[PaulW]

Welcome to the forum Hannah.

It’s not a place anyone wants to be but, thanks to the efforts of Pan, it does provide a much needed resource to those of us with questions on LFS – something that wasn’t available before.

Very best regards,
Paul.

[PaulW]

Great job on the new leaflet Pan, very clear, concise and still comprehensive enough to give people a good start on understanding LFS and its implications.

Paul.

[PaulW]

Hi Surinder,

I’m sorry to hear that you are feeling unwell and suffering from a lot of pain. Let’s hope the TACE was successful and your tumours shrink, hopefully resulting in you feeling better.

Best wishes,
Paul

[PaulW]

Hi Surinder,

Just a quick thought regarding the pain you are experiencing, I went through a period of having quite some issues like the one you describe, I was given fentanyl patches which I’ve been using since then, I found that they really helped – overall they were more effective for me than just using oral morphine. Everyone is different of course so they may not work for you but they could be worth a try.

Best wishes,
Paul.

[PaulW]

Hi Surinder,

Sorry to read about the mouth sores. I don’t have any experience with these, none of the chemo treatments I’ve had have caused this problem for me including pazopanib. I’m sure you will get some responses from the people on the ACOR LMS list. Also I’m unaware of any other LMS version of the trial you mention.

I do hope that you manage to make progress on both of these issues and that the pazopanib does a good job for you.

All the best,
Paul.

[PaulW]

Hi Surinder,

I’m sorry to hear that your disease is progressing once more. On the positive side, I can say that I had relatively few problems with Pazopanib. I also found it very convenient with it being in tablet form, much better than going to the hospital for an infusion.

If you do have problems with side effects then you could always discuss reducing the dose with your oncologist. The normal dose is 800mg / day but I have seen many people on the Acor LMS forum who have been able to overcome side effects by reducing the dose to 600mg or 400mg / day.

Very best of luck to you,

Paul.

[PaulW]

Hi Surinder,

I hope your scan shows that the gem/tax is working well for you and that your muscle and joint pain improves soon.

Best Wishes,
Paul.

[PaulW]

Hi Surinder,

I’m sorry to hear that you are not having much luck with the treatments you have tried. Doxorubicin did not work for me either and seems to have caused me some heart problems too.

Gem/Tax was the first chemo regime that I tried. It worked for me, producing some limited shrinkage of my tumours and then giving me around four or five months off treatment before the disease began to progress again. That time off was really precious as my wife and I were able to go on safari to Zambia and also to India which was wonderful.

The most significant side effect that I had with Gem/Tax was large amounts of fluid retention. This became worse as the treatment went on (I did six cycles in total). The fluid retention caused my legs, feet, hands and arms to swell. I recall going out for dinner to a restaurant one night and having to wear my slippers because I couldn’t get any of my shoes on! Fluid retention is not a terribly common side effect but if you do get my advice to you is to make sure you take good care of your skin in the areas where the swelling occurs. I found that using a cream called ‘doublebase’ morning and night was beneficial. I suspect something like E45 cream would be of similar use.

I don’t really have any other specific advice. There are lots of people on the ACOR LMS e-mail forum who have had Gem/Tax – I am not sure if you are a member of that list but if not you could join and ask people there for their experiences (although I’ve noted that a lot of replies to that kind of question come from people who had a particularly hard time on the chemo regime in question – so the responses do give perhaps a more negative and worrying impression than is justified). To join the ACOR LMS list follow this link and subscribe:

http://listserv.acor.org/scripts/wa-ACOR.exe?A0=l-m-sarcoma

The other chemo that has worked really well for me is trabectedin (yondalis). There is some evidence that this drug works better in people with tumours that do not have functioning P53.

If you have any more specific questions let me know,
Paul.

[PaulW]

Hi Surinder,

I’m really sorry to hear you are having such problems with the side effects of your chemotherapy, I know how difficult it can be to get yourself mentally ready for another cycle of the same in these circumstances.

If your scan after three cycles shows that the chemo is working then perhaps there is something you could discuss with your oncologist that could ease the side effects somewhat without compromising the treatment. There is some research that looks at the effectiveness of Doxorubicin + Ifosfamide vs. Doxorubicin alone. The results are not clear cut but I will quote here one of the research papers on this topic.

The study I am quoting below is here: http://www.ncbi.nlm.nih.gov/pubmed/19880438

“Ifosfamide is the third agent having demonstrated efficacy on metastatic soft tissue sarcoma. In our study, ifosfamide did not favorably influence the outcome of metastatic LMS and the combination could be associated with worsened outcome, probably due to increased toxicity. These findings were also consistent with published randomized trials investigating the benefit of the addition of ifosfamide for the
treatment of all histological subtypes of metastatic soft tissue sarcomas [8, 17–19]. Despite this evidence, combination chemotherapy remains widely administered. In our study, only 42 patients (36%) received mono-chemotherapy. Monotherapy was associated with better dose intensity in doxorubicin (65
versus 52 mg/m2/3 weeks, P = 0.003). We have known since the 1970s that response rate depends on the scheduled dose [20]. Combination chemotherapy regimens are associated with
a greater risk of decrease in dose intensity.”

