Sarcoma UK Connect: Summer 2015

[email protected]

sarcoma.org.uk

Summer 20 15The bone & soft tissue cancer charity

Meet ‘The Prof’Ian Judson on early diagnosis and improving the landscape for everyone affected by sarcoma

Two booklets launchedNew information on bone sarcoma and retroperitoneal sarcoma3 7 14Personal experience

“Achievement is only limited to your imagination.” Read Stan’s osteosarcoma story

Talking ResearchWe’ve hit the million pound milestone!

Two of our funded researchers tell us why your donations are so important.

Connect • Sarcoma UK • Summer 20 15 sarcoma.org.uk

From the Chief Executive

2

This issue reports on exciting developments into sarcoma

research; two new booklets on soft tissue and bone sarcoma; and our new website that is now live!

Thanks to your donations and generosity, we have now awarded over £1 million in scientific and medical grants to better understand these rare cancers. Our 2015 research investment is £350,000, our largest research call to date. We are so grateful for all of your hard work, effort, and time spent fundraising – without you none of this would be possible.

We were delighted that 130 of you attended our event, Talking Research in Manchester. We heard from our ground-breaking sarcoma researchers, as well as inspiring speakers such as our trustee, Dave Thompson, and Patron, Richard Whitehead. Thank you to all of you – and the wider sarcoma community for a hugely insightful day (page 8).

We have seen an increase in the number of patients contacting us for information on sarcoma subtypes. We have developed two new booklets on bone sarcoma and retroperitoneal sarcoma. Order your guide to help you understand your diagnosis and treatment (page 14).

Log on to our new website sarcoma.org.uk – the first port of call for anyone affected by sarcoma. I hope you enjoy the new look and feel.

As support from the sarcoma community grows, we are committed to pushing boundaries to help you get the right information, at the right time.

Thank you for all of your continued support,

Sarcoma UK is the only charity in the UK focusing on all types of sarcoma Our mission is to increase knowledge and awareness of sarcoma through ground-breaking programmes that inspire involvement and transform the landscape for everyone affected by sarcoma

We initiate change to raise sarcoma awareness and improve standards of treatment and care

We seek answers through research

We provide support & information for the sarcoma community

Our impactAwareness • Connect (Sarcoma UK’s publication) is sent out three times a

year and distributed to a database of 6,000

• Sarcoma Voices is our active sarcoma community – see how you can help and “Speak out!” sarcoma.org.uk/voices

Research • Sarcoma UK has awarded £1 million into scientific and medical

research grants

• Grants are awarded by our Research Advisory Committee to leading scientists, researchers and clinicians in centres of excellence around the UK

Support & information• We offer support to patients and carers online sarcoma.org.uk/

help. Subscribers chat to each other via email and gain valuable support from other patients in the same situation

• We provide support to 13 local sarcoma groups around the UK, and help new groups set up

• We are active on Facebook Facebook.com/uk.sarcoma and Twitter @Sarcoma_UK where our sarcoma community communicates with each other in an online social environment

• Our patient information is rated highly amongst patients and healthcare professionals. 50,000 About Sarcoma UK leaflets are sent out to individuals and hospitals each year

• We have Personal Guides and booklets such as Understanding sarcoma: a new patient’s guide to help you through your diagnosis

What is sarcoma?Sarcomas are rare cancers that develop in the muscle, bone, nerves, cartilage, tendons, blood vessels and the fatty and fibrous tissues.Sarcomas fall into three main types:

1. Soft tissue sarcoma2. Bone sarcoma3. Gastrointestinal stromal tumours (GIST)

• There are around 100 different sub-types of sarcoma • About 3,800 new cases of sarcoma are diagnosed each year in the

UK which makes up approximately 1% of all cancer diagnoses:– 3,330 people are diagnosed with a soft tissue sarcoma

(including GIST)– 500 people are diagnosed with a bone sarcoma

• 10 people every day are diagnosed with sarcoma in the UK

• Sarcomas make up 15% of all childhood cancers (0-14 years)

• Sarcomas make up 11% of all cancer diagnoses in teenagers and young people (15-24 years)

• In general, patients with a bone or soft tissue diagnosis tend to be younger than the majority of cancer patients

Lindsey Bennister Chief Executive

• In Scotland, just under 300 new cases of sarcoma are diagnosed each year

• In Northern Ireland, 100 new cases of sarcoma are diagnosed each year

Statistics from the West Midlands Knowledge & Intelligence Team (Public Health England)

The most common sarcoma sub-types are:Soft tissue sarcomas• Leiomyosarcoma• Fibroblasticsarcoma• Liposarcoma• Gastrointestinalstromaltumour(GIST)• Kaposi’ssarcoma(KS)• Angiosarcoma• Malignantperipheralnervesheathtumour(MPNST)• Synovialsarcoma• Rhabdomyosarcoma

Bone sarcomas• Chondrosarcoma• Osteosarcoma• Ewing’ssarcoma• Chordoma

sarcoma.org.uk Connect • Sarcoma UK • Summer 20 15

“Achievement is only limited by your imagination.”When entrepreneur Stan Bembenek was diagnosed with osteosarcoma in 1986 and had his leg amputated,

doctors warned him he may never walk again. Almost 30 years later, he has made an inspiring recovery –

with a formidable golf handicap of 28 among a long list of achievements – something he ascribes to his

positive outlook on life.

