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National 100k Genome Project focuses on CUP
In December 2012 the Prime Minister announced that the government has earmarked £100 million pounds for a ‘100,000 Genome Project’, offering the hope that it could transform how we diagnose and treat our most complex diseases: "The genome profile will give doctors a new, advanced understanding of a patient’s genetic make-up, condition and treatment needs, ensuring they have access to the right drugs and personalised care far quicker than ever before…. up to 100,000 patients over three to five years having their whole genome - their personal DNA code -sequenced."
In April 2013 it was announced that the Chief Medical Officer’s scientific priority group had selected CUP (along with lung cancer, infectious diseases, and rare diseases) as one of the clinical areas where this important effort will be focused.
In making this decision, it was stated that "...developing a molecular taxonomy is seen as offering a potential leap forwards for the treatment and prognosis of CUP." [Taxonomy = understanding and classifying]. The Department of Health advise that the intention is to start recruitment of patients to the project in April 2014.
What does this mean for CUP patients? Dr Richard Osborne, clinical lead for the NICE Guideline and member of the Cancer of Unknown Primary Foundation’s Advisory Board comments:
“Sequencing provides detailed information about the numerous abnormalities that exist in the DNA of cancer cells. By examining the patterns of defects in well-characterised groups of patients, it will ultimately be possible to know more about the causes and behaviour of the disease, as well as allowing more rational selection of treatment.
In the first instance, CUP patients recruited to the 100k genome project will play a part much like conventional blood donors do today – providing specimens of their cancer as a service for medicine. It is not expected that individuals will directly benefit routinely (though there may be instances where this happens). Instead, it is expected that in time a sufficiently detailed “map” of CUP biology will be developed, to assist in the care of other patients. The details have yet to be worked-out but this is the most fantastic news for CUP patients of the future.”
This new flagship development follows the NICE Guideline for CUP (2010) and the Peer Review Measures (2013) meaning that CUP is now on the radar of the Department of Health (DoH) and the wider NHS in a way that it never has been before. [May 13]
National Cancer Patient Experience Survey 2012
The latest Cancer Patient Experience Survey (CPES) offers us an insight into cancer patients’ experiences. We have looked at the data for CUP patients and compared it with the overall experiences of cancer patients (National Cancer Patient Experience Survey 2011/12. DoH Friday 17 August 2012):
- The survey shows, very obviously, CUP patients responses to be generally more negative than the national ‘all’. Using the DoH’s benchmark of less than or equal to70% as being ‘less positive’ there are 23 ‘less positives’ for CUP versus 16 for ‘all’. The lingering aura of nihilism surrounding CUP may be a factor in the lower scores but it should be recognised that there is a significant variation between the ‘big 4’ collectively and the less common tumour sites. There are wider variations. For example: ‘Given easy to understand information about the type of cancer’ (Q14) is 11% worse for Sarcoma patients than for CUP patients. There are some questions where the particular nature of CUP could be seen to make a negative response unsurprising.
- There are some ‘less positives’ that are easily rectified. Such things as: the lack of patient information and information about support groups. CUP CNSs are effectively being introduced as part of the CUP MDT and ‘information’ features in Peer Review Measures. Knowing that many hospitals draw on the information we provide in preparation for Peer Review we should see improved scores in the future.
- Albeit that this is a national snapshot, and may not represent individual hospitals, of particular concern to clinicians and nurses should be that: information and support, confidence and trust, and effective communication by doctors and nurses in relation to CUP patients are perceived to be significantly lower than the national ‘all’.
We are seeking to raise £55,000 to fund a molecular profiling pilot project that aims to develop more efficient treatment and management of CUP. The project is led by Dr Harpreet Wasan (Consultant & Reader in Medical Oncology Department of Cancer Medicine, Hammersmith Hospital). Molecular analysis technologies are evolving extremely rapidly, and in the last few years it has become feasible, both economically and scientifically, to look at the DNA sequence (whole genome) of previously banked or stored tissue biopsies. The project aims to uncover potential biomarkers (predictive and prognostic) of CUP by utilising clinical tissue samples, accrued at the Hammersmith Hospital, that show hallmarks of CUP metastasis, where no primary site of cancer is identified. Next generation sequencing will be performed on a subset of the samples as a pilot that will help us to understand the disease and detect potentially “drug-able” mutations. A successful pilot will enable further research. We have contributed £20,000 to our £55,000 target in 2012. [Jan 13]
New 'app' developed by Dr Richard Osborne for those on chemo
Dr Richard Osborne of our Advisory Board has developed a new smartphone app which helps patients deal with treatment toxicity.
