Jawad from Pakistan - Dx 2010

[fawad2168]

Greetings Everybody on icureASPS Forum,

First of all i am thankful to all of you especially Olga for doing this great service to the families and patients of ASPS.

My introduction: my name is fawad, postgrduate student in internal medicine.

My Issue: My younger brother Jawad, also a doctor( postgraduate student in surgery) has recently been diagnosed as having ASPS of Rt Thigh with multiple metastases in both lungs. His primary tumor is in Rt Thigh measuring 6x11cm, MRI scan has revealed that it has not involved underlying bone and neurovascular bundle thus making primary resection with free magins possible. We are stuck up with lung mets. They are about 50 in number involving both the lungs mostly concentrated in right lower lobe which contains almost 13 to 15 nodules of variable sizes. They are intra parenchymal in distribution, Most of the nodules are soft in consistency with peripheral enhancement. NO endobronchial lesion seen.

FNAC of lung lesion proved to be a spindle cell neoplasm and trucut biopsy of Rt. Thigh proved to be alveolar soft part sarcoma which on HISTOCHEMICAL STAINING is PAS positive, Desmin negative,cytokeratin negative,CD34 negative,S100 focally positive.

He has also undergone other investigations including MRI Brain with gadolinium contrast, Bone Scan, Ultrasound of Abdomen & pelvis to rule out any other metastasis. All these investigations are unremarkable.

Complete Blood count with peripheral film,LFTs,RFTs are within normal limits.These all investigations are done in the month of august 2010.

We have discussed with oncolgists and they want to start with chemotherapy/tyrosine kinase inhibitors first and then surgical removal of the primary at a later stage. After having gone through this forum, i am in a fix regarding their approach. I am now of the opinion that jawad should first undergo surgery for removal of the primary then chemo and then removal of lung mets.

I need help/suggestion from you guys regarding the most suitable approach, choice of chemo, role of anti-angiogenics( Sutent , Cediranib or Gemzar) and surgery..
Thanking you in advance for your cooperation
Best caring wishes and hope for all

Fawad

[Olga]

Fawad, hi and welcome to the board. You do not say what are the sizes of the lung mets, where do you guys live and if you can pay for the surgery by Dr.Rolle. I would take a time and to do some preliminary work in order to make an informed decision.
1.I would send the chest scan on CD to Dr.Rolle for a review so you know if the situation in the lungs is resectable in general, you should describe the overall staging and point out that the primary is resectable.
2.If he is saying yes, them I would proceed with the primary resection ASAP, backing this decision up by the Dr.Rolle's cosult. as a rationale, because in general the approach to resect lung mets in ASPS if they are resectable.
3. Then you see how it goes.
Why I would avoid starting from the chemo/tyrosine kinase treatment. If/when the resistance develops and the progression starts at both places, you are really stuck you can not do the surgery for the lungs if there is a primary intact (no surgeon will do it) so he will have to have a surgery for the primary first being off all meds, it takes a time and the lung mets in the meantime (being resistant with more active growth factors/off any treatment) can really progress to the point where they are not resectable anymore. If you do as I suggest, in event of the residual nodules found on the consecutive scans you will have less of them to deal with so ablation can be considered for some of them and the systemic treatment can be used, if the resistance develops then switching to something else with the less significant tumor load has more chances to work and you have more time to look for something else, more acceptance with the doctors for the other treatments/surgery.
If the mets are not resectable overall (even though he probably looses his lower lobe anyways but it is up to Dr.Rolle to say), then this is a diff. story with the diff.plan.

[Amanda]

Greetings Jawad,
I am sorry that your loved one has ASPS...

I jus read what Olga posted and i agree 100%

I would get the main tumor removed asap!
Only have it removed by a sarcoma specialist!
I had mine removed by Dr Brian in Los Angeles and if you would like to send him your information i am sure he would talk to to you and give you his opinion...
He has treated others with ASPS ...