It all started back in 1986 when I ran upstairs at work and slipped over. I thought I had pulled a

muscle but the injury seemed to linger and it was still there three months later. I went to see my GP, who was also a trained physio, and he said that I didn’t have a sprain as I had thought. I had a tumour, around the size of an egg. I was diagnosed with osteosarcoma, a rare form of bone cancer. I had never heard of sarcoma before.

I was taken into hospital to have a hip replacement to remove the tumour – I was 37 at the time. Swelling in my leg got really bad, and it came to light that when I had the operation there must have been some tumour left behind. It was confirmed that the cancer was back and I needed specialist care so I was referred to the Royal Marsden Hospital in London.

I cannot praise the Royal Marsden Hospital enough – I got an X-ray and the results within eight minutes of entering the building for my appointment. When specialists told me they were 95% certain that I needed my leg amputating, I said just do it. The doctors were surprised by my reaction and thought I hadn’t understood. I wasn’t depressed, I never have been – even though there have been some low moments – I actually felt relieved. I think my wife found it more difficult than I did, and my children. I consoled my wife by saying that it was happening to me and I promised it would not affect her life in any way.

I had a seven-hour operation to remove my hindquarter. For a few days I recovered in bed. When I ventured to

the toilet on my crutches, I felt light and free. The recovery period was difficult, and it took around six months for my brain to catch up that my limb had been removed; I would often get up out of bed and fall to the floor. At that time, my daughter used to lie on my bed where my leg used to be, which was a huge comfort and really sweet.

I had a prosthetic leg fitted at Roehampton Hospital. I named it Fred, but he is now relegated to my loft! I found moving around easier without wearing it and just using my crutches.

I coped by throwing myself into work, I was an entrepreneur and set up a total of eight different businesses, all within the telecommunications industry.

In May 1991, the doctors confirmed the tumour had returned. I had to have another operation to remove the rest of my pelvic bone and

Stan and friend

Stan takes a swing

My story

3

two inches of my spine. This was one of my darkest periods, but I still came fighting back.

I am now healthy and only go back for check-ups once a year. I have even taken up golf since becoming an amputee. Before the cancer, I was a keen footballer, but golf is my sport of choice now. I even strengthened my leg by going disco dancing; I can now dance on my leg for 20 minutes. My favourite song is Twist and Shout.

I am also Chief Executive Officer for the Disabled Golf Association, which I am developing and working closely with disabilities charities to get more people involved. Golf is a great sport for rehabilitation as it weighs up your own personal handicaps and means that you can play against anyone! We have a mixture of all ability golfers and organise fun and more serious games to suit anyone. Everyone is welcome, and we are proud to be the only golfing organisation to accept absolutely any disability.

I thrive by keeping busy – I volunteer at the Royal Marsden Hospital and enjoy supporting new amputees and their families. I also give inspirational talks and I am happy to offer advice to anyone who needs it. I have always been a very positive person and my amputation has only solidified that fact. I do not consider myself to be disabled at all – even though I am also deaf! To any new amputee or anyone waiting to have the op, I say: “Achievement is only limited by your imagination”. Just get out there and do what you want.

disabledgolfsociety.com


Blogging, Balls, and Bald Birds!Thank you to all of our supporters who always go the extra mile!

“Positivity!” Hope Stringer I have been blogging since my sarcoma diagnosis earlier this year, and have gained followers from all over the world.MysisterNancyandcousinBethany organised separate events – a gig night and a sit down ball – raising over £18,000!

Read Hope’s blog: https:// medium.com/@hopejoystringer

Running Reading Half MarathonA big thank you to all the Reading Half Marathon runners, and those who turned out to cheer them on. Together you raised over £6,300, making a real difference to lives across the UK.

Run like the wind Jeff White Despite what was meant to be terrible weather it was dry all route and was even sunny. Though running in to a headwind, I completed the Edinburgh Marathon in 4:02. A bit sore, but not too bad! Hilary and I raised over £3,600.

4

Fundraising

Maria Johnson To remember our colleague, Dave Culshaw, a group of us from Fujitsu trained hard and took on the gruelling Tough Guy obstacle course. Despite freezing temperatures and a lot of mud, we all finished and together raised over £8,400.

Culshaw’s Crusaders

Combining forces Sian Westlake and Clare Thomas We’ve been organising events, taking on challenges and inspiring our friends and family to raise money in memory of Adam and Amy. Together we’ve now raised over £17,000.

James Mulligan After hearing about my close friend, Tom’s sarcoma diagnosis, a group of us (known as ‘The Bird’), decided to shave our heads to show our support. After receiving incredible support from our community, we’ve gone on to run half marathons and complete skydives, raising over £16,000.