The idea is that if patients experience, say, excessive levels of nausea, vomiting, mucositis etc, the app prompts them to contact their cancer hotline and speeds up that process. The app also helps accelerate access for treatment if potentially serious infection is present. It is called the Cancer Emergency Response Tool (CERT) – and it’s available for download for free, for Android phones. It can be found by going to Google Play and searching for “cancer emergency response tool” . [Nov 12]
Peer Review Measures for CUP
The Peer Review Measures for CUP were launched by the National Cancer Action Team on 24 October 2012. This is a very significant moment for CUP patients of the future in England. The measures, based on the NICE Guideline of July 2010, are used by the National Cancer Peer Review Programme as part of the assessment of cancer services.
What this means is that Hospitals treating CUP patients in England from 2013 will be 'audited' to see whether they are 'compliant' with the 'measures'
The 2010 NICE Guideline has started to revolutionise the way CUP patients are treated but it is a 'Guideline'. The Measures add 'teeth' to the Guideline and hospitals treating CUP patients that are deemed non compliant with the Measures will be named publicly. [Oct 12]
NCIN Conference - June 2012
The latest data for CUP were available in a briefing sheet at the conference. The poster presented at the conference by the CUP Foundation argued that CUP was not a rare disease, although it is often described in this way:
- A disease description defined by numbers is seldom important for patients. It may contribute inadvertently to patient inequalities. Rarity is irrelevant for a cancer patient unless it brings benefits. It is their cancer. The concern for CUP patients is to be treated by a clinician with specialist knowledge of their disease, and supported by nursing staff who understand this very difficult, ‘heartsink’, diagnosis.
- CUP, at 7% of overall cancer mortality, is not rare. But it is a phenomenon that is poorly understood leading to uncertainty amongst clinicians (ameliorated by the advent of the 2010 NICE Guideline for England and Wales by the physicians who make reference to it) and epidemiologists. This has an impact on the quality of patient care and research.
- CUP incidence in the younger age groups may be masked by age standardisation and a headline decline in the incidence of ‘total persons’. Further analysis is needed. [Jun 12]
2nd International CUP Conference, London
On 27 April the CUP Foundation ran the triennial conference bringing together world experts with UK medical practitioners. Some 150 clinicians, nurses, managers, patients, and carers attended. Click on output for reportage and slides. [Jun 12]
Article from the 2009 conference printed in Annals of Oncology Vol 23. No. 2. Feb 2012. pp298-304.
An article reflecting the proceedings of our first international conference has been published in hard copy in the February edition of Annals of Oncology. Cancer of unknown primary: progress in the search for improved and rapid diagnosis leading toward superior patient outcomes can be accessed here. [Feb 12]
Draft Cancer of Unknown Primary Measures for the Manual of Cancer Services
A draft version of the Cancer of Unknown Primary Measures have been issued for consultation by NCAT (Start Date 21st December 2011 Close Date: 14th March 2012). The measures, based on the NICE Guideline of July 2010, are used by the National Cancer Peer Review Programme as part of the assessment of cancer services. They will help also to provide a ready specification for commissioning of cancer services within the NHS. They can be found on this page under “measures out for consultation”. [Posted Dec 11]
Evaluating the development of a pathway for patients presenting with CUP
A useful study has been undertaken at Chesterfield Royal Hospital in conjunction with Sheffield University and funded by Macmillan Cancer Support. The study looked at the development of a new pathway for CUP patients following the introduction of the NICE Guideline in July 2010. Data were collected on two cohorts of patients. The first related to the pre-pathway phase, and numbered 50. The second was from the pathway implementation phase, and numbered 38. The study confirmed the importance to patients of reducing delays, uncertainties and lack of continuity. The study appears to indicate that instituting a CUP pathway in a district general hospital has the potential to contain the length of time taken for investigation of CUP. [Posted Sep 11]
Acute Oncology (distilled from Macmillan Cancer Support /July 2011)
The National Confidential Enquiry into Patient Outcomes and Death (NCEPOD) Report (2008) raised significant concerns regarding the safety and quality of chemotherapy services. In response to this the National Chemotherapy Advisory Group (NCAG) Report (2009) highlighted the need to improve a number of areas including the provision of emergency care for cancer patients who develop complications following chemotherapy, but also for patients admitted suffering from consequences of their cancer. It recommends that all hospitals with an Accident and Emergency (A&E) department establish an acute oncology team (AOT), combining staff from A&E departments, acute medicine and oncology to coordinate the service in that hospital.
