Lisa From China - Dx 2014

[Lisa]

Hi everyone,

My name is Lisa from China and I am 23 years old now. I am so glad to find this forum where so many friends exchange their experiences and opinions about this rare disease. I knew this forum from other Chinese patients and I really obtained lots of useful knowledge and information here. Thank you guys!! It is you guys let me know that this disease is not that bad as I thought and it is totally treatable! After viewing this forum for several months, I decided to be here to share my experience fighting against ASPS and seek some suggestions:).

I was 22 years old when I found a lump at my left thigh in Oct 2013 and then the resection was performed in Jan 2014. The primary tumor was about 3cm*2cm and the whole muscle (about 8cm*25cm) was removed to ensure the negative margin . Hopefully there will be no recurrence in the future. After the resection, chest CT, abdomen CT and Pet-CT were done in Feb 2014, no metastasis was found (however, it was proved that I had lung mets already at that time).

In July 2014, several mets were found in chest CT done in Sydney (I studied in Sydney), the largest one was reported to located in the left lower lobe, measuring 15mm. The second largest one was about 7mm. Then I went back to China and asked to do CT scan again due to the loss of certain important image of the scan did in Sydney. This time the largest one was reported to be 12mm and there were more than 10 mets can be found in the images. I guess the difference of 3mm may resulted from difference machine used and I usually use 15mm to do the comparison . When the doctor reviewed the first chest CT scan done in Feb 2014, he found there were some mets there already, the biggest one was about 10mm. Therefore, it seems to be grown 5mm in 6 months.
Then I did brain MRI, primary MRI and bone scan, all the results were good, they were all clear.

I began to take Pazopanib from September 2014.I took 600mg per day, luckily, the side effects on me are totally tolerated. For the first month, the major side effects were diarrhea, high blood pressure (higer than 140/90), and hand foot syndrome. Besides that, I experienced really serious pain in waist during the first month, I even couldn't move for some days, but all of the side effects were tended to be relieved from the second month. And my hair started to turn to white after one month I took Votrient. Till now, I have been taken Pazopanib for about 5 months and there is no major side effect except my white hair. Overall, my life is not affected by this treatment at all, which is really lucky for me since i know some people experience really serious side effects.

I had a CT scan in Nov 2014, which was 70 days since I took Pazopanib, and the result was quite optimistic. Most mets were disappeared, and the largest one that was 15mm shrunk significantly to 3mm. I am so inspired by the result and hope for the good news from the next CT scan scheduled at the end of Feb.

I will have next CT scan at the end of this month, could I ask some advice from you guys since you are equipped with really professional knowledge and experience about ASPS.
The best result may be all the mets are disappeared, is that means I can stop taking Pazopanib? My understanding is that, if there is no met, it is unnecessary to continue the TKI treatment. Am I right?

It is really happy to be here, communicating with so many kindly friends, sharing our efforts to struggle with this disease. Best wishes to all of you guys~~~

Sincerely,
Lisa

[Olga]

Hi Lisa, welcome to the forum. We are glad that you find it helpful and useful! It is in our common interests to contribute to this common collection of information about ASPS and its management - even for the people when their treatments are unsuccessful - then other patients with ASPS could decide to try something else instead and later let us know so everyone would benefit.
Regarding Pazopanib. It is very good to hear that you are having a very good response to it and hoping for a complete response. The problem is an accrued resistance to TKI inhibitors, also there are the known cases of the faster regrowth of the mets when the anti-VEGF drug is discontinued, even if the patients appeared to be as having a complete response - i.e. no mets were seen anywhere on the scans. They could have been to small to see even with the 1-2 mm resolution or there might have been other dormant micromets that started to grow after the TKI was discontinued.
There is some article that explains the resistance developing http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3778288/
the tumors that respond well are at risk to develop resistance and you should watch that bigger met very carefully and if it starts to re-grow then you should treat it locally as soon as possible - cryoablation is avail. in China also RFA. There are also other TKI drugs that could be used if the resistance develops.
So to summarize, we do not have an information that would suggest that people having a complete response to TKI could safely discontinue the drug. We only have 1 person having a complete response to another TKI drug cediranib and she is on this drug as a part of the clinical trial for years now. She would love to discontinue cediranib but despite having no visible tumors lef she remains on the drug, fearing the recurrence.

