Connect info@sarcoma.org.uk sarcoma.org.uk
The bone & soft tissue cancer charity
Aut um n 20 14
Power to the Pedal 500km Cycle, 5 Days, 1 Mountain.
The Maritime Support Unit take up their momentous challenge.
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Carrying the baton Gordon Cowan proudly represents Scotland and the sarcoma community!
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You did it!
You made everybody ‘On the Ball’ for sarcoma. See the impact you’ve created!
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Head & neck sarcoma
With over 100 subtypes of sarcoma, Public Health England gives an insight into this specific type of sarcoma
From the Chief Executive
Sarcoma UK’s key messages Sarcoma UK is the only charity in the UK focusing on all types of sarcoma. Our mission To increase knowledge and awareness of sarcoma through groundbreaking programmes that inspire involvement and transform the landscape for everyone affected by sarcoma. What we do 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 impact Awareness Connect (Sarcoma UK’s publication) is sent out three times a year and distributed to a database of 6000
• • ‘Sarcoma Voices’ is our active sarcoma community – see how you can help and ‘Speak out!’ sarcoma.org.uk/voices
Research Sarcoma UK has awarded scientific and medical research grants worth over £700,000
• • 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
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eal impact is about changing practice for the better. The impact of our ‘On the Ball’ campaign is starting to be seen across the UK (p6) with almost 1,000 GPs having been personally handed information to help them recognise the signs of sarcoma so that they can quickly refer people to a sarcoma diagnostic clinic. Steve’s GP liked the campaign so much that he has promised to pass the information onto other GPs in the practice, helping us to create even more impact. You can also read about the experience of one of our Sarcoma Voices at a GP training event where the baked bean tin and golf ball really caught the eye of GPs! Thank you to everyone who got actively involved and ordered a pack. I hope you will continue to be involved in this ongoing campaign to bring about earlier diagnosis of sarcoma. Understanding the impact of our research funding is vital for Sarcoma UK. We are delighted to be able to report on breakthroughs from two research projects funded by Sarcoma UK (p14). Our five new research grants are also announced, investigating fascinating and potentially ground-breaking areas of sarcoma research. This has been made possible by the charity’s largest investment into research so far (£250,000) and I would like to personally thank Sarcoma UK’s supporters for their generosity in helping make this happen. Best wishes
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• We provide support to 12 local sarcoma groups around the UK, and help establish new groups • 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 broad categories: – Soft tissue cancers – Primary bone cancers – Gastro-intestinal stromal tumours (a type of soft tissue sarcoma found in the stomach and intestines commonly known as GIST)
• There are around 100 different sub-types of sarcoma • About 3,800 new cases of sarcoma are diagnosed each year in the
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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 Connect
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Lindsey Bennister Chief Executive
The most common sarcoma sub-types are:
Soft tissue sarcomas • Leiomyosarcoma • Fibroblastic sarcoma • Liposarcoma • Gastrointestinal stromal tumour (GIST) • Kaposi’s sarcoma (KS) • Angiosarcoma • Malignant peripheral nerve sheath tumour (MPNST) • Synovial sarcoma • Rhabdomyosarcoma Bone sarcomas • Chondrosarcoma • Osteosarcoma • Ewing’s sarcoma • Chordoma
The current sarcoma landscape
• 10 people every day are diagnosed with sarcoma in the UK • There are around 13,000 people living 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 Scotland, just under 300 new cases of sarcoma are diagnosed each year • In Northern Ireland, 100 new cases of sarcoma are diagnosed each year
www.sarcoma.org.uk
Personal experience
‘A magical experience’ Sarcoma patient Gordon Cowan proudly carries the Commonwealth Queen’s Baton.
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many years before! By New Year I had my prosthetic leg, and was prepared to look forward to 2012, which turned out to be tremendous year!
ordon Cowan, retired minister and life-long athletics fan, was first diagnosed with soft tissue sarcoma in May 2006. This July, the 74-year-old proudly carried the Commonwealth Queen’s Baton through the streets of Stevenston, Ayrshire past an incredible crowd of onlookers.
Although my leg (nicknamed Cassius) has slowed me down, it has certainly not held me back from enjoying life to the full. The years following my operation were filled with seeing the grandchildren, holidaying and doing the things I love; including preaching for a while at the ‘Wee Tin Kirk’ in Ardeer.’
‘It was autumn when the small lump on my left knee began to appear, and before long it had grown to the size of a golf ball. After previous diagnoses of arthritis, and a meniscal cyst, it was finally diagnosed as a soft tissue sarcoma; at this point it was the size of a tennis ball.
In December 2013, a body scan was arranged and a large sarcoma growth was found in his abdomen. Gordon is now being treated with votrient.
From then on, I was under the care of the Sarcoma Team at the Beatson West of Scotland Cancer Centre in Glasgow.’
‘Carrying the Commonwealth Queen’s Baton this year was a magical experience that filled me with pride and humility.
After a successful operation, probable remission was declared by Christmas. Unfortunately, secondaries appeared in February 2007.
My energy and appetite have both returned and things are looking up. I have so much planned. Life goes on – what’s the point in waiting?’
