Naaila from UK

[HaqStar]

Hi everyone

My name is Naaila (pronounced Ny-la. I am 21 years old (turning 22 next month). For the past few months I had been suffering from lower left back/leg pains which we thought was sciatic pain. I began regularly exercising which seemed to work for a few months. However, at the start of November 2012 the pain migrated to the left groin at which point my husband (a first year trainee in Family medicine here in UK ) thought this could be something to do with my ovaries. We went to my GP who referred me to a sonographer where they performed a few scans which revealed a mass on the left side.

At first we thought this could be an ovarian cyst, however, when we saw a Gynae consultant he said he wasn't sure what it was and didn't think it was ovarian, so he scheduled me for an MRI scan. Luckily, my husband was working in the hospital at the time and managed to get me an MRI within two days of the consultation. The next day I was called for a CT scan, and within a few days we were told this could be a sarcoma. A few days before New Years I had a biopsy and bone scan, the results of which confirmed I had ASPS in the left pelvis, and also numerous mets in both lungs. Obviously, this was a huge shock for the whole family.

Since then, we've seen a number of oncologists at UCH, who have told us that they could schedule me for daily six week intensive radiotherapy for the main tumour, and not at all for the lung Mets because they are bilateral. Having said this, they do not have a positive outlook, and so we've enquired about a number of clinical trials that I may be eligible for. Surgery is a very grim option since the size of the tumour is very big, and would involve removing half the pelvis and result in left leg amputation, something which I'm wanting to avoid at all costs. I'm seeing another oncologist at UCH this week regarding the trials currently in place, so we are hoping for some answers and a possible treatment plan.

Trying to be strong and stay positive, and thankfully have been blessed with very supportive family and friends. I hope I can find some more info on this site and gain inspiration from all the strong people going through what I am!

Sincerely, Naaila.

[Olga]

Naaila, welcome to this board. I hope that we will be helpful to you in finding the best possible way to manage this disease or at least not to feel alone, there are many people in the same situation like you that participate in this board.
I would suggest you to contact Pf.Judson as he is the most ASPS knowledgeable oncologist in UK and all the clinical trials that are suitable for ASPS are run under his supervision. He can be found here:
http://www.royalmarsden.nhs.uk/consulta ... udson.aspx
I think that it was recommended for UK oncologists to refer the ASPS and other rare sarcoma patient to him for his consultation, if your oncologist refuses to do so, make a self referral - contact Pf.Judson.
As I understand from your description, you have an unresectable pevic soft tissue tumor, bone pelvic metastasis and multiple lung mets - what are the sizes?
The plan that is offered to you complies to the general sarcoma treatment guides in UK. We have cases when this situation was treated differently in other countries but of course it depends on the size and location of the primary tumor and metastases. In some cases the less clean from the surgical oncology point of view was performed and metastases were treated later or selected most dangerous metastases were treated before going to a clinical trial - not with the curative intent but with the intent to improve the quality of life and prolong it - palliative, you probably realize by now that the metastasis to a pelvis might represent more immediate problem than the slow growing lung mets, this approach is rarely used in the faster growing cancers or sarcomas but in ASPS it may have a sense as even people with the metastases may survive for a long time - but only if they are controlled. I have seen in the publications, that cryoablation or neutron radiotherapy was performed to shrink the unresectable primary and make it resectable.

Some reading on the subject (your husband might have an institutional access to the full texts):
http://www.ncbi.nlm.nih.gov/pubmed/21514588
http://www.ncbi.nlm.nih.gov/pubmed/20574801
http://www.ncbi.nlm.nih.gov/pubmed/17415009
isolated pelvic perfusion (IPP)
http://www.ncbi.nlm.nih.gov/pubmed/22241290
http://www.ncbi.nlm.nih.gov/pubmed/18577862
http://www.ncbi.nlm.nih.gov/pubmed/14677093
http://www.ncbi.nlm.nih.gov/pubmed/3234087
I think its enough to start:(
Good luck,
Olga.