The Bird goes bald

Hope Stringer

James Mulligan

sarcoma.org.uk Connect • Sarcoma UK • Summer 20 15 5

Fundraising

Emma and Lisa

The Robson Family and Sarcoma UK’s Director of Fundraising , Vicki Smith

We’d love to support you whatever your fundraising activity.

020 7250 8271 [email protected]

Wendy Arnold Who wouldn’t want to run 5K whilst being doused in colour by spectators at the Manchester Colour Run? Together with friends and family we raised over £400, and had lots of colourful fun!

Raining rainbows!

Jessica’s Sarcoma Awareness The Robson Family What a fantastic night Jessica’s Fairytale and Superhero Ball was in Gosforth. We presented a cheque for £7,500 to Sarcoma UK in their efforts to find a cure. Thank you all from the bottom of our hearts.

facebook.com/Jessicassarcomaawareness

Running Retro! Emma Bytheway We supported our friend Lisa, by running as her favourite computer game characters: Mario and Luigi. Our team raised over £3,000.

Feel the burn! Justin Gomersall We took on the momentous Land’s End to John O’Groats cycling challenge, and conquered it! It was simply epic, and as a team we raised over £5,000.

Take a look online:

sarcoma.org.uk/fundraisers-

gallery

Connect • Sarcoma UK • Summer 20 15 sarcoma.org.uk6

Team Sarcoma – take a bow! Thank you to all 24 of you who completed the London Marathon 2015 and raised over £80,000.

2015 was another hugely successful year for our inspiring Team Sarcoma who

all completed the 26.2 miles London Marathon course. And also for all of you who sponsored our team and lent them your moral support. Over £80,000 has been raised this year, helping us to transform the landscape for everyone affected by sarcoma. Thank you to all the team, and their supporters, as well as everyone who attended our two cheering stations, giving much needed encouragement to our runners.

The atmosphere was electric and we managed to spot all of our runners amongst the 36,000 who took to the start line for the world’s largest fundraising event.

Runners, friends, family and Sarcoma UK representatives all had a chance to come together and celebrate at our Runners After-Party near Westminster Abbey. A truly momentous day and we’re already planning to do it all again next year!

Special thanks to Jan Cornell & friends, and our volunteer masseuses Robyn and Matt

If you have been as inspired as we have, and want to join Team Sarcoma in 2016, applications are now open.

sarcoma.org.uk/running

020 7250 8271

“We both agree that the sports massage was a lovely

touch, and helped us both with our recovery!”

Daniel Skelt & Pat Donnelly

“It was truly a pleasure to run for Sarcoma UK.”

Seigo Robinson

“It was a great boost to go through both your cheering points; me

and my family had an amazing experience.”

David Lee

Fundraising


Meet ‘The Prof’Professor Ian Judson is Head of the Sarcoma Unit and Professor of Cancer Pharmacology at the Royal Marsden Hospital; Chair of Sarcoma UK’s Research Advisory Group; and President of the British Sarcoma Group.

How can we improve the landscape for sarcoma? There’s one thing that would make the biggest difference – earlier diagnosis. We need to raise the level of education about sarcoma among GPs. For that reason I was very pleased to see that Sarcoma UK is supporting a new BMJ learning module to raise awareness of sarcoma amongst GPs and medical students. We need to get GPs more alert to the fact that lumps can be malignant, especially in young people. (I don’t want to be unduly critical, though – after all a GP only sees on average one patient with sarcoma in his or her lifetime.) At the same time we need to educate the general public to get bumps and lumps checked out.

What’s your advice to newly diagnosed patients? To stay positive and to be active. There’s good evidence to suggest that patients who stay physically fit do better – they tolerate surgery better, and in general recover more quickly. We’re not certain yet why – it may well be related to the immune system. I also advise patients to make sure they avail themselves of the information that Sarcoma UK and other support groups can provide. You hear all the time about patients dealing with feelings of isolation – getting in touch with other patients with the disease can be very helpful.

What are the most satisfying, and frustrating, parts of your work? The most satisfying thing is working in a field where I can establish long-term relationships with my patients. I’m so lucky to be surrounded by a brilliant team of

Professor Ian Judson

people I like and respect. The most frustrating aspect can be around issues of funding drugs. I was personally very disappointed to see funding withdrawn by the Cancer Drugs Fund for certain drugs on the

grounds of rarity when previously other drugs for similarly

rare conditions had been approved.

How does treatment of sarcoma patients today compare with 25 years

ago, when you were

appointed Senior Lecturer

at the Royal Marsden Hospital?

When I first started treating patients with sarcoma, apart from patients with very sensitive tumours like Ewing’s sarcoma, it was very difficult – we had limited resources and effectively only two drugs at our disposal. But in the late 1990s and in the first few months of 2000 we started to hear about successful trials of new, targeted agents. I remember treating my first patient

with one of these new drugs – Imatinib – in

September 2000, and it was just

a revelation, I had never seen anything like it! We haven’t had a

breakthrough of quite the

same level since then, although

the median survival of patients with advanced sarcoma has improved by about 6 months. Of course we have many more drugs

at our disposal now and instead of treating all sarcomas the same we increasingly adapt the treatment to the histological sub-type.