I am very glasd that the tests showed no further spread and i pray that it stayes that way. But that main tumor must be removed if possible asap it sends out cells

I will check back and see if you would like my doctors information

Take care and tell your brother that he is not alone and i send a hug

[fawad2168]

Dear Olga and Amanda
Greetings
1. I am thanful to you for such a brisk response and giving a deep thought to Jawad's case.
2. I forgot to mention that We live in Pakistan. Sizes of the lung mets are variable, about 04 are in cm the largest one is about 03cm in Rt lower lobe. Rest are in mm ranging from 1-6mm.I can tell you their exact size after having consultation with the radiologist. I want to send you Jawad's CT Scan of chest with contrast on CD for review by Dr Rolle. Should i send it through courier or share on some site. If through couries please tell me your address or i can send it directly to Dr Rolle aftrer discussing tha case with him to save th time. We sought opinion from various oncologic surgeons in Pakistan regarding the removal of primary with free margin and they say that it is possible. Regarding the disease stage, it is stage -IV due to lung mets. I think we can afford Dr Rolle's treatment. I read in one of your posts that you paid 9,000.00 euros for one session( correct me if i am wrong).
3. Dear Amanda: Thanks for sharing your experience of Dr Brian in LA. I will definitely love to consult him but let me first try to get it in Pakistan coz i want to save money for Dr Rolle. If i am satisfied with surgeons here in pakistan, we will go for the surgery here otherwise i ll seek your help.
My Regards, Best caring Wishes and Hope for all.

[Olga]

Dr.Rolle's contact info is here - the address, the e-mail etc.
http://www.ctsnet.org/home/arolle
You can send with any avail. courier the CD with the page enclosed in the same package with the name of the patient, age, general info, the assurance from the surgeons that the primary can be resected and that the result from the scanning elsewhere is negative.
You should send him an e-mail in the same time saying that you sent a CD and that people from the cureasps board told you so, that way he gets your e-mail contact info to answer when he reviews the CD. He tries to answer as fast as he can unless he is on the conference of sorts.
I agree about the primary - although it is very important that the surgeon is specialized in oncology, there should be surgeons qualified enough in almost any country to resect it, the reason we fly to have a surgery by Dr.Rolle on the lungs is rare availability of the laser/experience using it.
It is may be good to resect the primary even if the lung mets are unresectable. The primary that large produces a lot of immunosupressive stuff and he might be better without it anyways. Since there are only few lung mets that are bigger then 10 mm, you can try to ablate (RFA, Cryo) them in case Dr.Rolle could not do a surgery - just to buy a time, then to start some systemic treatment. We had a case when the multiple smaller lung mets went away after having very long course of the gemcitabine treatment (18 months was the time as far as I remember?? 3 weeks on 1 week off? reread all the patient stories), then in one of our patients lung mets responded to vinblastine/celebrex long term. The success stories are very rare and none of them happened with the primary intact, may be this is just a coincidence.
To facilitate the primary resected he might start to say that it is very painful, it helps:(

[fawad2168]

Thaks once again Olga for putting light on the subject. you are so thorough on the subject. I ll go by your advice and read all posts. I ve started working as per your guidelines. i ve sent a detailed mail to Dr Rolle with lab reports and CT Chest images in PDF format refering cureasps forum. I ve also sent a CD contining CT Chest, MRI Rt Thigh and MRI Brain images along with detailed history on a paper. Should I contact him now on telephone also? Waiting for your expert opinion.

With Best Wishes and Regards

[Olga]

His secretary is not really good in Engl speaking, but I know people managed to contact him on the phone - we usually use the e-mails. May be 'F' can comment on that one, I remember she spoke to Dr.Rolle on the phone at some point..?

[Fictional]

I'll post Dr. Rolles contact info below. Margit Kobisch is his secretary, but she doesn't speak much English.
Dr Rolle's English is OK, but don't write too much or he'll have trouble translating it or be slow to respond.

In general it seems to be a good idea to have the primary removed if possible because the primary may seed more metastases than other metastases. Dr. Rolle may not operate on the lungs if the primary has not been removed.

Some docs think being on a medication beforehand may improve the resection, but this is not certain. Our daughter was on Sutent x 6 months (primary didn't grow, but tiny lung mets did) prior to surgery, then 3 weeks off before surgery.

Surgical removal of large primaries can be a bit dangerous too though because it's thought large tumors may suppress the growth of smaller tumors (competition between tumors) - so rebound growth in metastases can occur.

In Paula / Mania's case, she had a very large primary removed - but was put on Sutent (I think?) afterwards and she has done well. Maybe the sutent suppressed rebound growth of metastases.