‘I vividly remember first hearing my prognosis, My Sarcoma Specialist Nurse advised my wife and I to go straight home to take in the news.’ By May 2007, the growths had significantly decreased, and by the summer they had disappeared. Gordon remained healthy, and continued preaching in Scotland, until he noticed another growth in the calf of his left leg in 2011. ‘Everything happened very quickly, and after a high-level hospital discussion, the best option advised was an above the knee amputation. I agreed and the operation took place in July 2011. I received marvellous physiotherapy following the operation – by chance I actually taught the Head of Physiotherapy in Sunday School www.sarcoma.org.uk
Tantalizing treats from The Great Burls Bake-off Gordon Cowan holding aloft the Commonwealth Queen’s Baton
Dr Jeff White (Gordon’s consultant medical oncologist) and Gordon’s wife, Nancy make some noise! Connect
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Fundraising
Tapas in Cardiff? Swim in London? Cycle in Edinburgh? Take your pick! Every day you inspire us with your fundraising ideas and stories. Here are a few recent highlights.
All stays bright and dry on the track for Burton fun run ‘Watching people walk around with Sarcoma UK balloons and seeing banners for Sarcoma UK has also raised people’s awareness, which, for me, was the really important part of the day.’ The weather thankfully stayed dry and 270 people raced in either the Fun Run or the 5K. There was a magician and bouncy castle to entertain the children, a plate smashing stall and cakes, which of course all proved very popular! However, the highlight of the day was the attendance of Richard Whitehead MBE who started both races, running the 5K himself and presenting medals to winners! He posed for numerous photos and chatted to everyone; what a fantastic patron for Sarcoma UK he is, a true gentleman!
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The day itself has raised over £3,000. Geraldine Marsden Connect
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Ready, set, go at the Burton Fun Run
Wales boost funds in memory of Adam Jones and raise awareness of Ewing’s sarcoma ‘We are all so lucky and fortunate to have had Adam in our lives, and we will carry on and fight this disease, in his honour and memory so one day, when we have beaten this and young people are being saved, we will all know that Adam had a hand in saving them.’
Drivers swap lorries for bikes!
As a Cardiff City fan, Adam’s passions were sport and football. The football club has given a memorial brick to the family to place on their memorial
wall. His friends have already taken on running in Race for Life and the Cardiff half marathon; the ‘Big Fat Lorry Drivers’ have swapped their cabs for bikes, cycling 104 miles; and friends and family are busy organising a gala dinner complete with charity auction and songs from the West End. Alison Harvey
Team Thekla go big with a cycle from Loch Lomond to Edinburgh ‘Raising funds for sarcoma is essential. However, we also had a fantastic time and take away the most amazing memories of a very special day.’ Earlier this year some of my family, friends and I decided to set ourselves a personal challenge to have some fun and get fit. We arranged for 20 of us to take part in a cycle from Loch Lomond to Edinburgh. We had a great time training hard (in all weather, living in Scotland!), getting fit and forming new www.sarcoma.org.uk
Fundraising
friendships. We also contacted our local ASDA for help with provisions, along with a training centre, who provided a bike maintenance workshop, and our newspaper to let them know our aim; support was incredible. Since the event, we’ve had a re-union, and we all got a copy of a DVD, which captured our special day forever. We’re now planning our next event: a Triathlon, and will hopefully have new team members to help us raise more funds! Team Thekla
Team Thekla all geared up!
Connect cover stars, the Maritime Support Unit, notch up miles in the saddle! ‘We will swap our bikes for walking boots and walk up Mount Snowdon!’ Our team, who offer operational IT support to the Royal Navy and Royal Marines throughout the world, have been training incredibly hard to cycle 500 kilometres from Portsmouth to Snowdon; that’s 4 gruelling days of cycling before trekking to the summit of the mountain! mcsusnowdonchallenge14.org @MCSU_SNOWDON_14 Maritime Support Unit
Open water swim to raise funds and awareness ‘I lost my leg to sarcoma nine years ago, and recently completed an Open Water swim!’ Having lost my leg to sarcoma nine years ago, I wanted to complete a sporting challenge. I entered the 750m Open Water swim at Dorney Lake to test my stamina and fitness, and to raise funds for Sarcoma UK. The swim itself, the encouragement from other swimmers/organisers and the amount raised before the day, meant it was a real success – I loved it! 1500m next year... Janine Jackson
Want to fundraise? Get involved: sarcoma.org.uk/Fundraising Prudential Ride London-Surrey 2015 Fundraising target: £700
London Marathon 2015 Fundraising target: £3,000
This is an incredible chance for avid cyclists and beginners alike to follow the route made famous by the 2012 Olympic riders! Why not get a group together to take up Sarcoma UK’s places? Register your interest now fundraising@sarcoma.org.uk
With over £51,000 raised by Team Sarcoma last year, this year we’re very excited to offer 14 Sarcoma UK places – the most we’ve ever had! An incredible experience for seasoned runners and total beginners – with over 35,000 people taking part! Apply for your place via our website: sarcoma.org.uk/running
For more information: sarcoma.org.uk/cycling
Deadline for application: 3 November 2014 You don’t even need to run to participate in the London Marathon! Amongst our supporters along the route, Jan Cornell and her family and friends set up a cheering point and fundraising event at the Porter’s Lodge: mile 23. They managed to raise a staggering £10,000 on marathon day! The Cornells have raised over £55,000 to date in memory of Stephanie.
www.sarcoma.org.uk
Connect
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Awareness
‘On the Ball’
Is it sarcoma?
1,000 packs were distributed during sarcoma awareness week alone – that’s double our target! And this is just the beginning!