What have been the most striking developments in the last decade? The process of concentrating care in a small number of specialist centres has had a significant impact. We are seeing more consistent treatment, more awareness, earlier referral (although I think we can do a lot more) and better surgery. In short, far fewer patients are having inappropriate operations performed by inexperienced surgeons. Another big change in the last 10 years is the fact we are treating older and older patients – this is partly to do with changing demographics and partly because of the emergence of some effective drugs that we can safely administer to patients over the age of 70.

How important are clinical trials to improving survival? Long term we only make advances through clinical trials - that includes new surgical trials and radiotherapy trials, it’s not just all about a new wonder drug. Just about everyone involved in the field of sarcoma has a research focus.

What changes can we look forward to in the treatment of sarcoma? I am sure that things will continue to improve as we find more diseases that can be treated with specific therapies. It’s possible that immunotherapy can provide the next big breakthrough. If we are able to establish that modulating the immune system can be beneficial to sarcomas then it will offer hope to patients with diseases we can’t currently treat successfully. So yes, we can be very optimistic.

“The most

satisfying thing is working in a field

where I can establish long-term relationships with

my patients. ”

ResearchIn the hot seat


Research

Talking Researchhttp://dizzytix.com

It’s here again. Sarcoma Awareness Week.

Sarcoma UK hosted an event for people affected by sarcoma, whether it be in a patient capacity or a medical capacity and the room was full. The theme this year was Research.

The charity sourced excellent speakers who had received funding towards their area of research and they shared their stories. Here’s a few titbits.

One researcher talked about angisorcoma which is cancer of the blood vessels and can be found commonly in the scalp.

Common, you say?

NowwhenIsay‘commonly’it’snotcommonatall.Approximately3,000people a year are diagnosed with sarcoma and of those people between 10-15 will have this sub type. The researcher is working with a veterinary oncologist to look at treating this sarcoma in dogs so he can find a link and help to treat humans with the same type. Wow. Just wow! This talk showed that no matter how rare your cancer is, there’s always someone fighting your corner.

Cancer in childrenAnother researcher focused his work on trying to understand the right treatment for children with Ewing’s sarcoma. Whilst it was upsetting to think of children going through this, it was comforting to hear the progress being made.

Desired effect of chemo = desired effect of alcoholOne cancer researcher explained why you feel ill after having chemotherapy (chemo) in a way that I perfectly understood.

The dosage of chemo drugs required for the good of your health (to attack the tumours) unfortunately is the same dose that causes the awful after effects.

The amount of alcohol required to have a good time and be merry is, unfortunately, the same amount which will give you a hangover…

The special guest of the day was the lovely Richard Whitehead – Paralympic gold medallist and Patron of Sarcoma UK.

I met Richard last year at another Sarcoma UK event but didn’t get to hear his story at the time. On Saturday he gave a speech to us all and it was so inspirational. As a double leg amputee he has faced many challenges through his life. Overcoming these challenges, Richard became a marathon runner and for the 2012 Olympics he took part in the 200m – his first time as a sprinter. And he won!

To have such a wonderful person support the charity is just so lovely. He understands what we are going through and has nothing but words of encouragement and support.

A motivating and fantastic end to a fantastic day, so a huge thank you to both Sarcoma UK and Richard for putting the day together.

I would also like to thank all supporters of Sarcoma UK and I can personally vouch that it’s a worthy cause.

Me and Richard

Talking ResearchTo kick-start Sarcoma Awareness Week in June, we invited members of the sarcoma community – patients, carers, friends, health professionals, and researchers – to join us in Manchester for our Talking Research event. It was a day to mix and mingle with Sarcoma UK-funded researchers and scientists. Two of the attendees, Emma and Roger share their experiences in their personal blogs.


Research

Talking Researchhttp://rogerwilson.me.uk

What a great day!Sarah McDonald (SUK’s Director of Research & Information) had done a tremendous job in selecting and briefing top scientists and clinicians and the audience of patients and carers (about 130) engaged and learned. Everyone spoke at a sensible level of understanding, scientific terms were explained, and concepts which are difficult to get hold of used explanatory slides. As a scientific meeting with a patient focus it was exemplary. I’ve been to a few in my time but none to beat this.

Dr Gareth Veal from Newcastle gave the best explanation of cancer pharmacology and its role in helping get the best dose of drug suitable for an individual patient. A translational study is running alongside the EuroEwing 2012 clinical trial so the Newcastle unit is turning its research understanding into clinical value and, hopefully, increased benefit for each patient in the study.

Dr Robin Young is a medical oncologist in Sheffield with an academic research role as well. He was working on angiosarcoma then and his work has taken an unusual direction, looking at the commonalities between angiosarcoma in humans and dogs. The tumour similarities are apparently strong and the study is looking at how development of new treatments might be trialled in dogs and those results could benefit human patients too.