[Bonni Hess]

Dear Lisa,
I join Olga in welcoming you to this Discussion Board and Hoping that you will find the shared information and support helpful in fighting your disease. I am so very sorry for your ASPS diagnosis and all that you have been through thus far, but am grateful that you found your way to the CureASPS Web site and that you have now reached out to share your diagnosis and treatment information and to request input and advice from other ASPS Community members. I am grateful too that you have been tolerating the side effects of your five months of Pazopanib treatment well and that thus far you have had a successful response to the Pazopanib with significant disappearance/shrinkage of your lung mets. I am holding very tight to Hope that your upcoming CT scan at the end of this month will show continued disease stability with no new tumors and additional shrinkage/disappearance of your remaining lung mets. Hopefully your CT scans include the abdominal and pelvic area as well as your chest as it is critically important to monitor all of these areas with regular scans every 3-6 months in addition to an annual brain MRI and full body bone scan to ensure that any new mets are found at the smallest most treatable size. Based on our 13 and a half years of ASPS experience with our daughter Brittany, we have found that even though mets may be appear stable in one area of the body such as the lungs, they unfortunately may develop and grow in other areas which is why it is so very important to adequately scan all areas of the body and to not just rely on/trust chest CTs to accurately indicate the status of the disease as too many oncologists naiively/erroneously do. I strongly agree with Olga regarding the concern about discontinuing Pazopanib even if there are no visible mets on your next or subsequent scans. It is our daughter Brittany who Olga referred to as the patient who has had a very successful and sustained response to Cediranib thus far with very thankfully five and a half years of stable disease and apparent disappearance of her previously innumerous and widely disseminated mets. However, although it appears on the scans that she is now tumor free, her very knowledgeable and experienced Clinical Trial oncologist feels that she may be macroscopically tumor free, but unfortunately not microscopically with microscopic tumors too small to be seen on the scans still in her body, and that discontinuation of the Cediranib could devastatingly result in rapid disease progression which is a chance that we are unwilling to take. Brittany will therefore continue to take the Cediranib as long as it is providing disease stability, and until a more permanent treatment and cure can be found. I am not sure where you heard/read, or who told you that "My understanding is that, if there is no met, it is unnecessary to continue the TKI treatment.", but it is not something that I am personally familiar with despite my extensive research and experience. I would be very concerned about you making the decision to discontinue your Pazopanib if your scans show no mets, unless there is well documented and verifiable up to date researched and anecdotal data to support this decision and it has been thoroughly discussed with and agreed to by several different ASPS knowledgeable oncologists. Our theory and approach has been not to rock the boat if something appears to be working especially when there are so very few promising treatment options available for ASPS at this time. Additionally, even though the Pazopanib thankfully appears to be stabilizing the progression of your disease and shrinking and destroying your mets, our experience has shown that sometimes this may just be the nature of this unpredictable disease rather than the effect of the systemic treatment, and it requires continued vigilant monitoring to try to determine whether the treatment is actually being effective or the disease is just doing what it does. I applaud your pro-active approach to your disease dear Lisa and encourage you to proceed with caution and as much knowledge as possible before making any treatment decisions. I also encourage you to actively participate on this Board as your time and the situation allow because shared information is one of our most powerful weapons in fighting this extremely rare and very challenging disease. Take care, keep in touch, stay strong, let Hope lead you through each day, and know that my most caring thoughts and very best wishes are with you.
With special hugs, deepest caring, healing wishes, and continued Hope,
Bonni Hess, mother of now 32 year old Brittany diagnosed 13 and a half years ago at age 19 in July 2001

[Lisa]

Dear Olag and Bonni Hess,

Thanks for your quick reply! Your words do give me some helpful deeper thoughts

I did regular check of my primary, brain and abdomen every three months, everything is clear. As to the pelvic area mentioned by Bonni, I did a pelvic MRI about 4 months ago to check my iliac bone ,is it necessary to do it regularly if everything is good?