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t’s all down to you, the sarcoma community: patients, families, friends, work colleagues, healthcare professionals and Sarcoma UK supporters, who ordered an ‘On the Ball’ pack to inform your own GP face to face, about the signs and symptoms of sarcoma. We know that information sent in the post or by email rarely reaches GPs, and mass mailings are not viewed positively. By talking to your GP during your appointment, you helped us to get our information directly to thousands of GPs across the nation. Steve Mayer, wrote a cover letter before visiting his GP (right inset). ‘My GP thanked me for the pack, which he read in detail and found very interesting, quoting some of the statistics. He promised to pass it to other GPs in the practice, and he would also be happy to receive our support group leaflets for patients to see! So thank you for running the campaign! In my GP’s case it has been a great success and I hope it will be replicated around the country.’
June 2014
re: On the Ball campaign Dear GP,
Last year, I joined a Sarcoma Support Group, which helps with the emotional side of being diagnosed with sarcoma as well as offering practical advice. I found it extremely helpful.
The group is run by Sarcoma UK: a charity that deals with all types of sarcoma. Sarcoma UK are concerned that recognition of sarcoma is not as apparent as it could be, due to sarcoma being so rare (less than 1% of all cancers in England), and it is sometimes quite difficult to
diagnose. Unfortunately, the survival rate is only 55% - the aim is to dramatically increase this. A recent report from Cancer52 on National Cancer Intelligence Network shows that the survival rate for soft tissue sarcoma is 98% if the GP refers a patient within a month.
I consider myself very fortunate that you recognised my tumour and referred me, immediately,
but unfortunately not all GPs do. Statistically speaking, a GP is not likely to see more than one patient with sarcoma throughout their career making diagnosis that much more difficult.
With this in mind, Sarcoma UK has produced diagnosis packs, so I would be grateful if you could share the packs with the rest of the practice. Many thanks for your wonderful care Steve
Paralympic hero helps Sarcoma UK get on the ball against cancer
Nigel Phillips, Emma Tickle, Prof Rob Grimer and Richard Whitehead at the Royal Orthopaedic Hospital, Birmingham
Print and broadcast press attended our media launch at The Royal Orthopaedic Hospital, Birmingham. Our Patron, Richard Whitehead MBE and sarcoma patients, Nigel Phillips and Emma Tickle, backed our ‘On the Ball’ campaign and reached out to the public. Richard said, “I am backing Sarcoma UK’s ‘On the Ball’ campaign, to help people who are going through a terrible ordeal. I encourage the public to order an information pack and take it directly to their GP - from Sarcoma Awareness Week and beyond!”
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BBC Midlands, Radio Worcester and ITV local news picked up on the campaign with both broadcasters using footage in the local evening news. National coverage included a sarcoma story in the Daily Mail during the week.
Connect
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BBC Midlands Today: health correspondent Michele Paduano reports how sarcomas are often diagnosed at the size of a baked bean tin
www.sarcoma.org.uk
Awareness
Your impact Our ‘On the Ball’ tracker shows all the GP surgeries you targeted. Because of you, our campaign went national!
29% of you learnt about the campaign through Facebook or Twitter. One post reached 9,752 people with 123 shares. That’s how to raise awareness!
Face-to-face with GPs Sarcoma Voice, Karen Delin, took action at a GP training session in Havering. “As the only charity attending the event, the GPs appeared bewildered as to why I was there. Usually 10cm the doctors visit the stands of drug reps and instead, there I was with nothing to sell. Instead, my stand had a tin of baked beans sitting next to the Sarcoma UK ‘On the Ball’ information. This tin raised eyebrows, and also encouraged questions. It provided a visual example of what the campaign is all about; encouraging GPs to make early referrals to a sarcoma specialist centre. We know that the smaller the size of tumour at diagnosis, the better the outcome for the patient. I received positive comments from the GP who took on the message that early referrals will mean sarcomas can be diagnosed at the smaller golf ball size instead of the size of a 10cm tin. I hope such links can further develop allowing sarcoma awareness amongst GPs to continue to grow, improving the referral pathway for people with sarcoma.“
Lumps, bumps, and sarcomas: a guide
54% increase on our website hits during this particular week, with 1,204 website hits directly to our ‘On the Ball’ link.
The British Medical Journal Learning launched a new sarcoma online module during sarcoma awareness week. This promotes continuing professional development for GPs, GP trainees, hospital doctors, foundation doctors, practice nurses and emergency medicine practitioners. sarcoma.org.uk/BMJModule
We will be evaluating the impact of this campaign in more detail throughout the year and will keep you updated. You can still order your ‘On the Ball’ pack, today: sarcoma.org.uk/Awareness/ontheball www.sarcoma.org.uk
Connect
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Support & information
Listening to patients and understanding their views News from the wider sarcoma network, what they’ve been getting up to and how the community can further support you. Everybody welcome! The East Midlands Sarcoma Service is holding its first ever conference: Listening to patients’ views on sarcoma treatment and research. This conference is aimed at sarcoma patients, their carers and family and gives the opportunity to learn about the current treatments available to people with sarcoma and hear updates on the research looking into possible treatments in the future. Highlights of the conference include an open floor debate on research into sarcoma, an update from Mr Rob Ashford, Consultant Orthopaedic Surgeon from the East Midlands Sarcoma Service, on surgical treatments for sarcoma and a Key Note speech, from Roger Wilson CBE, Honorary President of Sarcoma UK. Date: 28 November 2014 Time: 10am -4.30pm
National Cancer Research Institute (NCRI), age limits on clinical trials need to be made more flexible to allow more teenage cancer patients the chance to access new treatments, so that young people don’t miss out on clinical trials. Simon Fuller, director of services for Teenage Cancer Trust, said: “Too many young people miss out on clinical trials, we have been working with patients, politicians, the NCRI and other organisations to increase awareness of this lack of access. Changes are critical to improving the quality of life and chances of survival for young people with cancer aged 13 to 24. We need everyone involved in the commissioning and regulating of clinical trials to work together across the UK, Europe and internationally to help save young people’s lives.” Lindsey Bennister, chief executive at Sarcoma UK, comments: “Having a diagnosis of sarcoma can be devastating to children, teenagers and young adults, and their families.