I had the privilege of giving a short lecture in memory of Paul Robson, an active advocate locally in the East Midlands and latterly nationally too, working with Sarcoma UK as a Sarcoma Voice. My personal reflections about involvement in research as a patient go back more than 12 years, starting when times were different. Sarah Welby, the sarcoma research nurse from the Christie Hospital, described her role and how they worked to ensure that patients in clinical studies received the best care.

After lunch Professor Ted Hupp from Edinburgh talked about the p53 gene and his research into how this gene, which seems ubiquitous in cancer, probably has a key role to play in the treatment of sarcomas. A study with an experimental drug targeting p53 showed benefit for a cohort of liposarcoma patients. Sadly the company owning the agent decided against developing it further – a story we have heard rather too often with sarcoma drugs. It is clear that the pharmaceutical industry business model is far too focussed on common cancers.

Dr Paul Huang from the Institute of Cancer Research in London looked at drug resistance to tyrosine kinase inhibitors, with a focus on pazopanib (Votrient). He started with a diagram of a cancer cell, and then said it was a simplified version, to everyone’s gasp of horror. He described how pathways could be defined and using a London Underground metaphor explained how cancer was clever, and found new routes to its objective when its first route was blocked. Understanding this in sarcoma patients will help identify those more suited to targeted therapy and also point to other therapies if applicable. This research is opening up new understanding of how cells work and could have ramifications much more widely for development of tyrosine kinase inhibition.

The last speaker was Dr Nick Gough, a palliative care specialist who undertook one of the first studies funded by Sarcoma UK. It is an extensive view of quality of life in advanced sarcoma with both qualitative and quantitative elements to it. It generated a lot of interesting data and Nick provided an updated view (there have been posters and previous presentations at various conferences). The final paper has been prepared and will hopefully be in print later in the year or early 2016.

The morning session was chaired by Dr Mike Leahy, medical oncologist at the Christie, and the afternoon session by Professor Lee Jeys, orthopaedic surgeon from Birmingham and Chair of the National Clinical Research Institute Clinical Studies Group for Sarcoma. Both have a relaxed and easy approach which patients readily appreciate and which the speakers could echo. There was real appreciation of the accessibility of the talks.

To close the day Richard Whitehead MBE, Sarcoma UK’s patron, Paralympic gold medal winner and the world’s leading marathon runner on prosthetics, talked about his 40-day 40-marathons run from John O’Groats to Lands End. A truly inspiring man, a great story and a real high with which to finish a great day.

Roger Wilson CBErogerwilson.me.uk

Full house

View presentations from

the day on our website: sarcoma.org.uk/

TalkingResearch2015


Research

sarcoma.org.uk

Sarcoma UK’s research funding hits £1 million milestone Thanks to your donations, this year brings our total investment into high-quality sarcoma research to £1 million. To mark the occasion, we took a trip to visit the Institute of Cancer Research to interview two of our funded researchers, Paul Huang and Barbara Tanos.

Paul Huang Please give us an overview of your lab’s research focus. My laboratory is interested in understanding how the complex communication wiring within normal cells malfunctions which can lead to the development of cancer cells.

We have developed biological “tools” to listen in on these communication highways within cells and figure out what makes these cells change.

Our aim is to develop therapies to correct these abnormalities in cells, which will help to ultimately overcome the disease.

How did you get into sarcoma research? Sarcoma is such a complex disease with so many different subtypes and underlying biology and genetics – for which we know so little about, in part because of its complexity and its rare nature.

As a scientist, the complexity of sarcoma really offers an opportunity to use the tools that we have developed in the lab to unravel the unknown mechanisms in the development of sarcoma disease and fill a much needed knowledge gap.

The Institute of Cancer Research has a close working partnership with the Royal Marsden Hospital and we work closely with the clinical

staff at the Sarcoma Unit, led by

Paul Huang

Professor Ian Judson (in the hot seat, page 3), to ensure that our research questions are relevant to patients.

This integrated relationship allows us to address sarcoma research questions in a translational manner along the “bedside to bench and then back to bedside again”

pathway to benefit patients.

What made you look at pazopanib resistance? Pazopanib is a drug that is approved for the treatment of soft tissue sarcomas with the exception of liposarcomas.

Recent analysis of clinical trial data has shown that a significant number of sarcoma patients (30%) have lived for more than 18 months while being treated on this drug.

Importantly, not all patients respond to this drug and there are some sarcoma subtypes that appear to respond better to this drug. There are also patients who initially respond to the drug and subsequently acquire resistance and we do not understand why this is the case.

Because we do not understand why some patients respond more than others, it is challenging to

“Notall

patients respond to pazopanib and

there are some sarcoma subtypes that appear to respond better to

this drug. ”


Research

11

predict which patients to give the drug to. Perhaps pazopanib as a treatment alone is insufficient but if we administer it with another drug, we could start overcoming the resistance.