[Bonni Hess]

Dear Naaila,
As with everyone else on this Board, I am so sorry for the reason which brought you here, but I am grateful that you found your way to this Web site and that you reached out for information and support. My heart breaks for all that I know that you and your husband have been going through, but I am so glad that you have a very supportive family and friends which is so important in this difficult battle. As you now know, ASPS is an extremely rare, little known (even by many oncologists), and challenging disease. CureASPS is an invaluable interactive resource for vitally important shared researched and anecdotal treatment information and strengthening support and encouragement for ASPS patients and their families all over the world. I Hope that you and/or your family will actively participate on this Board because shared information is one of our strongest weapons in fighting this insidious disease.
Olga, who is extremely knowledgeable, has given you some excellent information and advice, all of which I agree with. I too strongly recommend that you contact Professor Judson at the Royal Marsden in London as he is a very experienced and highly respected sarcoma specialist who has treated a couple of other UK ASPS patients who I am personally familiar with. Since your large primary pelvic tumor is apparently unresectable without a major and very extreme resection it is very important that some other treatment be used to try to shrink/destroy it. Based on my personal experience and knowledge I think that this needs to be the highest and most immediate priority, and then treatment of your lung mets can be addressed once the primary has been shrunk and Hopefully successfully removed/destroyed. It seems that cryoablation or radiotherapy of the primary may be your best treatment options at this time since most currently available systemic treatments have unfortunately shown very little success in shrinking/destroying large ASPS tumors.
I know that you must be very frightened and overwhelmed with everything right now dear Naalia, but know that you are not alone in your battle and that those of us on this Board are here to try to help you in any way that we can with shared information, input and advice based on our combined experiences and knowledge, and with our support. Please take care, stay strong and positive, let Hope lead you through each day, and keep in touch as you are able. We truly care and understand.
Reaching out to share gentle hugs, special caring thoughts, healing wishes, and continued Hope,
Bonni Hess, mother of 30 year old Brittany diagnosed at age 19 in July 2001

[HaqStar]

Dear Olga and Bonni

Thank you for the response. The tumour is about 9x8x7, and I've spoken to three oncologists now who have all said that radiotherapy will be ineffective (due to the lung mets which are up to 18mm). They have scheduled me for an MRI and CT scan two months from now, so as to map the size of the tumours. If they find that they are growing, then they will start me on the Cediranib trial, which I understand is under the supervision of Prof Judson. If the tumours are not growing, then they will start me on the Crizotinib trial.

Symptom wise I am doing quite well, and the doctors and nurses are all quite surprised that I am on minimal pain killers given the size and location of the main tumour. They've reassured me numerous times that the tumours are very slow growing and so I can afford to wait the two months before any treatment plan is offered.

In the meantime, I am trying to carry on living my life as normal, and may start attending university again if I feel I am able to handle the commute!

[Bonni Hess]