15% of all childhood cancers (0-14 years) are sarcomas, and sarcomas make up 11% of all cancer diagnoses in teenagers and young people (15-24 years). It is vitally important that children, teenagers and young adults have access to clinical trials to give them the greatest chance of survival and to maximise their quality of life.” Sarcoma UK encourages sarcoma clinicians to consider clinical trials as an option for younger sarcoma patients and to discuss this with them and their families. We would also urge researchers who design clinical trials to consider whether inclusion criteria for younger sarcoma patients is relevant for their study. There are a number of clinical trials currently available for bone and soft tissue sarcomas for younger patients. Information is available via Sarcoma UK’s website: sarcoma.org.uk/ Takingpartresearch Sarcoma UK provides funding through our research grants programme to some of these clinical trials.
Venue: Hothorpe Hall, Theddingworth, Leicestershire, LE17 6QX Register: paul.robson182@btinternet.com
Children, teenagers and young adults get better access to clinical trials
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A study funded by the National Institute for Health Research and Teenage Cancer Trust found that trials designed with broader age limits resulted in more teenagers and young adults going on clinical trials. According to a report from the Connect
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www.sarcoma.org.uk
Support & information Research
A personal insight into head and neck sarcoma Ross Anderton (now 6 years old) was diagnosed with Orbital Rhabdomyosarcoma at the age of 1. Lesley, Ross’ mother, gives us insight into their family’s sarcoma experience.
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e had noticed that Ross’s eye looked puffy. We took him to our GP who said it was probably a blocked duct and would refer Ross to the eye clinic for clarification. Five weeks later, Ross’s eye started to get bigger, the eye clinic appointment had not happened so I returned to our GP surgery. The doctor said that there was not much more he could do, so I decided to use private medical insurance. The doctor referred us to the private hospital and I called to make an appointment. When I phoned, I was told that there was only one eye specialist who would see children and he was on holiday. The following week Ross’ eye grew rapidly so I took him back to the same GP, who I’d seen the week before, and managed to persuade him to refer Ross as an emergency to the eye pavilion. We got an appointment for that afternoon. Once the eye specialist looked at Ross, I started to feel really worried. He consulted with the senior doctor who sent us straight for an ultrasound and booked us in for a biopsy; we were told that Ross had a sarcoma cancer. The days that followed were just a blur of hospital appointments and procedures for Ross. The staff on ward 2 at Edinburgh Sick Children’s hospital were fantastic and helped us through everything that happened in those first few weeks. There was so much information to take in – so many things to learn. Chemotherapy
www.sarcoma.org.uk
was tough, as Ross was kept in hospital, hooked up to fluids constantly. The cancer did not respond as well as expected after three intensive cycles of chemo, so a course of radiotherapy was due. This didn’t sound too bad to us and the ward staff did not make a big deal out of it. We met with the radiotherapy oncologist and learned of the side effects of traditional radiotherapy, including
Ross and his sister, Katie
“After a scan in 2009, we heard the fantastic word ‘remission’!”
an increased chance of developing a secondary cancer throughout his life. As any radiation would be targeted under the eye, it is close to the pituitary gland responsible for his growth, also the front of his brain where his learning and development could be affected. There’s a likelihood of cranio-facial surgery, and his facial bones (and their growth) would be affected by it. As Ross was only one year old, the side effects would be more severe for him. Connect
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Support & information
We went home and I researched the internet extensively to find Proton Beam therapy. The downside was that this was not available in the UK. I then started to contact the leading NHS specialists and approached our radiation doctor. She was happy to submit Ross for approval. After four weeks of fighting and stress, Ross had NHS funding and approval (the first patient in Scotland) to travel to Jacksonville, USA, for five weeks of proton treatment. We had to organise everything ourselves, as well as looking after a very sick child. Priority on arrival in the US was that Ross had to get titanium screws fitted into his skull. This was to ensure the utmost accuracy during the treatment. Proton is accurate to 2mm as opposed to over 1cm with traditional radiation – a huge difference in a one year old child; it also does not have an exit point which meant very little radiation to his brain. Our time in the US was long but it was enlightening and interesting. We met other families and experienced US culture and hospitality. The positive outcome of our journey is that other families have been able to follow us over to the US for treatment. After a scan in 2009, we heard the fantastic word ‘remission’! See page 14 for news of a Sarcoma UK part funded research project into Rhabdomyosarcoma Proton Beam Radiotherapy is not currently available in the UK but is funded by the NHS for overseas treatment for patients who meet very specific clinical criteria. If you want to find out more about whether this type of radiotherapy is relevant for you or your child, please speak to your oncologist. Your oncologist will assess whether you meet the clinical criteria and can refer your case to the UK Proton Clinical Reference Panel (a panel of experts) for consideration. There is reciprocal funding arrangements in place for patients in Scotland, Wales and Northern Ireland. It is planned that two NHS centres in the UK will provide proton beam therapy from 2018 for certain types of cancer.