Regarding to the idea that I want to stop taking Pazopanib, there's one doctor suggested me that TKI treatment should not be considered when the disease doesn't metastasize to other areas. He also mentioned that if there is no visible tumor, there is no need to use TKI drugs as well. I am not sure is that because Chinese doctors do not have much experience in using these drugs, especially Pazopanib.

There is one Chinese patient who took Sutent for some time, and then she discontinued the treatment as the scan showed all the mets disappeared. 2 years later, there's some new mets shown in CT scan and she considered some treatments again.
My original thought is that I can stop treatment and monitor it regularly, if there's new mets appear, continue TKI treatment or cryoablation. Since there is no visible mets, it is hard to say whether the drug is effective in maintaining the progress of disease. And the average time that Pazopanib develops resistance is about 1 year, should I continue to take the drug after one year if the disease is still stable (no new mets)? And there are also some damages to liver and heart resulted by pazopanib cannot be ignored.

But your replies let me to make more considerations. Thank you guys again, this forum is really a valuable resource for me and all the patients. I will keep to participate on this board and update my situation timely~

Lisa

[Olga]

Hi Lisa,
it is very interesting to hear that there is a patient with ASPS that had a complete response to sunitinib (if I got it right). Good to know and she is def. a good candidate for another TKI drugs.
I completely agree that TKI treatment should not be considered when the disease doesn't metastasize to other areas. As the matter of fact I think that many of the metastatic stage ASPS patients could also benefit from leaving this TKI option for later if there are other treatment options available - local - surgery, cryo or RFA ablations, radiosurgeries, as the resistance usually develops. If there are other TKI available, they can be tried in case of resistance event but the response from the second/third line TKI is usually shorter. On the other hand since there is no Phase 3 clinical trial re. if they actually prolong patient's lives in ASPS, this is all left to a patient to decide. The stats are really limited. My hope is that may be Chinese doctors would be willing to collect more info re. TKI uses in ASPS or even conduct a clinical trial as the population size would allow for the faster clinical trial enrollment? What do you think? Is the cost of the TKI drugs covered by the government?

[Bonni Hess]

Dear Lisa,
I agree with Olga that TKI treatments such as Cediranib or Pazopanib should be reserved for those patients whose disease has metastasized to areas of the body, or who have mets, that cannot be treated with resection, ablation, or radiosurgery. This was the case with Brittany when she developed an unresectable/untreatable met in the head of her pancreas and the only choice and treatment option that we had was a systemic treatment like Cediranib which could cross the blood brain barriar to prevent new brain mets since Brittany was experiencing continuing and increasing development of brain mets in addition to rapidly developing and growing other innumerous and widely disseminated mets throughout her body. We understand that Cediranib is unfortunately not a cure, but our Hope is that it will provide sustained disease stability for Brittany until a permanent treatment and cure can be found. Since you have already begun treatment with Pazopanib, I personally feel that it would be risky to discontinue the drug even if you have no scan visible mets, due to the fact that microscopic mets are not visible on scans (as is verified when Dr. Rolle, the lung met laser resection surgeon in Germany, digitally feels and removes many more lung mets than are visible on scans), and the chance of rebound and aggressive disease progression is a well known possible consequence of discontinuing TKI drugs. I know that there may be possible risks of long term Pazopanib treatment such as possible liver and heart damage that you mentioned, but you have to weigh the risk of developing disease progression that may not respond to other treatments against the other risks. As with everything with fighting this extremely rare and poorly understood disease, treatment decisions are very difficult, but equally critical and the patient needs to be as well informed and knowledgeable as possible.
I am grateful that your doctors are so pro-active in providing regular scans to monitor your disease which is so very important. Regarding your question about having regular pelvic scans, they are equally important to the every 3-6 months chest/abdominal scans since ASPS mets can and do develop in the pelvic area. Actually, we have found that ASPS mets can unfortunately develop in almost every area of the body and regular and vigilant scanning is crucial in fighting and managing this unpredictable disease.
My most positive thoughts and very best wishes are with you for very good scan results at the end of the month and I will be anxiously awaiting your update and your treatment decision. Take care dear Lisa.
With special caring thoughts, healing wishes, warm friendship, and continued Hope,
Bonni

[D.ap]

Hello Lisa

My name is Debbie and our son Joshua is an ASPS patient since Sept of 2012.
I was reading your posts and came across a New England study on sunitinib verses pazopanib discussion.
It is not specific to ASPS however it gives comparisons in how it was tolerated by the two groups and the response stats as well to controlling the renal cell carcinoma.

