My story

[PanP]

Hi. One of the members of our forum also has LFS and leiomysarcoma and has a lot of experience of treatments. I’ve alerted him to your post and hopefully he’ll be along to offer advice. Has there been any progress with your new oncology team? Have they suggested any treatment options?

Pan

[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.

[sunny]

My new team is working hard and treatment they advised is liver resection as the biology of tumor gives them limited options as it does not respond well to chemo or radiation .one of tumor can be easily removed but the one near portal vein is very difficult.I have been told there is a risk of death with this operation.I need lots of prayers to survive this and hopefully I will survive this to tell the story.Is there anyone who have survived portal vein grafting liver resection ,please share your experience?

Surinder

[sunny]

Thank you Paul for your valuable advice.After reviewing pathology of the tumor my oncologist and sarcoma team is confident that this particular type of leiomyosarcoma does not respond to chemo and if it respond well they think with Li-fraumani patients ,chemo can induce some sort of cancers in future.My best bet is to go liver resection with portal vein grafting and this is very risky operation but I have no option but to undergo this surgery.If I will survive this operation on 14th ,I will post my sarcoma team’s next step.

Surinder

[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.

[sunny]

Hi Paul,

Thank you for your Best Wishes and I Wish the same for your treatment.I would like to share another forum with other members,some might know about it others might find it informative.
http//csn.cancer.org/node/194105 .

Surinder

[PanP]

Best of luck, Surinder. You know that you have all our best wishes.

Pan

[SKH]

The very best of luck Surinder

[sunny]

My surgery has been canceled, as latest scan shows several spots in the liver.Flying back to Sydney from Brisbane.Need to fight on but who knows how long for?

Surinder

[pennysophia]

So sorry to hear that Surinder. I will be in touch by email.

Pan

[SKH]

That’s incredibly tough Surinder. I’m so sorry to hear this.
Thinking of you

[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.

[sunny]

Thank you Paul.Systemic therapy is the only hope and depend upon the response because the gastric tumour was CD117(c-kit) negative.But I hope something might work.

Surinder

[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.

[sunny]

(When I reviewed this case I specifically did so to make sure that it was not a GIST and I am very confident that this is a true leiomyosarcoma based on both the morphology and also the immunohistochemical profile.

The tumour demonstrated negative staining for cKIT and DOG1 and positive staining for desmin which excludes the possibility of GIST and given the morphology confirms the diagnosis of leiomyosarcoma.)
Above is the part of email from my pathologist.
I have no idea what they gonna give me.I will keep you posted once I find out.

Surinder

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