Hello again Naaila,
Thank you for the additional information. I am grateful to hear that you are not currently experiencing very much pain from your large primary tumor, and that you are able to control the pain with minimal pain medications. I am confused about the oncologists feeling "that radiotherapy will be ineffective (due to the lung mets which are up to 18mm)." Perhaps I misunderstood and you mean that they think that radiotherapy would be ineffective for the lung mets, but if they are referring to it being ineffective for the primary due to the lung mets, I don't understand that there is any correlation between the two situations. Based on our personal experience with Brittany's lung mets and my observations of Ivan and other ASPS patients whose lung mets have been Cryoablated, Cryoablation can definitely be successful in treating 18 mm. lung mets and could possibly be a consideration for you in the future, but would need to be done before the mets become larger than 3 centimeters. However, I continue to personally feel that treating/shrinking the primary needs to be the primary and immediate focus at this point, and I don't understand why the doctors want to delay for two months given the large size of the tumor which will unfortunately most likely continue to grow and increase in size without any type of intervention. My concern regarding a systemic treatment with Cediranib or Crizotinib prior to shrinking the primary remains that neither of these treatments seem to have shown much success in shrinking/destroying large tumors, so it seems that radiotherapy or Cryoablation should be used prior to systemic treatment to try to shrink the tumor as much as possible and thus enable the systemic treatment to be more effective. I would again urge you to schedule a consultation with Professor Judson as soon as possible and not delay for two months until you have another MRI and CT. I also encourage you to request a brain MRI and full body bone scan as soon as possible, if they have not already been done, to ensure that there are no mets in those areas of your body. Complete, vigilant, and regular scanning is vitally important in fighting/managing this disease so that any new mets can be found and treated at their smallest possible size. I know that your doctors have reassured you that the tumors are slow growing since ASPS is typically an indolent disease, but when there is a large tumor burden such as your primary the disease can also become more aggressive and requires very pro-active treatment to try to remove/shrink the large tumor to help slow/stabilize progression of the disease. I really admire your very positive attitude which is very important in this difficult battle, and your desire to continue Living Life as normal as possible, but I think that it is critically important right now to be extremely pro-active in aggressively pursuing treatment of your primary as your first priority until progression of your disease can be stabilized. I know that I am not a doctor, and there are certainly no definitive answers with this extremely rare and poorly understood disease, but based on my eleven and half years of ASPS experience, relentless research, and constant networking with other ASPS patients and their families, I have learned that the doctors do not always have the best answers or advice and it is extremely important to be as well researched, knowledgeable, pro-active, and assertive as possible in fighting this very challenging disease and making treatment decisions. I Hope that I am not frightening or offending you with the information/input that I have shared dear Naaila, but I care so deeply about you and all of our ASPS Family, and I just Hope that you and others can benefit from my hard earned experience and I can help prevent some of the mistakes that have been made. Please take care, let Hope lead you through each day, and keep in touch as you are able.
Heart to heart with special caring thoughts, healing wishes, and continued Hope,
Bonni

[HaqStar]

Dear Bonni and Olga,

This is Faisal, Naaila's husband (using her login with permission of course, lol). Naaila asked me to take a look at Bonni's post about managing the primary.

Firstly, thank you both for the interest you are showing in her case and for her well-being. Being a believer, I say may God reward you for the good in your intentions. The collective expertise, knowledge and sense of community gleaned in a forum like this is invaluable, not to mention encouraging.

Further to Bonni's most recent post, allow me to clarify: Naaila has a large pelvic primary probably arising in the left Obturator Externus (a muscle just behind the buttock muscle)and invading the posterior aspect of the pubic bone, filling the pelvis, pushing the vagina, the colon and indenting on the bladder. it is a highly vascular tumour (as you'd expect) with lots of signal uptake. her Hb has dropped from 10.5 last year to 9.8 simply on account of this. According to the surgeons at the London Sarcoma unit, primary surgery would involve hemipelvisectomy and hind quarters amputation and would not improve the long term survival on account of mets. We have requested a second opinion from another surgeon in Leeds (which is a smaller unit that London) to reconsider whether a less debilitating surgery would be possible to take the primary. Do let me know if you guys have any info on this.

Radiotherapy to shrink the tumour was considered but upon discussion with the radiation oncologist (Dr. Cassoni at the University College Hospital in London) it seemed that radiotherapy would not be particularly effective apart from palliation, which Naaila doesnt need at the moment. Radiotherapy would also render her infertile, which was something Naaila struggled with more than the prognosis of the illness itself. We were given no indication that shrinking the primary with radiotherapy would improve the likelihood of future removal.
Further to Olga's post previously, I discussed the use of cryoablation for the primary and Nd:YAG laser for the lung mets, and was informed that although the NHS has access to cryoablation and laser therapy, her mets were considered too numerous (i counted about 15 myself, these are bilaretal, multilobular) and widespread for this treatment. This was my conversation with Dr. Strauss a member of the EORTC, and a colleague of Prof. Judson, also re-affirmed that removing the primary would not affect her long term survival, and symptoms she doesn't have. would you still recommend a second opinion with the Prof? please advise.