Hope this helps, 'F'

M. Kobisch, secretary of
Axel Rolle MD PhD
Professor of Surgery
Chief of Department of Thoracic and Vascular Surgery
D-01640 Coswig/Dresden, Neucoswiger Straße 21
Germany
Tel.: +49(0)3523 65-102
FAX: +49(0)3523 65-103
E-mail: prof.rolle@fachkrankenhaus-coswig.de

[fawad2168]

Dear 'F' / Olga

Thanks for giving contact info of Dr Rolle . Infact, I talked to him on telephone and told him briefly about my disease. He was very nice and listened about my disease. He told me to send him a CD of my scans, which i will be sending him soon.

With Best Wishes and Regards for all

[Ivan]

Great. Meanwhile, are you looking into obtaining cediranib somewhere?

[Olga]

Cediranib is not approved yet and is only avail. on the clinical trials and the only clinical trials that is open and enrolling ASPS patients is in US...

[fawad2168]

I ve searched about it and it is open in National Institute of Health Clinical Center (CC), 9000 Rockville Pike, Bethesda, Maryland 20892 USA. I ve read about their inclusion/ exclusion criteria & Jawad qualifies for that. Can somebody tell me what should be my next move to get into the trial? Should i contact Dr Kummar or just email them my documents. I can bear the expenses to fly to America and also have relatives there.

With Best Wishes and Regrads

[Olga]

We have very extensive discussion re this trial at this link -
http://www.cureasps.org/forum/viewforum.php?f=45
I understand that it takes a time to read through all the pages we have here on the forum, we are working on it:(
Some people are from outside of the country too, so they may add their comments as how did they enroll, but there is usually the contact info on the trials page at the clinicaltrials.gov
As you probably see now, there are a few options as how to proceed but you have to plan the steps carefully.

[Ivan]

I ve searched about it and it is open in National Institute of Health Clinical Center (CC), 9000 Rockville Pike, Bethesda, Maryland 20892 USA. I ve read about their inclusion/ exclusion criteria & Jawad qualifies for that. Can somebody tell me what should be my next move to get into the trial? Should i contact Dr Kummar or just email them my documents. I can bear the expenses to fly to America and also have relatives there.

With Best Wishes and Regrads

Depending on what Dr. Rolle says, that could be the best option for you. I would call them, and ask if they have space.

[Bonni Hess]

Dear Fawad,
I am so sorry about your brother Jawad's ASPS diagnosis, but am grateful that you are so supportive of him and so pro-active in reaching out for information and support to help guide him in making the best possible treatment decision. I agree with the knowledgeable advice and recommendations that have been made by the others on this Board, and, although I am not a medical doctor, would strongly encourage Jawad to pursue resection of his primary tumor before be proceeds with other treatment. Based on the anecdotal experiences of other patients who I have communicated with and closely followed during the past nine years of our daughter Brittany's ASPS battle, resection of the primary tumor prior to undergoing other treatments seems to be the best treatment approach in order to reduce the tumor burden and Hopefully help to prevent the tumor from disseminating more tumor cells into the bloodstream. The large tumor burden of Jawad's primary tumor can suppress the body's immune system and make it more difficult for his body to fight the disease, as well as making it more difficult for a systemic treatment to be effective/successful in shrinking his tumors. Several ASPS patients who I know of who opted not to have their primary tumors resected due to the difficult or unresectable location of the tumor which would have resulted in amputation, subsequently tragically lost their courageous battles despite undergoing other types of systemic treatments. The treatment decisions that are made at this point in Jawad's diagnosis are especially critical and should be thoroughly researched and evaluated. Once the primary is successfully resected and Jawad has recovered from the surgery, laser surgery with Dr. Rolle seems like a very good treatment option to remove Jawad's multiple lung mets. Once the primary tumor and the lung mets have been treated, systemic treatment with a tyrosine kinase inhibitor like Cediranib or Sutent would probably be a good approach to Hopefully help stabilize the progression of Jawad's disease and prevent the growth of new tumors. My special thoughts and best wishes are with Jawad and your family, and I will be anxiously awaiting your next update regarding the decision that has been reached regarding the treatment plan. Take care Fawad and keep in touch as you are able.
With special caring thoughts, healing wishes for Jawad, and continued Hope,
Bonni Hess, mother of 28 year old Brittany diagnosed at age 19 in July 2001