How did you arrive at your research question? Given the clinical challenge, we decided to address a number of pertinent questions.Are there specific subtypes of sarcoma that respond better to pazopanib? If so, what are the mechanisms of this different sensitivity to pazopanib? Can our understanding of drug sensitivity help use design strategies to treat patients who are resistant or develop resistance to pazopanib?

What has your research shown so far? Initially, we screened a panel of sarcoma cells from different subtypes in the laboratory to identify which cells are particularly sensitive to pazopanib treatment.

We then designed experiments to mimic the process of acquiring resistance in patients in the laboratory by subjecting the sensitive cells to long term treatment with pazopanib.

Constant treatment with the drug allows cancer cells to adapt and become resistant to the drug over the course of several months (much like in the clinic).

The original sensitive and newly created resistant cells were then subjected to molecular analysis to determine what makes these specific sarcoma subtypes so special in their ability to respond to pazopanib and what changes occur to make them acquire resistance.

Using this approach, we identified that a particular rare form of cancer that affects children, known as malignant rhabdoid tumours, are particularly susceptible to pazopanib treatment. This is one of the most aggressive forms

of cancer in children and our research would suggest that pazopanib may be a good therapeutic option for these children.

In addition, when we analysed the cells that have acquired resistance, we identified additional targets and drugs which when used in combination with pazopanib, will enable us to overcome pazopanib resistance.

We then went back to our original panel of sarcoma cell lines from

different subtypes to show that these drug combinations work

much better than pazopanib alone in killing tumour cells.

This further highlights the need for research into rare cancers such as sarcomas because it may lead to

fundamental discoveries which can be extended to other

cancer types as well.

How could this benefit sarcoma patients? We are really excited to translate our laboratory findings into the clinical setting, working with our colleagues Professor Ian Judson and Dr Robin Jones in the Sarcoma Unit at the Royal Marsden Hospital.

It has enabled us to frame a clinical question that we can now test in the hospital setting through a clinical study. This study involves analysing the tumour biopsies from sarcoma patients to determine if the molecular markers of sensitivity are present and if so, whether these patients ultimately respond to pazopanib treatment. We are also looking for markers of resistance so we can identify patients who may respond to the drug combinations that we have identified.

Barbara Tanos How did you get into research? As a child, as far as I remember, I was always “running” experiments. I would mix things up, leave them

by the window and record how they changed over time.

I got my first microscope when I was 10, I looked at everything I could get my hands on to see what it looked like up close, took pond samples and was generally curious about the world.

What specifically made you get interested in sarcoma research? A close family member was affected by sarcoma, and this made me realise how little we know about sarcoma. In a way, I feel as if I owe it to him and to other patients to work to find a cure. I feel that it is the 21st century and we are still relying on radiation and chemotherapy. We need targeted therapies.

What made you look at the role of cilia in sarcoma? Cilia are structures found on all normal cells, their function is to sense the environment around the cell. Highly metastatic sarcomas lack cilia, which is puzzling. From my previous research, I wanted to understand why metastatic sarcoma cells lose their cilia, what impact this has on the cell cycle and how this affects proliferation and invasion.

How will your research benefit all sarcoma patients? Understanding how the basic biology of the cell and its routine working is altered in sarcoma will help us identify vulnerabilities in cancer cells and aid design of targeted therapies. Without a detailed understanding, cancer therapy is equivalent to taking a potion blindly and we can’t explain when it doesn’t work or has an unwanted effect. I want my research to change this and ensure sarcoma patients have effective therapies and a good understanding of how they work.

Will your research have a wider impact on all cancer patients? Yes, certainly. We’re looking at a phenomenon which alters how a cell senses its environment and

“This study

involves analysing the tumour biopsies from

patients to determine if the molecular markers of sensitivity are present and if so, whether

these patients ultimately respond to pazopanib

treatment.”


Following a diagnosis of sarcoma, many people want to make positive changes to their lives.

Taking steps to live a healthier lifestyle is often a major part of these changes.

For most people, a daily balanced diet includes:

• Lots of fruit and vegetables

• Plenty of starchy (carbohydrate) foods such as bread, rice, pasta and potatoes

• Some foods that are high in protein such as meat, poultry, fish, nuts, eggs and pulses (beans and lentils)

• Some milk and dairy foods such as cheese, yoghurts and cream

• Just a small amount of food high in fat, salt and sugar

Before making changes to your diet, it can help to talk to a dietitian, your GP or sarcoma specialist.

The role of a dietitian?Dietitians assess whether you need a special diet and can advise on the most appropriate nutritional support to help you before, during and after treatment for sarcoma. Some treatments for sarcoma can affect the way you eat. Some chemotherapy drugs affect the way food tastes and even make your mouth so sore it is difficult to eat in comfort. If you have had surgery for sarcoma you may have had part of your gastrointestinal tract (an organ system responsible for consuming and digesting food) removed, or part of the bowel or stomach, which will affect how you eat.