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If you would like further information about Proton Beam Radiotherapy, please contact Sarcoma UK.
Connect
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Head and neck sarcomas of the bone and soft tissue Public Health England’s Knowledge and Intelligence Team (West Midlands) continue its articles for Connect, with a specific focus on head and neck sarcomas in this issue.
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etween 1990 and 2010, 4,796 patients were diagnosed with a sarcoma arising in the head and neck (H&N) region, of which 793 arose in the bones of the skull and face, and 4,003 within the connective and soft tissue. The most common histological diagnoses in the bones of the skull and facial skeleton were chondrosarcoma (232, 29%), osteosarcoma (190, 24%) and chordoma (129, 16%). The most common H&N soft tissue sarcoma morphological sub-types were leiomyosarcoma (724, 18%), sarcoma not otherwise specified (NOS) (663, 17%) and rhabdomyosarcoma (545, 14%). Connective tissue sarcomas were the most common type of H&N sarcoma in males and females (45.1% and 36.0% respectively) (Figure 1). Bone sarcomas constituted 20.9% of all head and neck sarcomas in females and 14.3% in males. The highest male to female incidence ratios were seen for soft tissue sarcomas; skin tumours (2.78 to 1.00) and connective tissue and nerves (2.40 to 1.00). There were no significant differences in the male (0.8 per million) and female (0.6 per million) agestandardised incidence rates over the period 1990-2010 (Figure 2) for skull and facial bone sarcomas. However, while female age-standardised incidence rates varied little between 1990 and 2010, male agestandardised rates in 2005-2010 were
1.5 times as high as those in 19932004. This difference is statistically significant. Age-specific incidence rates for bone sarcomas increased gradually with age, from 0.3 per million in the youngest age group (0-4 years) to 1.9 per million in the 80-84 year age group (Figure 3). Between the ages of 50 and 74 years, age-specific incidence rates in males exceeded those in females by a ratio of 1.8:1, a statistically significant difference. The age-standardised incidence rates of soft tissue H&N sarcomas for all persons over the period 1990-2010 oscillated around 3.5 per million population. Incidence rates in males (4 - 5 per million) were higher than those in females (2 per million) (Figure 4). This difference is statistically significant from 1995 onwards. Male age-standardised incidence rates increased significantly between 1993-1998 and 2005-2010 from 4.4 per million to 5.2 per million, while female incidence rates remained relatively constant. Soft tissue H&N sarcoma age-specific incidence rates increased rapidly with age from the age of 65 years onwards and were significantly higher in males than in females. In males aged 85 years and over, the incidence rate (60 per million) exceeded that in females (10.7 per million) by a ratio of 5:1 (Figure 5). www.sarcoma.org.uk
Statistics
Figure 1: Head and neck sarcomas in males and females (England: 1990-2010)
Bones Connective tissue, nerves Skin Nasal, sinuses, throat Head & neck Oral Male Female
Eye Glands Undefined
Key facts • With an incidence of approximately 38 tumours per annum,
bone sarcomas of the skull and facial skeleton account for 10% of all bone sarcomas Soft tissue sarcomas of the head and neck (H&N) region • are slightly more common with an annual incidence of 190 tumours and account for around 9% of all soft tissue sarcomas • The overall number of H&N sarcomas diagnosed in males exceeded those diagnosed in females by a ratio of 1.9 to 1 • Age standardised incidence rates of H&N bone and soft tissue sarcomas remained stable between 1990 and 2010 for male and female bone sarcomas and female soft tissue sarcomas • Male soft tissue sarcoma incidence rates increased significantly between 1993-2004 and 2005-2010. Reasons for this increase are currently being investigated
0 10 20 30 40 50
All sarcomas (%) Figure 2: Skull and facial bone sarcoma age-standardised incidence rates (England: 1990-2010)
Female
Male
1.6 1.4 1.2 1.0 0.8 0.6 0.4 0.2
2 1.5 1 0.5
0–4 5–9 10–14 15–19 20–24 25–29 30–34 35–39 40–44 45–49 50–54 55–59 60–64 65–69 70–74 75–79 80–84 85+
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010
Age at diagnosis (years)
Year of diagnosis Figure 4: Head and neck soft tissue sarcoma age-standardised incidence rates (England: 1990-2010)
Female
Figure 5: Head and neck soft tissue sarcoma age-specific incidence rates (England: 1990-2010)
70
Male
7
Female
Age-specific incidence rates (per million)
Age specific incidence rate (per million)
Persons
00
0
8
Male
6 5 4 3 2 1 0
Male
Persons
60 50 40 30 20 10
1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010
0
Year of diagnosis
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0–4 5–9 10–14 15–19 20–24 25–29 30–34 35–39 40–44 45–49 50–54 55–59 60–64 65–69 70–74 75–79 80–84 85+
Age specific incidence rate (per million)
1.8
2.5 Female
Age-specific incidence rates (per million)
2.0
Figure 3: Skull and facial bone sarcoma age-specific incidence rates (England: 1990-2010)
Age at diagnosis (years) Connect
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Research
Sarcoma UK grants target ground-breaking research Thanks to you, we have funded a quarter of a million pounds worth of research and we’re expecting big things from our researchers and their latest projects. Here you can find out more about each one… Studies of genetic susceptibility using exome sequencing in patients with sarcoma and an additional primary tumour Dr Clare Turnbull, Institute of Cancer Research, London £92,635 for 2 years
Discovering more about the genetics of sarcoma… This project aims to identify genes which make people more susceptible to developing sarcoma by looking at a set of patients who have been diagnosed with sarcoma and another primary cancer. This is important because any genes found could be used in genetic testing for sarcoma patients and their families in the future.