A further NCEPOD Report (2009) highlighted issues over the lack of ability of health care professionals and teams to identify patients approaching end of life (EOL) and inadequate involvement of palliative care teams and deficits in end of life care patient management. This often resulted in admission when it was inappropriate. Numerous reports have underlined the fact that many patients nearing the end of life are admitted inappropriately and may die in hospital when they would rather die elsewhere.
The final Acute Oncology Measures were published in April 2011 and for the purpose of the cancer quality measures and peer review, acute oncology will be reviewed as a separate activity, integrated across each cancer network in England. Whilst the national guidance has been developed in England, the clinical issues are the same across all four nations.
Aims of an Acute Oncology Team
- Better care of patients presenting with acute complications of their cancer treatment
- Better care of patients presenting with emergencies caused by the disease process itself, whether the primary site is known, unknown or presumed
- Early identification and appropriate care of patients approaching EOL
National Confidential Enquiry into Patient Outcomes and Death (2008) For better, for worse? A review of the care of patients who died within 30 days of receiving systemic anti-cancer therapy.
National Chemotherapy Advisory Group (NCAG) Report (2009) Chemotherapy Services in England: Ensuring Quality and Safety.
National Confidential Enquiry into Patient Outcomes and Death (2009) ‘Care to the end? A review of the care of patients who died within hospital within 4 days of admission’
National Cancer Action Team (2011) Acute Oncology - including metastatic spinal cord compression measures.
[Posted Sep 11]
Paper accepted for publication
A paper based on our last conference has been accepted for publication in the prestigious Annals of Oncology. The title of the article is: "Cancer of Unknown Primary: Progress in the Search for Improved and Rapid Diagnosis Leading Toward Superior Patient Outcomes". See the abstract here. [Posted Jun 11]
Research on how CUP data are collected by hospitals and presented by cancer registries
Clear definitions and good data collection underpin the ability to undertake high quality research and audit. Without this it is impossible to improve the management of, and outcome for, CUP patients.
A recent study by Kathie Binysh, Richard Osborne and John Symons - Capturing Cancer of Unknown Primary (CUP) Data: Known Unknowns or Unknown Unknowns? - was presented at the National Cancer Intelligence Network Conference in mid June 2011.
The aim of the study was to understand how CUP data are recorded presently by Cancer registries. The study’s objectives sought to establish (1) the completeness and accuracy of the data (2) whether there is consistency of practice between registries (3) the extent of analysis and data presentation and (4) how the quality of data collection might be improved in the future.
Replies were received from eight out of the eleven registries in the UK. In short, it is likely that CUP is under-reported and that there is a lack of consistency in collating data. The more specific findings below are likely to be of interest, particularly to researchers and clinicians:
- The WHO cancer code C80 (Malignant neoplasm without specification of site) appears to be the most commonly used code within the usual range of C77-C80.
- Registries were asked the total numbers and proportion of cancers of unknown primary they registered during 2008. This varied between registries from 0.7% to 4.0%. This variation could be due to a difference in the incidence of the disease but could also result from differences in coding practice.
- The incidence of cancer of unknown primary appears to be falling. This might be due to real changes in the incidence but could also reflect improvements in identifying the primary site, such as better access to diagnostics; the effectiveness of MDT working or changes in coding practice. Anecdotal evidence suggests that CUP patients reviewed at MDTs are often classified as having a probable primary tumour which corresponds to the site-specialty of the MDT. While this is understandable, and indeed may be correct in some cases, this approach involves unreasonable assumptions, as well as being non-uniform (different MDTs will have different “thresholds” for attributing a probable site-specific diagnosis).
- The supporting evidence used to come to an unknown primary coding included death certificate, pathology, MDT records and clinical notes. Some registries mentioned talking to clinicians, both within secondary care and primary care. One registry mentioned that it would discuss the classification with the coding department in the reporting hospital.
- Distinguishing between unknown primaries and tumours that may be a metastasis from an existing tumour registration, showed that the greatest emphasis was placed on the morphology. If there was an existing primary with the same morphology then it was assumed that it was a metastasis from the primary tumour. Basal cell carcinomas were excluded from this rule. If there was no morphology or there was doubt, then one registry stated that they would register a second tumour. Other registries also talked about discussion with pathologists or clinicians involved with the case before coming to a decision. [Posted Jun 11]
National recording of CUP statistics
In recent months we have been discussing the presentation of CUP statistics with the National Cancer Intelligence Network (NCIN) and Cancer Research UK (CRUK). Very significant changes have been agreed which will give CUP greater prominence. This is important for raising awareness that translates into possibilities of research funding and patient benefit. Dr Richard Osborne of our Advisory Board describes this news as: "a huge leap forward in CUP care."