A dietitian will use their knowledge of sarcoma and its treatment to provide practical advice that will help you to eat and maintain your quality of life, including:

• Enriching your meals and drinks with extra calories and protein to help prevent weight loss or overcome a small appetite

• Adapting the texture of your foods when your mouth is sore or you have problems swallowing or chewing

• Nourishingdrinksorfoodsupplements for weight loss and swallowing problems

• Altering the flavour of your food to cope with taste changes

how this is altered in sarcoma. The research will be applicable to other cancer types with similar regulatory function.

How big is your team? Who are they? The team right now is three people; myself, my postdoc Dr Andrew Jenks, and Abdulaziz Almalki a medical student who has joined us for a year.

What’s the biggest breakthrough you’ve ever made? I hope my biggest breakthrough is yet to come! However, my paper on Genes and Development where I identified a group of proteins required for cilia formation, has been widely cited and has helped characterise a number of rare ciliopathies.

This has made me very proud because it has helped a number of families to link their disease to alterations in these genes. This is a step forward in trying to establish treatment, and eventually a cure.

I hope I can continue making great discoveries like this one.

What’s next for you research-wise? We hope to fully characterise the role of cilia in sarcoma.

Describe your average day at work? We tend to set up experiments early; then I have time to read journal papers, review results with Andrew and Abdulaziz, plan other experiments, talk to my collaborators and other scientists and keep on top of general work.

What do you do to relax, when you’re not in the lab? I like to go running. I also write poetry and paint. Recently, 10 months ago, my son was born, so I spend lots of time playing with him and taking care of him.

What would happen if your funding stopped? I would need to apply for more grants. It costs £140K a year to run my lab with three of us working, it’s a very challenging environment to work in.

For more insight into our current research projects visit sarcoma.org.uk/research

“This is a

step forward in trying to establish

treatment, and eventually a

cure.”

Barbara Tanos

We cannot fund this vital research without your donations.

Research


Support & information

13

Living well with sarcomaIn this new, regular feature for Connect, we will highlight helpful ways of living well with and beyond sarcoma.

Here we focus on healthy eating and the role of a dietitian in your care. In future editions we will cover topics

such as emotional and psychological support, exercise and information on prosthetics.

• Food safety advice to prevent avoidable infections

• Adjusting your intake of dietary fibre and fluids to promote normal bowel function

• Artificial feeding (nutritious liquid through a tube) when you are not able to eat enough

• Weight management and healthy eating when you are well

Further informationIf you feel you would benefit from dietary services, help is at hand! You can speak to your sarcoma clinical nursespecialist(CNS)oramemberofyour medical team and ask them to make a referral.

Alternatively, if you are not seeing your hospital

medical team regularly, it may be easier to speak to your GP, who can make a referral to a local service.

If you’d feel better speaking to a member of the Sarcoma UK team first, please call us.

0207 250 8271

[email protected]

Download our rehabilitation

factsheet: sarcoma.org.uk/

resources


Support & information

14

Euro Ewing’s: Can you help to influence research into bone cancer?

Ewing’s sarcoma can affect any bone in the body but they mostly occur in the pelvis, thigh and shin bone. The majority of Ewing’s sarcoma patients are in their teens and it is very rare indeed over the age of 30.

The Euro-Ewing’s Consortium (EEC) was recently launched as a pan-European programme to improve the lives of those with Ewing’s sarcoma. The group is made up of scientists and practising doctors and its members are seeking Ewing’s sarcoma patients and carers to join a network which will influence how the project moves forward – which is where you come in!

With rare diseases, it is necessary to recruit beyond national boundaries in order to find sufficient participants for research evidence to be reliable. It is intended that these trials will roll out in 17 countries and recruit a thousand participants in the coming five years.

Through online communication or perhaps face to face meetings, there will be the opportunity for you – patients and carers – to participate in a dialogue about how EEC should evolve. It is hoped that a European wide network can be created through Sarcoma Patients Euronet (SPAEN)andthatthiswillnotonlyinfluence the direction of research into Ewing’s sarcoma but also provide support to families.

Interested?

[email protected]

euroewing.eu

New booklets on bone sarcoma and retroperitoneal sarcomaOur range of booklets provide helpful information on different sarcoma types, treatment and support available to you from diagnosis, through to treatment, and beyond.

Order our booklets

sarcoma.org.uk/resources [email protected] 0207 250 8271

Understanding bone sarcoma focuses on the four main types of bone sarcoma: chondrosarcoma, osteosarcoma, Ewing’s sarcoma and chordoma.

Understanding retroperitoneal sarcoma focuses on soft tissue sarcomas that are in the retroperitoneum – a space deep inside the abdomen and pelvis, behind the abdominal cavity, where organs such as the major blood vessels, kidneys, pancreas and bladder are located.


News

News round-upFind out what’s been happening in the world of sarcoma and see how you can get involved!

sarcoma.org.ukOur brand new website is designed to further help you through your diagnosis and ensure that you have sarcoma support and information at your fingertips.

Members of the sarcoma community – patients, carers, supporters, health professionals, and researchers – have all been involved in the website design. Our users are at the heart of what we do, and we want to ensure that we reflect this by listening to your needs and providing you with up-to-date information. Whether you are a patient seeking advice, a supporter wanting to take up a challenge, or a researcher wanting to apply for funding, our new hub is now live – and it’s now mobile friendly too! Take a peek!