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sarcoma and additional cancers. They will also collect samples from at least 400 additional sarcoma patients at the Royal Marsden Hospital. This will be a sample series in which to further investigate their findings, which will also form the basis of additional future studies into the genetic basis of sarcomas. Dissecting the mechanisms of pazopanib in soft tissue sarcoma Dr Paul Huang, Institute of Cancer Research, London £31,301 for 1 year
Pazopanib resistance – why does it happen?
Pazopanib, a drug that targets a class of genes known as ‘kinases’, Genetic testing can be useful in the treatment of sarcoma. was recently approved for the treatment Identification of particular genes may offer opportunities of advanced sarcoma. While some patients respond for individualised therapy by giving information about well to pazopanib, the drug does not work in all cases, the behaviour of the tumour and how to treat it. It could meaning that some patients are exposed to the potential also give knowledge about the risk of future additional side effects of the treatment for little or no benefit. At cancers, for which prevention and/or screening could present, it is challenging to predict who will respond be offered. Testing could also be offered to a patient’s to pazopanib, and it would be helpful to find tumour family to provide reassurance or the possibility for risk markers that enable doctors to identify the right patients “We aim management. to administer the drug. Furthermore, because it is to identify genes unknown as to why some patients do not respond There are currently 10 known inherited gene to pazopanib, there is no rational basis for selecting that make people mutations, which give a higher risk of developing alternative treatments in such cases. Data from sarcomas. At the moment, relatively few sarcoma more susceptible to other cancer types suggest that kinase drug patients are offered genetic testing and typically therapy ‘rewires’ the cellular communication developing sarcoma” machinery in cancer cells to drive drug resistance. this is only for one or two of the genes. Via application of new sequencing technologies in clinical Dr Clare Turnbull diagnostics, it will soon be possible to offer genetic In this project, the team proposes to identify these testing for multiple genes to more sarcoma patients. ‘rewiring’ events as potential targets for the treatment of sarcomas. Using a tool known as mass spectrometry, The team will collect DNA samples from two hundred they will map the cellular communication machinery individuals across the UK who have had sarcoma and activated in sarcoma cell lines of different types and an additional primary cancer. They will use the latest assess how this ‘rewiring’ facilitates drug resistance. By technology to sequence 20,000 genes (the exome) in grouping the ‘rewiring’ maps from different sarcoma cells these samples. The primary purpose of this research using computational methods they can determine which project is to identify new sarcoma susceptibility genes, cellular components are commonly found in resistant cells but a secondary outcome will be to gain an overall picture and are responsible for conferring drug resistance. The of the frequency and contribution of all known cancer data from this project will be used as a starting point for predisposition genes in patients who present with further research. Connect
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www.sarcoma.org.uk
Research
This research will not only provide a molecular portrait of sarcoma biology, but also result in the identification of predictive markers to identify patients who are likely to benefit from pazopanib treatment. Additionally, gaining an understanding of the ‘rewiring’ events in sarcoma cells that are resistant to pazopanib, will provide a starting point for the rational selection of alternative treatments for patients who do not respond to this drug.
Study to investigate correlations between age-dependent pharmacokinetic variation, side effects of chemotherapy and biomarkers of toxicity in Ewing’s sarcoma Dr Gareth Veal, Northern Institute for Cancer Research, Newcastle University £43,490 for 3 years
How can we tailor drug doses in Ewing’s sarcoma to maximise benefit and minimise side effects? This project will be investigating what happens to the key drugs administered to Ewing’s sarcoma patients once they have entered the body – how they are broken down and what factors are important for determining response and toxicity, to gain more information in order to improve treatment strategies. This may be particularly important for teenagers and young adults, the most commonly diagnosed group, as they “The drugs may handle drugs differently to younger children. Modifying drug doses for studied, and different patient groups will allow the the findings, will be achievement of drug exposures which are most likely to be beneficial, whilst applicable across many minimising side-effects.