As you may have guessed, the impression I have been given so far is that although there is a lot that can be done, but not much that would be effective in her case. I went through the first four of Olga's links with great interest, however do note that many of the procedures presented were for sarcomas in general and
not specific for ASPS. Have yourselves had any different expereinces to this? Do you know anyone with a large pelvic primary treated with cryo or radio f/b surgery? She has had a bone scan that hasnt picked any other mets, and a brain scan has not been performed as according to several studies, asymptomatic brain mets were never seen. (i'd appreciate if you'd enlighten me on this as well...)
We have sought a second opinion from John Hopkins and they are awaiting her imaging pictures. Is there another centre in the USA where you would recommend a second opinion perhaps?

Thanks a lot.
Sincerely
Faisal

[Olga]

Faisal, welcome to the forum.

We had few cases with the similar location of the large ASPS primary that was initially said unresectable but later removed by the second (third etc) opinion surgeons in some cases with the suboptimal marginal resection, no hemipelvictomy was done and it really shouldn't in stage 4 cancer. In one of these cases it seems that there is a local recurrence few years later. In all cases there were no direct bone invasion (and it is also rare for ASPS to invade the next structures as it grows very slowly and usually just displaces them). So it would need to be investigated if there is indeed the bone invasion?

There is a contact info re. surgeons performed one of these cases:
Cancer center at the University of California, Los Angeles (UCLA). Drs. Frederick and Fritz Eibler are a father-son team specializing in sarcoma
http://www.cancer.ucla.edu/index.aspx?page=383

I do not remember if we had any personal experiences with the neutron or cryo to the abdominal primary, we had a positive experience with the radiosurgery to that location but on a much smaller size. We had a positive experience with the neutron radiation to the primary and ASPS is listed as a neutron sensitive sarcoma at the Fermilab http://www.neutrontherapy.niu.edu/neutr ... ssue.shtml

We had a very extensive experience with the laser assisted lung surgeries and it is my strong belief that 15 mets should not be considered too numerous not for the laser assisted surgery not for the regular one (cherry picking). We even had the cases of the really long term survival after the incomplete lung surgery with the bigger mets resected followed or preceded by the chemotherapy treatments or IFN and the lung surgery is a well established treatment modality for ASPS as the response to the chemotherapy in ASPS mets is always heterogeneous (if it responds at all) and some mets need to be removed.

I am not sure what to say re. consultation with Pf.Judson. He is a very valuable researcher for our ASPS community and most of the important ASPS clinical trials originate under his supervision. He is also one of the authors of the UK Guidelines for the management of soft tissue sarcomas and all the docs are saying to you is said according to it - they are focused on optimizing a health care in the situation with the limited financing. Your wife case is very advanced and yes, she may loose the quality of her life from the primary surgery faster that it is going to be taken by the ASPS itself. And no lung surgeon is going to even contemplate the surgery before the primary is removed as it is not what is going to shorten her life (the same goes for the question if there are possible brain mets - it is not the immediate problem). But on the other hand the primary esp. if there is a direct bone invasion is going to be her problem soon and I would concentrate all the efforts at looking if there is anything that can be done to shrink it and remove it or at least to control its growth.
Asymptomatic brain mets are often seen - when they are smaller than 5-7 mm - which is also the size when the radiosurgery is very effective, but again it is not the first concern now.
I would start contacting docs from the links with the interesting modalities and asking them if they are agree to review the scans and if they had any positive experiences with the ASPS. But it is very unlikely that UK health trust is going to pay for it and going out of country for a treatment is a very expensive and contradictory move - you can go into the conflict with the local docs and that might be a problem.
You can also ask Prof Vogl:
http://radiologie-uni-frankfurt.de/cont ... x_ger.html
Anyways you have 2 months they decided to wait to measure the growth so use this time for the consultations. You will need the copies of the abdominal scans on the CD and the pathology report.