On 20th April 2011 CRUK published their 2008 CancerStats Report which includes CUP. See here for details of CUP incidence and mortality.
CRUK plan also to have a new CUP section on their CancerStats website in due course. There, they will include the graphs for CUP incidence and mortality.
NCIN and CRUK will be publishing shortly a collaborative data briefing on CUP. This will look at CUP in more detail, examining how the incidence varies by age, by sex, by ICD-10 site code, both in the latest years available and over time. This collaborative work will help to explore any variation in the cancer registration processes to try to make the use of data recorded as “C77-C80” as “CUP” equitable across the country, and as clinically relevant as is possible. [Posted May 11]
Research looking at the familial associations of CUP
The Journal of Clinical Oncology (May 2011) has an interesting article - the abstract is here. The research suggests a familial association of CUP with kidney, lung, and colorectal cancers. [Posted May 11]
Oncology – a magazine with a wide distribution amongst oncologists carries, in the latest edition, a comprehensive article about CUP by Dr Richard Osborne with a half page advert for Jo’s friends alongside. We are trying to ensure that all oncologists in the UK are aware of our existence and can point patients and carers towards our services. [Posted Mar 11]
With other health organisations, we have encouraged NICE to upgrade the cancer referral guidelines for GPs. Early diagnosis is the key to effective treatment. NICE have decided to embark on a revised Guideline which is good news. Jo’s friends is registered as a stakeholder in the process which means we get a chance to comment at various stages as the Guidelines are researched and written. [Posted Mar 11]
Improving outcomes - a strategy for cancer
The Government published this document on 12 Jan. It refreshes the Cancer Reform Strategy directing NHS cancer services. It aims to bring cancer outcomes up to the European average through earlier diagnosis. [Posted Feb 11]
Living with cancer research
Warwick Medical School Clinical Trials Unit would like to invite UK patients to take part in an innovative study to explore what it is like living with cancer. They want to do it through words and pictures (photography). They will interview you and, if required, lend you a camera. For further details contact C.Balmer@warwick.ac.uk or ring her on 02476 151179. [Posted Jan 11]
The September edition of Cancer Nursing Practice carries an article by James & Symons on the implementation of the new NICE Guideline for CUP. [Posted Oct 10]
The study recruitment has now begun to take off with a significant increase in the recruitment figures over the last 2 months. With additional sites opening imminently and new sites expressing interest in the study enthusiasm for the study remains high and it is anticipated that CUP-One will meet the projected recruitment within the anticipated timelines.
31 patients have been recruited to date from the following centres: Aberdeen Royal Infirmary, Beatson West Of Scotland Cancer Centre; Hammersmith Hospital; Kent Oncology Centre; King Edward VII Hospital, Windsor; Peterborough Hospital; Wexham Park Hospital; Royal Marsden Hospital; Doncaster Royal Infirmary; Weston Park Hospital, Sheffield. New centres are opening shortly. [Posted Oct 10]
NICE Guideline for CUP
The NICE Guideline for the management & treatment of CUP was introduced on 26 July 2010. This is the most important thing ever to happen for CUP in England & Wales (NICE does not cover Scotland and N. Ireland but it is likely that the other national authorities will be informed by this guidance). Of course, we have to recognise that this is Guidance and not mandatory but initial reactions to the recommendations are encouraging. Jo’s friends is a Stakeholder and the Director has been a member of the Guideline Development Group since it started work in 2008.
There will be costs involved in the implementation of the Guideline, but there should be savings from the more rational management of patients passing through the system with improved pathways for investigations and decreased length of hospital stays. In essence it is thought likely that implementing the Guideline will be cost neutral. The Coalition Government have committed to achieving “cancer survival rates among the best in the world” by 2015. Survival rates are presently amongst the worst in Western Europe but the introduction of the CUP Guideline, if implemented, is an important step on the journey to achieve the Government's aspiration.
Until now a patient presenting with CUP has suffered from ad hoc approaches. He or she has often “bounced” between site-specific Multi Disciplinary Teams as each tries to find a primary site of the cancer with different tests. Expertise should now become more focused; and patient support is clearly defined in this evidence-based approach to the management and treatment of CUP. The Guideline is very comprehensive and can be found here.
Highlights of the Guideline. We highlight some key points from the patient perspective:
- The diagnosis is defined in three distinct phases:
- Malignancy of undefined primary origin. Metastatic malignancy without a probable primary site identified on the basis of a limited number of tests.