types of sarcoma”
Dr Gareth Veal As another part of the project, the team will also perform a series of blood tests used to predict side effects of chemotherapy in adult patients to see if they are helpful in children. Although this study focuses on Ewing’s sarcoma patients, the drugs studied, and the findings, will be applicable across many types of sarcoma. Role of TTBK2 and cilia in sarcoma progression Dr Barbara Tanos Institute of Cancer Research, London £58,415 for 1 year
Back to basics – the role of cilia in sarcoma Most cells of the human body have structures known as cilia, which are critical for the cell’s ability to sense its external environment. It has been suggested that there is a potential link between www.sarcoma.org.uk
these structures and cancer development. In fact, 70100% of sarcomas show loss of these primary cilia and the structures that give rise to cilia: centrioles. However, the role of cilia, and the genes associated with cilia formation in the development of sarcoma, is as yet unknown. Dr Tanos has previously studied a protein involved with cilia known as TTBK2. This protein has been found to be mutated in a number of cancers, and so may also be involved in cancer development. Preliminary data shows that cells with a TTBK2 mutation are unable to grow cilia. This is a new, unexplored area but a hypothesis is that the presence of cilia could impose a barrier to sarcoma development. This project will examine the role of TTBK2 in a sarcoma model by removing the TTBK2 gene and seeing how this affects cancer development. Findings will then be validated in a patient-derived cell line. The team will also analyse patient samples to see whether the presence or absence of TTBK2, and another protein, can be used as a marker for changes in the cilia in sarcoma. This research hopes to increase our knowledge of sarcoma at the most fundamental level, and to provide tools for the discovery of new ways to treat and cure sarcomas. Assessing the potential of circulating tumour DNA as a new biomarker for low grade bone and soft tissue neoplasms Dr Tim Forshew, University College London £25,680 for 6 months
A blood test for sarcoma? It has been established that certain high grade tumours release mutant DNA into the blood and that this can be used to monitor response to therapy, and to detect early recurrence of disease. Dr Forshew was the first to prove that it’s possible to read large parts of this faulty DNA from the blood and was involved in the largest study on this new test in patients with breast cancer. He will now apply these skills to bone and soft tissue sarcomas and low grade connective tissue tumours, using a large collection of bio-banked samples at University College London. They will take this work forward and test if the faulty DNA that occurs in benign and low-grade tumours can also be detected in the blood of patients. The team will analyse the DNA of low-grade bone and soft tissue tumours for frequently occurring mutations. If mutations are found they will gather data, which will allow them to progress onto a larger research project in a clinical setting. The potential benefit of this work is that if this Connect
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Research
approach is found to be successful for detecting faulty DNA from low grade tumours in a patient’s blood, it would mean that a simple blood test could be used to monitor these tumour types. This could be used to monitor patients for recurrence of disease, and where
relevant in some tumour types, for response to treatment. An advantage of a blood test is that it could be performed by a GP rather than visiting a hospital. It may also be cost efficient, if it allows us to reduce the need for expensive tests such as x-rays and CT scans.
Sarcoma UK funded studies make breakthrough in saving lives Thanks to your donations, two funded studies for Sarcoma UK make breakthroughs!
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t has been a fantastic few months for Sarcoma UK’s growing research programme. Two of our funded projects have published their findings in high quality scientific journals, moving forward the knowledge about sarcoma and how to treat it.
Limb saving therapy Researchers at the Institute of Cancer Research, who were awarded a Sarcoma UK research grant in 2009, have discovered that combining viral therapy with traditional chemotherapy, in a limbsaving technique known as isolated limb perfusion, boosts the effectiveness of the treatment. This involves giving treatment directly to the affected limb without circulating it through the entire body.
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Professor Kevin Harrington, the Principal Investigator, says: “Our research shows that a virus that targets and kills cancer cells could significantly improve an existing treatment for advanced skin cancer and sarcoma in the arms and legs. Combining modified virus and isolated limb chemotherapy doubled survival times in the laboratory, which gives us hope that it might be effective in the clinic. We have approval to start clinical trials on the combination therapy and hope to begin
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testing in patients in the near future. The beauty of this technique is that the arm or leg is isolated, making it harder for the virus to be destroyed by the immune system – something that has been a challenge for virus treatments in the past. The study also showed that the virus didn’t cause any adverse effects, adding to evidence that it should be safe to use as a cancer treatment.” The team had their findings published in the International Journal of Cancer, you can find a link to the paper on our website. sarcoma.org.uk/limbsavingtherapy
Rhabdomyosarcoma breakthrough!
Dr Henning Wackerhage said: ‘Our identification of the Yap protein’s crucial role in the development of rhabdomyosarcoma is the first step on the road towards understanding how we can target this rare disease. Our work will now focus on how the Yap protein works in cancer and how its activity can be controlled. If we can achieve inhibiting Yap locally in the tumours, we could cause the cancer to stop and regress by turning the RMS into normal muscle instead. This would most likely produce significantly less side effects than the current therapies.’ The findings were published in the journal Cancer Cell. You can find a link to the full paper on our website. Let’s wait to see what this project brings for year two! sarcoma.org.uk/AberdeenUni
A collaborative study led by scientists from Harvard and involving Sarcoma In other news… UK grant holders from the University Professor Adrienne Flanagan, Research of Aberdeen has revealed, for the Advisory Committee member and first time, the key role that a protein Sarcoma UK grant holder, has been called Yap plays in triggering a awarded the prestigious honour of being type of sarcoma common elected to the Academy of in young children: Sciences. The Academy is “Inhibiting rhabdomyosarcoma an independent body (RMS). in the UK promoting Yap would most medical science and likely produce less The Yap protein is involved its translation into in the development benefits for society. side effects than of new muscle cells. In the current rhabdomysarcoma, the Yap Professor Flanagan is protein remains active rather considered a leading therapies” than ‘turning off’ (as it normally expert in the field of would) like the accelerator of a car chordoma research, both in becoming jammed. the UK and internationally.