So if you have a positive result to the scan (and I sincerely hope you do) you could discuss with your oncologist dropping the ifosfamide and taking only the doxorubicin. Also, there is another form of doxorubicin called liposomal doxorubicin which has a lower level of side effects than conventional doxorubicin – that could also be worth looking at and discussing with him.

Very best wishes,
Paul.

[PaulW]

Do they think your cancer may be a Gastrointestinal Stromal Tumor (GIST) then rather than Leiomyosarcoma?

In my experience c-kit mutation status is not something that plays a major role in LMS treatment selection. LMS is generally more responsive to chemo than GIST but LMS does not normally respond to imatinib.

For LMS the initial systemic treatment options are usually considered to be doxorubicin or a combination of gemcitabine & docetaxal. Trabectedin is a very good option once those two first line choices have been exhausted.

Paul.

[PaulW]

I’m very sorry to hear this news Surinder.

Hang on in there. There are several options for systemic treatment that you could try that may give you prolonged respite and enable you to enjoy a good quality of life.

Paul.

[PaulW]

Hi Surinder,

I wish you the very best with your surgery on the 14th. I hope you make a speedy recovery from the operation.

Very best wishes,
Paul.

[PaulW]

Hi Krysha,

I’m sorry to hear about your cancers but I agree it’s very good news that you have found these early through SIGNIFY. It’s great that this trial is taking place. Together with the work that people like Pan are doing in creating this forum and in researching the condition there is reason to hope that the management of LFS will improve in the future.

I wish you all the best for your forthcoming surgery and for a speedy and complete recovery.

Paul.

[PaulW]

Interesting thread.

Pan, I was really struck by your comment:

[quote=”pan” post=294]The other thing to note is that – as I understand it – most LFS patients do not have cancers where both copies of the TP53 gene are deleted or mutated. This is somewhat surprising, but it’s most likely that the mutated gene has some ‘gain of function’ that provides the tumours with survival advantages.
[/quote]

I’d be really interested in any links to research papers that have more information on this if you have any?

Thanks,
Paul.

[PaulW]

Hi Sunnysarcoma,

I’m the person Pan refers to in his post above. Like you I have LFS and LMS. I was very sorry to read your post.

I’m 45 years old and from the UK. I was diagnosed with LMS in April 2011. The cancer was very advanced when I was diagnosed, I had tumours in my abdomen and several tumours in my liver. You can read more about my experiences with LMS on my blog: http://alotbs.blogspot.co.uk/, however I will briefly list some of the key things I’ve learnt about LMS.

1) It is very important to be treated by a doctor who is an expert in sarcoma – I think from you post you already know this but it is worth stressing as it has been shown to be an important factor in how well sarcoma patients do.

2) There is generally good international agreement on how sarcoma should be treated. Surgery to remove the cancer with wide margins is the ‘gold standard’ but if the cancer has already spread or is inoperable systemic treatment with chemotherapy is generally recommended.

3) Radiotherapy may not be such a good option for people with Li-Fraumeni, not just because of the risk of further cancers but also be it may not work so well when the cells being targeted do not have functioning TP53 genes.

4) Some chemotherapy agents work better or worse depending on whether the tumour cells have a functioning TP53 gene or not. The extent of evidence about this varies greatly from one chemotherapy agent to another. Personally I like to try and find out what is known about the interaction between any given drug and TP53 before I start the treatment. I do this by searching research articles (www.pubmed.org is a good place to start) and also by getting my oncologist to ask the drug manufacturer.

5) There is an e-mail based forum for people with LMS. There is a lot of good information posted on there though it can be difficult reading emotionally. I think there are some patients from Australia on that list. Go to this link to subscribe to it:
http://listserv.acor.org/scripts/wa-ACOR.exe?A0=l-m-sarcoma

6) Don’t expect your oncologist to know anything specific about Li-Fraumeni – you may be lucky but I think for my oncologist treating me has been the first time he has had a patient that is known to have this syndrome.

7) I’ve had three types of chemo so far – first Gem/Tax, then Doxorubicin and most recently Trabectedin. The Gem/Tax and Trabectedin both gave me some benefit (shrinkage and then a period of stability), the Doxorubicin didn’t work for me.

I’d be very happy to answer any specific questions you have – just reply and I’ll do my best to respond.

My very best regards to you,
Paul.

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