We would like to thank our friends at Agile Cooperative agile.coop and all of our users who gave up their time to be interviewed and assist with user testing on this project. We hope you enjoy the new look and feel and we welcome any feedback at [email protected] – or give us a call.

Quality Standard for Sarcoma An analysis of the NHS Quality Standard for Sarcoma from our Honorary President Roger Wilson is one feature on our redesigned website. As we reported in the last edition of Connect, the Quality Standard, published in January 2015, was built around six statements designed to drive measurable improvements in the provision of services for people with sarcoma. To get the inside track from Roger, who was part of the Committee that created the Quality Standard, visit: sarcoma.org.uk/about

Talk to others

We offer a range of support for anyone diagnosed with or concerned about sarcoma. Sometimes it’s good to share your experience with others. We have information about the many support groups around the country. There may be one close to you. If not, you can always join in the discussion in an online support community.

Recently, members of our online gynaecological (gynae) support group met up in person. This unique email support group allows women with Endometrial Stromal Sarcoma or Uterine

Leiomyosarcoma to talk together in an open, honest and supportive forum about things specific to gynae sarcomas. Members from as far as Scotland travelled down to London to meet others in similar situations.

Karen Delin, group member said: “It was an excellent opportunity to put faces to names, get to know each other better and share stories and experiences. The event was a positive experience and thank you to everyone who attended”.

Find support: sarcoma.org.uk/help

Board of TrusteesLesley AbrahamDr Jane BarrettKaren DelinProf Rob GrimerLeigh HibberdineIan HughesSharon ReidHelen StradlingDave ThompsonDr Jeff WhiteSam WhittamGlyn Wilmshurst

Honorary President Roger Wilson CBE

Medical/Scientific Advisor Prof Ian Judson

Patron Richard Whitehead MBE

Disclaimer: Please note that personal views and opinions expressed are not necessarily endorsed by Sarcoma UK. The material in this publication is provided for personal, non-commercial, educational and informational purposes only and does not constitute a recommendation or endorsement with respect to any company, medical professional or product. Sarcoma UK makes no representations and specifically disclaims all warranties, expressed, implied or statutory, regarding the accuracy, timeliness, completeness, merchantability or fitness for any particular purpose of any material contained in this or attached document/s. The information contained in Connect is not intended to replace advice or medical care from your doctor. NopartofthispublicationmaybereproducedinanywaywithoutpriorpermissionfromSarcomaUK.

Registered as a charity in England and Wales (1139869) and in Scotland (SC044260). A company limited by guarantee in England and Wales (7487432)

Editorial and production teamEditorial: Vicki Smith, Glyn Wilmshurt, Alice Snape Photos: David Baird & Ian RandallDesignandlayout:INQDesignLtd02077375775

sarcoma.org.uk

020 7250 8271

[email protected]

@Sarcoma_UK

uk.sarcoma

49-51 East Road, London N1 6AH

Donate! We rely solely on your voluntary donations to raise sarcoma awareness, fund research and provide support & information.

You can now donate online:

you could provide the necessary equipment for Reema to continue her vital sarcoma research. We fund high quality research projects looking into the causes and treatments of sarcoma. £50 could buy three Coplin Jars used every

day in the laboratories by researchers investigating cancer genetics.

With £50

you could prevent Harry from having to visit his GP three times before being referred to a sarcoma specialist. £20 could fund 10 golf balls to send to GPs as part of our Golf Ball Awareness Campaign, helping to improve GP awareness, and

resulting in earlier diagnosis of sarcoma.

With £20

you could help Tom find the support he desperately needs. We provide grants to sarcoma regional support groups, helping them provide sarcoma support, locally. Tom could meet other sarcoma patients and carers face to face in a relaxed, non-clinical environment.

With £100

you could banish Abdul’s feeling of isolation. With only 3,800 people newly diagnosed in the UK each year, some patients may never meet another person going through the same situation. £10 means Abdul can receive our newsletter,

Connect, three times a year for two years, bringing him closer to the sarcoma community and making him feel less alone.

With £10 you could enable sarcoma Clinical Nurse Specialist, Julie, to give every newly diagnosed patient a Personal Guide to Sarcoma. Newlydiagnosedpatientstellusthey often feel confused. £5 could supply a newly diagnosed patient with their own ‘PersonalGuide’–afolderpackedwithinformation, tailored to their needs.

With £5

How?By Text: Text SAUK00 £10 to 70070Minimum donations £1 and maximum donation is £10. Gift Aid can also be added.

By cheque: Make payable to Sarcoma UK and post to Sarcoma UK, 49-51 East Road, London, N1 6AH

By Bank: Lloyds TSBAccount name: Sarcoma UKSort Code: 30-97-62AccountNo:02884274Reference: Your name

sarcoma.org.uk/donate

Sarcoma UK Connect: Summer 2015

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