[Bonni Hess]

Dear Faisal,
I am glad that you have joined the Board (but am very sorry for your reason for being here), and deeply appreciate the additional information that you provided which is very helpful. I agree with everything that Olga has said and her referral to Dr. Fritz and Frederick Eiber who are excellent, very experienced, and highly regarded sarcoma surgeons in California who would certainly be worth trying to obtain a second opinion from regarding the possibility of surgically removing Naiila's very large and difficultly located primary tumor. Dr. Fritz Eiber was able to successfully remove a pelvic area tumor from one of our young ASPS patients with no impact to adjacent organs when other surgeons had said it would be necessary to remove many of her pelvic organs. I, like Olga, continue to feel that removal/shrinkage/treatment of the primary tumor needs to be the greatest and most immediate priority and focus. Although treatment of the primary needs to be the focus, I do feel that a brain MRI should be done and I strongly disagree with the studies you have read which indicate that "asymptomatic brain mets were never seen." Based on our experience with Brittany's multiple brain mets, once the brain mets are symptomatic, they are usually too large to be successfully treated with Gamma Knife as happened with her first three initial brain mets, and if they can't be safely resected then there tragically may be no remaining treatment options. ASPS brain mets must be found at their smallest most treatable size through an MRI scan (a brain CT is inadequate to diagnose small ASPS brain mets). Several of Brittany's brain mets were found through regularly scheduled brain MRI's when she was not experiencing any symptoms, as opposed to her three largest brain mets which were diagnosed only after she began experiencing headaches and other concerning symptoms.
Please know that my continued most caring thoughts are with Naaila and you, and that I am here to try to help in any way with shared information/input based on my eleven and a half years of ASPS experience, knowledge, and research. Take care Faisal, give Naaila and yourself special hugs from me, and keep in touch with the Board as you are able.
With deepest caring, healing wishes for Naaila, and continued Hope,
Bonni

[Ivan]

Faisal

How large are the biggest of the 15 mets that you saw on the lung CT? I think it would be very good to find someone who is willing to operate on the primary tumor without amputation.

Cediranib is a good bet, but you must come off it to have surgery. Once you are off, they won't let you back onto the trial!

[Olga]

I was wondering if you guys have seen this article:
Alveolar soft part sarcoma of the retro peritoneum.
http://www.ncbi.nlm.nih.gov/pubmed/20479565
it is avail. in a full text - link on the page - and it is interesting how they describe the preop imagin findings - that it was in a close contact with the surrounding structures - and the Intra operative findings revealed a large, encapsulated tumor that had no adhesion and invasion into anything it was pushing at.

[HaqStar]

Thank you Olga, Bonni, Ivan for your replies

Unfortunately I can confirm that the original has invaded the left pubic bone. It was for this reason this was initially thought to be an Ewing's Sarcoma of left pubis. Histology and immunohistochemistry changed the course of thought. In retrospect I can now relate that the tumour may have arisen from the Obturator because Naaila would complain of 'bum pain' after long journeys- it was always the left one. ( we blamed it on the car seat then)

I have emailed Drs. Eiber, Vogl and the fermi lab for their input, apart from the review @ John Hopkins originally mentioned. Thanks for the recommendations. I am also going to contact the team tomorrow to ask for brain imaging. It will be a difficult struggle, but I think in a condition as rare as this to have one case with asymptomatic brains is enough to warrant a surveillance scan. If the NHS doesn't pay for it, the we will. (Still need GP referral for that though)

I agree about Olga's comment about cost-effective management, and although we are not too well-off, we do have enough to pay for more enthusiastic treatment, if that is the right thing to do. My concern is overenthusiastic treatment done simply because it can be done , which may not really improve the long term outcomes, given the slowness of the original illness.....

Ivan, the largest of the mets was 18mm in diameter.

