertebral body, mixed lytic and sclerotic lesion involving the bilateral sacral, greater on the left, sclerotic focus at the S4 vertebral body are grossly stable.

December Scan

There is no pneumothorax. Pulmonary nodules:There is again
almost complete resolution of the previously described pulmonary nodules, particularly compared to the CT scan of the chest from June 2016. The pulmonary nodule in the apical segment of the left upper lobe now measures approximately 1.9 mm (previously, 7
mm in June and 2.4 mm in September). The reminder of the pulmonary nodules are relatively stable and measures approximately 2 mm. There are no new pulmonary nodules.
Liver: There are again multiple ill-defined hypodense lesions, some of which with mild peripheral enhancement, throughout the right and left hepatic lobe, when compared between the portal venous phases. There is questionable interval decrease in size of
some of the hypodense lesions as follows: Target the lesion in the dome of the liver now measures 2.4 x 1.5 cm (previously, 2.7 x 2.1 cm). Target lesion in the segment 8 of the liver now measures 1.7 x 1.1 cm (previously, 2.1 x 1.7 cm). Central target
lesion in the segment 4A/8 now measures 1.3 x 1.1 cm (previously, 2.7 x 2.0 cm). However, there are several arterially enhancing lesions throughout the hepatic parenchyma, only seen on the arterial phase, not appreciated on prior study, suspicious for
metastatic deposits (previous examinations did not have similar arterial phase)
There is again widespread predominantly osteoblastic metastasis throughout the axial and appendicular skeleton, relatively unchanged since prior examination.

When I first took Pam to Miami she had to use a walker to get around, she is now walking on her own and is much stronger. We are very pleased with the results we are having. We know things can change at any time but for now we are glad to finally have some good news. As you all know there is much more to our story but we wanted to give hope to others that needed to know as you said in your first post, Olga Dr. Wilky is a very determined and compassionate person who really cares for each of her patients. She is still recruiting patients and we know that the other ASPS patients are having some good results. We will update our next scan in March and pray for further reduction in the remaining tumors.

[D.ap]

Hi Karl
I'm sure Olga will respond when she's able
Pams and your story is truly inspiring
With Dr Wilky behind the wheel you are in good hands for sure
When Pam was diagnosed , was it with a chest primary or a spine primary? Had she had prior experience of sarcoma removal to date ?

Our son has been on Opdivo since July but had a small dosesge of sutitinib of 6 weeks ,9 months prior
Joshua's personal

http://www.cureasps.org/forum/viewtopic.php?f=4&t=750

We are too experiencing a wonderful recovery with Opdivo of weight gain and expoidential energy to date
We share our stories too as we are at a disemmedated challenge as you and Pam are / were

http://www.cureasps.org/forum/viewtopic.php?f=82&t=1297

The thankfulliness is shared

Each day for everyone , you snd I as well as all, are a true gift

Love
Debbie

[khp]

Hi Debbie, So sorry to hear about Joshua but I'm glad he is having some success with Opdivo.
Pam had no other experiences with sarcoma. Pam was first diagnosed with breast cancer, Invasive Mammary Carcinoma, which was removed with good margins in September 2011. One of her scans showed she had cysts on her liver and our radiation oncologist wanted a closer look before he started treatment on her breast. The scan showed that there were cyst but it also showed something behind her liver. After several biopsies that let them know it was cancer but not what kind Pam had surgery to remove the tumor. The tumor was attached to her spine and aorta and of course they were not able to get margin on her spine but managed to on her aorta which was negative. Pam then received radiation to the area where the mass was and had CT scans every 6 months until December 2013 when her liver was shown with a tumor. We had a Pet/CT scan done at that time which showed not only was there a tumor in her liver but also her C2,C7,T1 vertebrae and other bones. After having radiation to those areas we were sent to MD Anderson Sarcoma unit in Houston. Pam went through all the necessary tests to get into a trial with Sutent or Cediranib but at the last minute AstraZeneca decided due to Pam having breast cancer two years earlier they didn't want to take a chance that it would reoccur and tilt the trial results. At that time our Oncologist put Pam on Sutent in April 2014 along with Xgeva injections monthly. This lasted until September 8th 2014 when she was switched to Votrient due to the bad side effects of Sutent. Pam stayed on Votrient until July 2015 when her tumors began increasing. We were during this time looking for a trial to hopefully come along as tumors had started showing up in other parts of her spine, ribs, liver and her lungs. I had been watching the web sight clinicaltrials.gov for some time when I saw Dr. Wilky's trial had started recruiting and that is how we ended up in Miami.

Debbie, thanks for the links you sent to Josh's journey. Our heart goes out to all of you as this is certainly not something none of us ever expect to happen to us. We too are learning how little the medical field knows about sarcoma and that most oncologist are clueless at what they are dealing with. We are going to a cancer center here in Louisiana and we feel they are good doctors but don't have a clue about treating sarcoma. We now know Pam should have not only been getting CT scans but also Pet/Ct scans as the tumors in her neck were well advanced when her liver tumor was found. I just wish more of us were informed by doctors that we need to seek out a sarcoma center for treatment.

Merry Christmas!
Karl

[D.ap]

Karl and Pam

Wow. You were given so much to deal with back in 2011. I'm so sorry that happened to you. You are so kind to show such care and inspirational news in light of what has happened. Thank you both.

The bone involvement is sure a tricky aspect to treat with ASPS and it sounds like the Pem and axitinib are a good match for the majority of the tumors.

Are you able to take any medicine to aid in bone preservation?
And how often are you able to do brain MRI's?

Merry Christmas and happy new year to you both

Love
Debbie

[Olga]

Hi Karl,
thank you so much for the very comprehensive description of the scans and an overall medical history that preceded to this trial enrollment, I read it with great interest. The response so far is fairly impressive and of anything it reinforces the meaning of the clinical trial - they are intended for the people with no other proven options so when there is a response - the patient benefits regardless of what future holds, regardless of the durability of the response. We all hope that it will be a good and long lasting one, but for now we are very grateful that your wife quality of life improved greatly.
It would be helpful if you opened a new topic for your wife in the Personal Stories and Updates, you can just copy most of your the last post with the details of her ASPS history, leaving the detailed description of her clinical trial story here. We can ask then there for any additional info if needed. I have to say that her ASPS story is highly unusual.

[arojussi]

Thanks for good news. In other cancers (melanoma, kidney cancer and non small cell lung cancer) PD-1 inhibitors can achieve long term surival benefit. Looks like 3 year survival rate is even higher than response rate. This is something that I still find difficult to believe, but many sources say same thing. Of course we dont know how keytruda works in asps, but I am hopefull. Happy holidays for you.

[khp]

Pam was taken off the Keytruda trial on 3/06/17 due to an increase in her liver mets. She was then started on Nivolumab and Ipilimumab on 4/12/17 but this combination caused her to have colitis and was taken off of that treatment. She began the Anlotinib trial on August 16, 2017.

[D.ap]

Pam on Anlotinib

https://cureasps.org/forum/viewtopic.php?f=91&t=1699