- Provisional carcinoma of unknown primary (provisional CUP). Metastatic epithelial or neuro-endocrine malignancy identified on the basis of histology/cytology, with no primary detected after an initial screen of investigations.
- Confirmed carcinoma of unknown primary (confirmed CUP). No primary detected despite further specialised tests and specialist reviews.
- Every hospital with a cancer centre or unit should establish a CUP team, and ensure that patients have access to the team when malignancy of undefined primary origin (MUO) is diagnosed.
- The CUP team should consist of an oncologist, a palliative care physician and a CUP specialist nurse or key worker as a minimum.
- The CUP specialist nurse or key worker should:
- take a major role in co-ordinating the patient’s care in line with the guideline
- liaise with the patient’s GP and other community support services
- ensure that patient and carers can get information, advice and support about diagnosis, treatment, palliative care, spiritual and psychosocial concerns
- meet with the patient in the early stages of the pathway and keep in close contact with the patient regularly by mutual agreement
- be an advocate for the patient at CUP team meetings.
- The CUP team should take responsibility for ensuring that a management plan exists which includes:
- appropriate investigations
- symptom control
- access to psychological support
- providing information.
- A CUP network multidisciplinary team (MDT) should be set up to review the treatment and care of patients with confirmed CUP, or with MUO or provisional CUP with complex diagnostic or treatment issues.
- Every cancer network should establish a network site-specific group to define and oversee policies for managing CUP. [Posted Jul 10]
For NICE press release with Q&A (26 Jul 10) see these documents:
and for a short published review by James & Symons click here.
Trial centres are now open for the start of this important multi-centre, Phase II trial which is being lead by Dr Harpreet Wasan – a Member of our Advisory Board.
The study, of which Jo’s friends is a stakeholder, is in two parts: the aim of the first (translational) part is to validate prospectively new tools in diagnosis (including molecular profiling, metabonomics of blood and urine for response and toxicity prediction and a proposed immunohistochemistry classifier). The hope is that, in the future, scientists can find a diagnostic indicator of the primary site simply, rather than subjecting the patient to a whole barrage of investigations.
Patients in whom a primary site is not identified can then be entered into the second (clinical) part of the trial. The trial may help in the future with selecting the best chemotherapy for patients with CUP. This part of the trial aims to establish the efficacy of the ECX chemo regimen (ECX stands for the drugs: Epirubicin, Cisplatin and Capecitabine) with and without Vandetanib (an inhibitor of different intracellular signalling pathways involved in tumour growth, progression, and angiogenesis). The treatment will last initially for 3 months and there will be subsequent monitoring. Patients can withdraw from the trial at any time without giving a reason.
Overall, this study should allow a more logical framework to be derived, both clinically and biologically, as to how highly metastatic cancers may be efficiently and economically investigated and managed in the future. It will be some years before the findings are available from this trial.
Trial centres that are now open are within hospitals in: Hammersmith, Glasgow, Aberdeen, Manchester, Doncaster, Sheffield, Slough, Peterborough, Poole, Kent. More details of the trial are shown here.
[Posted Jul 10]
NHS White Paper - Biggest changes since the NHS began
The Department of Health published the NHS White Paper, Equity and Excellence: liberating the NHS on 12 July 2010. Key points include:
- Putting patients first. “No decision about me, without me” will be the guiding principle for patient involvement
- The use of patient experience data and clinical audit will be expanded, giving patients greater information on which to make choices
- Choice will be extended to cover treatment options, choice of team and choice of GP
- Improving healthcare outcomes. A consultation on an outcomes framework will be launched shortly setting out quality benchmarks against which services should be commissioned and delivered
- Local authorities will provide local accountability and will be charged with ensuring greater integration across health and social care. PCTs and SHAs will be abolished
- All trusts will become foundation trusts
- GP consortia will assume the statutory responsibility for commissioning
- Cancer Drug Fund (Interim measure) to operate from April 2011 to support patients to get the drugs their doctors recommend
Management costs will be reduced by 45%, releasing £1 billion for patient care.[Posted Jul 10]
At first I was tired of answering the questions about CUP .. but this website has helped me realize that the world needs to know about us.
Your site is so great. I have a hard road ahead but feel calmer now that I've started a targeted chemo treatment. I fully understand the importance of your website and help.
Soon after my diagnosis my daughter contacted you and you were kind and gave her hope.
The Wilson family present money raised in memory of Tracy. Photo: The Mansfield Chad and Hucknall Dispatch.