Meanwhile Naaila continues to improve clinically, with minimal functional limitations apart from the odd twinge here and there.
I intend to pray for Brittany and Ivan tomorrow
Olga, how did you come to this forum? Do you also know someone with ASPS. Thank you.
Faisal

[Bonni Hess]

Dear Faisal,
Thank you again for the additional information. I am so sorry that Naaia's primary pelvic tumor has invaded the bone which I know unfortunately poses additional obstacles to resecting the primary. However, despite the bone invasion, I still feel that it is extremely important to seek second, third, and more if necessary opinions from other experienced Sarcoma surgeons regarding the possibility of successful resection of the primary. I applaud you for being so pro-active in so quickly moving forward with seeking a review of the situation and recommendations from Drs. Eiber and Vogl. I am holding VERY tight to Hope that you will receive some positive and encouraging responses and input from them. I Hope too that you will be successful in obtaining a brain MRI for Naaila. I don't understand why the doctors would be resistant to scheduling this vitally important scan given the documented risk/nature of ASPS to metastasize to the brain, and knowing the critical importance of finding and treating ASPS brain mets at the smallest most treatable size.
Thank you for your very kind and caring thoughts and prayers for Brittany and Ivan in the midst of all of your own great heartaches and challenges right now. As very difficult as fighting ASPS is, we in the ASPS Community are fortunate to have the special support and encouragment of each other which is so strengthening when the battle with this extremely rare disease can feel so lonely and isolating.
Regarding your question about Olga, she is Ivan's incredibly devoted mother who has aggressively worked to help fight Ivan's disease in every way possible since his diagnosis, as well as to provide shared invaluable and extremely knowledgeable researched and anecdotal treatment information to not only those of us in the ASPS Community, but also the general Sarcoma Community. In her very generous and dedicated efforts to help other ASPS patients and their families, Olga is a co-founder of CureASPS along with a couple of us other parents of ASPS patients, Web master for the CureASPS.org Web site, and a very active participant on the Web site. Her contributions to the ASPS Community have been, and continue to be, immeasurable and invaluable.
With special caring thoughts, healing wishes for Naaila, and continued Hope,
Bonni

[Olga]

Faisal, I am Ivan's mom and we founded this web-site and forum long time ago - Josef Landesman, me, Bonni and few other parents. It is run by volunteers and financed by the patients or their family members/friends.
I agree that in case the primary tumor invaded the bone, the reg. surgery would be of no use and one of the Chinese patients has already e-mailed to me that we have a case where the direct pelvic bone invasion was confirmed by the exploratory surgery that was aborted after this finding with the incomplete tumor resection.
In some cases if the invasion is small/peripheral it may be treated by the cryo and cemented. You can also go on the cediranib trial and be ready to come off it if the primary tumor responds well and became resectable or try to negotiate at the enrollment point that the primary tumor would not be designated as a target/control lesion but the lung mets would (unlikely).

But also do not overlook the regular radiation treatment for the primary as according to the latest new full free text review from Japan
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3362210/
"Four patients with stage IV disease received radiotherapy (40–66 Gy) as the only treatment for the primary tumor because of the advanced nature of their disease. None of these 4 patients showed progression of the irradiated primary tumor until the time of death or last followup (13–24 months; mean 20.5 months)." I actually have seen good (complete) responses of the ASPS bone metastases to the conventional radiation, although they were not big and mets may respond differently from the primary tumor.
Some add reading:
http://www.ncbi.nlm.nih.gov/pubmed/23169067

[Bonni Hess]

Dear Naiila and Faisal,
It has now been seven months since you last posted on the Board, and I Hope that you are both doing alright. It is always concerning when anyone loses contact with the Board for too long, but Hopefully no news is good news and you are both just busy with Life's normal activities and demands.
English ASPS patient Katrina Palmer who was until last week on the Cediranib Trial being conducted by Professor Judson, has asked me to share the following information with you so I assume that you may have been in contact with her requesting some information:

Oh and u may want to tell naiila on the curwasps board that my primary also invaded the wjole left cavity of the pelvis, it was resected down to the bone, ans still had positive margins. Luckily iv had no reocurrence in that arwa. Therebis a new brilliant sarcoma specialised surgeon based at frenchay hospital in bristol, by the name of paul wilson. I reccommwnd naiila seek advice from him about resection cudbu kindly pass this info on to her for me please xx

I Hope that this information is helpful and beneficial to you. I will be anxiously awaiting an update when your time and the situation allow. Please take care and keep in touch as you are able and know that you are both held very close in my heart and most caring thoughts.
With deepest caring, healing wishes, and continued Hope,
Bonni