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Research
Meet our new Head of Research: Sarah McDonald Helping us to reach our goals, seek answers through research and increase sarcoma awareness amongst scientists and researchers.
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come to Sarcoma UK from my role as Senior Research Development Officer for the Science and Medicine Cluster at the University of Sussex. Here, I supported academic faculty in the School of Life Sciences with research development and funding, having previously supported Brighton and Sussex Medical School.
Getting patients more involved in research is also critical. This isn’t about going straight into clinical trials for drugs, there are lots of ways to get involved, from giving informed consent for additional samples being taken, explaining and promoting understanding around tissue banking, and helping put patients and researchers together.
Prior to this I spent over 14 years working in Histopathology as a HPC registered Biomedical Scientist, working at a variety of Trusts. I was a Fellow of the Institute of Biomedical Science, sat on the Scientific Advisory group for Cellular Pathology, and was also a Chartered Scientist. I have held senior management positions within the NHS before leaving to working in Research Administration and Management.
What are your aims as Head of Research – what would you like to achieve? Working proactively with all of those involved in the charity: patients, donors, Research Advisory Committee (RAC) and the Board to support all research aspects of the charity’s goals. To ensure that all investments in research reach their full potential by working closely with the researchers we fund to capture and promote their outputs and results, and that this is fed back appropriately to all involved in Sarcoma UK and the sarcoma community. Supporting the RAC, making sure that we are attracting the best proposals to review for funding. It is important that governance around the awarding of research grants is robust leading to high quality research. www.sarcoma.org.uk
Have you seen many changes in research over your working years?
Sarah McDonald
provide better classification around diagnosis; from “cancer” to “sarcoma” to sub-types “liposarcoma; rhabdomyosarcoma; chondrosarcoma”, and again basic science has given evidence to this.
What impact do you hope to make? This is an exciting new post and I am very excited to be starting and working alongside the great people at Sarcoma UK. I think the impact of the post on donors will be supporting the RAC around investing in the best projects, ensuring Sarcoma UK’s goals are reached. Working with those funded researchers, we can pick up on results, and ensure that donors are informed. Generally, contributing to the range of resources and information about sarcoma.
I’ve spent most of my working life with cancer, which from a scientific point of view is a fascinating disease system. I’ve seen first-hand the changes to patient treatment and care which research has driven. When Then there is impact on the research I first started in Histopathology, community. Researchers are keen for we used to receive large buckets of their research to have outputs and surgical specimens where the only impact beyond their own community. treatment for cancer was whole Many want to work with patients organ removal, this then progressed and families when designing research to specimens around resection of proposals, the opportunity to link up margins and clearance. directly with patients and see Changes driven by “I’ve seen their work make a difference research in both is incredibly rewarding and science and first-hand the motivating for them. technology changes to patient mean that for many treatment and care And apart from cancers, we research, what else which research has makes you tick? can now make a confident Spending time with my family, driven” accurate diagnosis sewing, knitting, and playing either from a biopsy the baritone horn in the Lewes or more advanced technology. Glynde & Beddingham Brass Band. We are now able to classify tumours Mostly you will see me riding my and as we move towards personalised bicycle, having taken up the London to medicine, we can stratify treatment Brighton challenge (twice!). and predict if a patient will respond to a specific drug treatment. Check out our research pages sarcoma.org.uk/research to There has also been a drive to keep updated! Connect
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Donate!
Giving a one-off or regular donation ensures that we can continue to raise awareness, provide support & information and fund vital medical research into sarcoma
sarcoma.org.uk
£5
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You could enable sarcoma Clinical Nurse Specialist, Julie, to give every newly diagnosed patient a Personal Guide to Sarcoma.
You could banish Abdul’s feeling of isolation. He can receive our newsletter, Connect, three times a year for two years.
You could educate ten GPs as part of our ‘On the Ball’ campaign. The Golf Ball reminder could prevent Harry from having to visit his GP three times before being referred to a sarcoma specialist.
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How?
You could provide the necessary equipment for Reema to continue her vital sarcoma research.
You could help Tom find the support he desperately needs at a sarcoma support group in his area.
Online: sarcoma.org.uk Text: Text SAUK00 £10 to 70070 Minimum donations £1 and maximum donation is £10. Gift Aid can also be added. Cheque: Make payable to Sarcoma UK and post to Sarcoma UK, 49-51 East Road, London, N1 6AH Bank: Made via any branch of Lloyds Bank using the following details to fill out one of their paying-in-slips: Payee: Sarcoma UK Sort Code: 30-97-62 Account No: 02884274 Reference: Your name
sarcoma.org.uk 020 7250 8271 info@sarcoma.org.uk @Sarcoma_UK uk.sarcoma 49-51 East Road, London N1 6AH 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 team Editorial: Vicki Smith, Scott Temple, Katharine Tucker, Glyn Wilmshurst Design and layout: INQ Design Ltd 020 7737 5775 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. No part of this publication may be reproduced in any way without prior permission from Sarcoma UK.
Board of Trustees Lesley Abraham Dr Jane Barrett Karen Delin Prof Rob Grimer Leigh Hibberdine Ian Hughes Peter Jay Nicky Mellows Sharon Reid Helen Stradling Dave Thompson Dr Jeff White Sam Whittam Glyn Wilmshurst Honorary President Roger Wilson CBE Scientific/Medical Advisor Professor Ian Judson Patron Richard Whitehead MBE