My story

[sunny]

I would like to share my story what happened.I was born in small town in North part of India(Jammu and Kashmir)
During my early childhood I began to notice my mother was always sad because she lost her mother at about the same age I lost my mother (about 17 year of age)I am now 42.In 1983 my Aunty(mother’s sister) was diagnosed with leiomyosarcoma of uterus and she passed away same year.After a year or so my uncle (mother’s brother) was diagnosed with lung cancer and he also died at age under 40.Having witnessed all that I had a strong feeling that there is something wrong here.My mother had recurrent kidney stones and she had multiple major surgeries.In 1984 my mother was diagnosed with infiltrating duct cell carcinoma and she passed away with multiple organs involvement and renal failure in December 1990.
In early 2010 my elder sister(age 39) became sick and was diagnosed in may 2010 with stage 4 adenocarcinoma colon with liver and lung metastasis and after palliative surgery and chemotherapy she passed away in Feb., this year in India.
I migrated to Australia in 2001.My health remained fairly good before 2007. I was diagnosed with active tuberculosis of lungs in march 2007 and cured after taking medication for about a year.
In early 2010 I was continuously feeling dull pain in my tummy saw my GP had tests for h-pylori which came negative.I requested my GP to guide me after having a feeling that I have a cancer or likely get soon.Due to lack of knowledge I was not getting anywhere but constant worrying about when and where I will get cancer.
In May 2010 I had internal bleeding in my stomach and felt fainted couple of time went to emergency straight and they admitted me straight away.Had endoscopy, my gastroenterologist said it is a ulcer bleeding and he mistook it as benign and given the family history of cancers decided to repeat endoscopy in a years time.I continue to have acid reflux and was given nexium for that.After second endoscopy in April 2011 my gastroenterologist said it look suspicious and sent me for endoscopic ultrasound in royal north shore hospital which confirmed it likely to be low grade leiomyosarcoma. I had partial gastrectomy in July 2011 and surgeon was confident that he removed all cancer which was a great relief for me which did not last long as I had vit B12,vit D deficiency and bowel obstruction.
During all these months I was also seeing my geneticist and he was trying to work out what condition it might be.After I told him the name of the tumor he was pretty sure it is rare condition of Li-fraumeni syndrome.
After my surgery I started seeing medical oncologist who unfortunately was not expert in sarcomas and he believed it will hopefully not comeback and I was cured.After watching my sister dying in Feb., this year with liver failure,I saw my GP and Gastroenterologist and told that I might have recurrence as I had big lump on my scar on tummy and I was referred to my previous surgeon to fix post operative Hernia which he repaired in June 2013 and he ruled out any recurrence.
After meeting with my geneticist in August this year I was enrolled in study through Peter Maccallum cancer center in Melbourne and they performed full body MRI and found cancer in my liver which was confirmed by CT scan and image guided biopsy in early November this year.Unfortunately my previous oncologist admitted that he is not expert in this rare cancer and said it is inoperable and he referred me to new oncologist and the sarcoma team which is still working out best way forward but the process is painfully slow.

[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.

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