Titled "Pazopanib versus Sunitinib in Metastatic Renal-Cell Carcinoma"

http://www.nejm.org/doi/full/10.1056/NE ... Discussion

Hope you are doing well.

Sincerely
Debbie


I second Olga's statement

I completely agree that TKI treatment should not be considered when the disease doesn't metastasize to other areas. As the matter of fact I think that many of the metastatic stage ASPS patients could also benefit from leaving this TKI option for later if there are other treatment options available - local - surgery, cryo or RFA ablations, radiosurgeries, as the resistance usually develops. If there are other TKI available, they can be tried in case of resistance event but the response from the second/third line TKI is usually shorter

AS Olga and a lot of people who've been dealing with this sarcoma have said time and time again, sometimes it better to do nothing than something. I know as a patient you just want it gone but using the wrong technique can do more harm than good in the ling run

[Lisa]

Hey everyone,

Having been busy recently. Sorry for late updating my recently check-up.

After taking Pazopanib for 5 and half months, I had the second chest CT scan since the treatment at the end of February and luckily, as my best expectation, there is no nodule on my both lungs (at least, no visible mets on the images). I know most of you worry about the risk of terminating TKI treatment, but after discussing with doctors, I decided to stop taking Pazopanib and do the chest CT scan regularly. if there are new nodule appears, I would think about the TKI treatment again or cryo ablation for some mets in big size.


Now I'm back to the normal live without side effects of Pazopanib and I'm trying to strengthen my immunity by healthy diet and regular exercises. I hope my lung could be clear in the next CT scan scheduled in late May.

[Bonni Hess]

Dear Lisa, It is so very good to hear from you with the very good news of your most recent chest CT scan results which very thankfully and encouragingly showed no remaining scan visible lung mets I am so happy for this wonderful.news, and am holding very tight to Hope that you will.have continued and sustained disease stability with no rebound now that you have discontinued your Pazopanib treatment. You said that you will now be followed with regular chest CT scans with the next one scheduled in May, but I strongly encourage you to also have regular abdominal/pelvic CT scans at the same time as your chest CT's because with this unpredictable disease, ASPS mets can sometimes develop in other areas of the body even if the lungs appear stable and it is critically important to be vigilant in having adequate scans including a brain MRI and full body bone scan at least once a year. In the meantime, take care, celebrate the great joy and relief of your good scan results, enjoy your freedom from the negative side effects of the Pazopanib now that you are no longer taking the drug, and keep in touch with the Board as you are able. Sharing the great joy of your good scan results with happy hugs, best wishes, and continued Hope, Bonni

[MartinBube]

Hi Lisa

excellent news!!! Hope that you continue to be in excellent health

I do agree with Bonni regarding the CT but since it does have big radiation maybe MRI of abdomen and pelvic will be enough.

[Bonni Hess]

Hello again Lisa, I agree with Martin that abdominal/pelvic MRIs are preferable to CT scans due to the concerning radiation exposure from CT scans, but unfortunately it is sometimes difficult to persuade the doctors to order MRIs or to get the insurance companies to agree to pay for MRIs instead of CT scans due to MRIs being much more expensive than CT scans. The important thing is that your disease be vigilantly monitored with adequate regular scans and not only with chest CT scans as many doctors erroneously and inexplicably do since chest CT scans alone do not always adequately reflect the status of the disease which may appear stable in the lungs but yet might be developing in other areas of the body and can go unnoticed until mets become symptomatic if regular scans are not done to find mets at their smallest most treatable size. With special caring thoughts, healing wishes, and continued Hope, Bonni

[D.ap]

Hey everyone,

Having been busy recently. Sorry for late updating my recently check-up.

After taking Pazopanib for 5 and half months, I had the second chest CT scan since the treatment at the end of February and luckily, as my best expectation, there is no nodule on my both lungs (at least, no visible mets on the images). I know most of you worry about the risk of terminating TKI treatment, but after discussing with doctors, I decided to stop taking Pazopanib and do the chest CT scan regularly. if there are new nodule appears, I would think about the TKI treatment again or cryo ablation for some mets in big size.


Now I'm back to the normal live without side effects of Pazopanib and I'm trying to strengthen my immunity by healthy diet and regular exercises. I hope my lung could be clear in the next CT scan scheduled in late May.

Hi Lisa

I am glad to hear of the getting off of pazo. What good news of no visible tumors!
The mg was 600? How was that determined? Dose reduced as per side effects?
What was your doctors explanation for the reduction?
Please check in and let us know how the scan in May looks.
My hope for you is regaining your strength and immune system quickly and that you start to feel better with each sunrise.

What was your mitotic rate on your small tumor?

Look forward to hearing from you when time allows

Love
Debbie

I hope you begin to feel better with each day.

[Lisa]

Hi Bonni and Martin, thanks for your remind of abdomen, pelvic and brain check. In last and half years, I did ultrasound examinations every three months . And I did abdomen CT once, pelvic MRI once and full body bone scan once. For the brain, I did MRI scan every three months. Are ultrasound examinations able to detect small metastases to abdomen and pelvic? As to abdomen and pelvic check, should I do MRI with contrast or without contrast?

Debbie, yes, I took 600mg during the 5.5 months treatment. The initial dose that advised by drug producer is 800mg. However, doctors think 800mg may be too much for Asian because most of patients can only tolerate 400mg. Therefore, they suggested me starting with 600mg. Since I didn't have any serious side effects, I continued this dose. Some Chinese patients have some serious side effects that cannot bear, they reduced dose to 400mg. "mitotic rate"? I have no idea about it. how this can be calculated?

And guys, I have a question about hormones. Some Chinese patients recently found some relations between progress of disease and sex hormones, and considering whether ASPS is a kind of hormone-dependent neoplasm like some breast cancer patients who have positive Estrogen-receptor (ER) and Progesterone receptor (PR). And some of us did the Estrogen and Progesterone Receptor Test, there are about 60% of patients have positive ER and/or PR, although most of them are weak-positive （only have one '+'） and sample size is limited (till now, about 15 patients have done in total). It also found weak-positive ER in male patients. There are two patients have 40%+ in PR. And me, has the strong positive results in both, with 80%+ ER and 80%+PR.
Is there any medical publications investigating this problem? We didn't find any useful literature. I know this is just a basic wild guess based on the simple theory but is it possible to try endocrine therapy like breast cancer? Stopping the progress of tumor by blocking the supply of hormone?

[MartinBube]

Hi Lisa,

I believe that MRI should be done with contrast, also ultrasound is not enough according to me to determine any mets until they become bigger.

Regarding mitotic rate it should be in your pathology report. If not there you can not calculate it. Basically it shows how fast is the split of the cells.

nice weekend

[Bonni Hess]

Dear Lisa,
Thank you for the additional information. It is concerning to me that only abdominal and pelvic ultrasounds have been done to monitor your disease because ultasounds are inadequate to detect small mets. I strongly encourage you to request and have regular (every three to six months) abdominal/pelvic CT's or MRI's with contrast to adequately monitor those areas of your body for any possible mets, and to continue to have at least annual brain MRI's and full body bone scans. Adequate and appropriate scans are vital to try to successfully manage and fight this upredictable disease. Unfortunately and inexplicably, too many doctors fail to provide adequate and appropriate scans for ASPS patients often only following the disease with chest CT scans which ARE NOT adequate since this insidious disease can, and too often does, metastasize and grow almost anywhere in the body.
Regarding the hormone issue, based on our research and anecdotal experience observations, we have long suspected that hormones may increase ASPS disease progression and for that reason Brittany does not take any hormone supplements, use contraceptive pills, and even if she wasn't on the Cediranib which prohibits pregnancy, pregnancy is not an option for her for now due to the risk of the pregnancy hormones causing disease progression. Unfortunately there does not seem to be much definitive published data regarding this issue. More research needs to be done, and more anecdotal evidence documented to confirm or disprove the theory of hormone related ASPS disease progression.
With special caring thoughts, healing wishes, and continued Hope,
Bonni