What do we want?
Overall in the UK, despite the incidence of some cancers increasing, people are living longer as a result of improved treatment. This is not the case with CUP (cancer registration data suggest some 13,000 -14,000 cases and approximately 25,000 "Hospital Episodes" of CUP each year in the UK). In some cases Cancer is becoming a controllable chronic condition: this is not the case with CUP.
CUP is a little known, under-researched phenomenon yet it is undeniably one of the ten most common causes of cancer mortality in the world.
We believe CUP mortality to be under-recorded in the UK. It is likely that about the same number of people die from CUP in the UK as from breast cancer (some 12,000 each year). In England and Wales it is the 4th commonest cause of cancer death.
Table based on 2008 data published by CRUK which, with the exception of CUP, show UK mortality data (UK overall =156,000).
- Lung 35,261
- Colorectal 16,259
- Breast 12,116 - 10,692 - England & Wales
- CUP - 10,585 - England & Wales
- Prostate 10,168
We need to break this Catch 22 where the prognosis for CUP is poor because the research needed to improve treatment is not happening. Advances in treatment are dependent largely on benefits from research into linked areas (e.g. metastasis); and advances in equipment such as MRI scanners.Why is there an absence of CUP research? The short answer is because there is insufficient demand to make it happen. In the UK we have seen the impact on breast cancer as a result of popular demand. Resources and research have been triggered as a result of demand and survival rates have improved dramatically. CUP offers an even bigger challenge with an even bigger reward: it is possible to make the unknown known. We want to stop CUP being the unspoken orphan of the cancer world.
Jo’s friends aim to raise public awareness and understanding of CUP, in order to trigger demand for research and improvements by biologists, chemists, geneticists, histopathologists, diagnostic radiologists, physicists, medical engineers and others working in cancer-related fields. In particular, we want:
- Wider recognition of the unique problems for patients facing CUP. To have an unknown cancer creates added psychosocial problems for patients and their loved ones. Patients with less common cancers report worse experiences in their pathway than those with common cancers. CUP patients need additional support through their cancer journey when sometimes the tendency is to sideline them as a medical embarrassment.
- An improved patient pathway through the implementation of the NICE Guideline and Peer Review (audit) Measures. Until the advent of the NICE Guideline on CUP in July 2010, patients faced very different approaches in different cancer centres in the UK. Oncologists are site, not problem-specific, specialists which means that the initial patient referral may be incorrect requiring further referrals. As a result, patients may face yet more referrals and unnecessary tests. This "ad hoc" approach is time consuming and increases the patient's uncertainty and concern. Whilst continuity of support from a specialist nurse – sometimes called a Systems Navigator in the USA - is usually available to a known cancer patient, the CUP patient can often be overlooked whilst awaiting a definitive diagnosis. An improved pathway will identify treatable patients earlier and reduce CUP mortality; for those where a cure is impossible, a recognised pathway will allow palliative care to "kick-in" earlier and unnecesary tests can be avoided.
- Specific CUP – related research. We know next to nothing about the biology of CUP. Until we understand the biology of a disease it is very difficult to treat as the biology determines the treatment. Because the unique characteristic of CUP is its unusual metastatic pattern (when compared with the pattern for known primaries) it seems possible that CUP represents a specific biological entity. If this is the case, by understanding the genetic profile of CUP it can be treated more effectively. We need also research into patients, carers and clinicians to identify how to manage CUP patients appropriately.
- The condition known by one name! Without one commonly accepted name, it is difficult to get the disease taken seriously. Presently it is captured under various names such as occult cancer, metastatic malignancy of unknown origin etc. Without one name, incidence and mortality will not be correctly attributed. Without one name, it is less likely to appear in "league tables" which impact on research funding. We want Cancer of Unknown Primary to be the only term used.
- Improved gene understanding. As part of biological understanding, we need to know more about the genes, molecules and chemical messengers of Cancer to target treatment (chemotherapy, particularly, lacks precision) . Geneticists are working on: manipulating faulty cells to repair themselves or stop cancer from spreading; stopping or “arresting” cancer cells as they spread through the pathways of the body (not necessarily killing dividing cells but stopping them at “checkpoints”). Better understanding of genes may help make cancer cells more sensitive to established cancer treatments while making normal tissues more resistant to chemotherapy. 'Gene expression profiling' offers a new technique for cancer diagnosis and prognosis which may help with the identification of primary sites. Hopefully this will lead shortly to a point where the diagnostic "read-out" will prompt clinicians to say: this is the pattern of the cancer which we can treat precisely, rather than search for a primary.
- Sequencing research. A step beyond profiling is Sequencing. It is now possible to take a sample of a person's cancer tumour and read its genetic code. We know that cancer occurs through genetic changes. Sequencing involves reading out the entire genetic code of a cancer genome. Understanding the molecular faults in CUP tumours will allow scientists to understand more about CUP and to tailor treatment specifically to overcome particular faults. Whilst this may well be the future for cancer treatment at the moment it is in the research stage - we need CUP sequencing research.
- Better diagnostic techniques. Better diagnostic techniques are needed to interpret and access the smallest of cancer lesions. Improved PET/CT, & MRI scanners are needed that can peer even more effectively into our bodies and hopefully identify primary cancer sites. This is the work of diagnostic radiologists, (nuclear) physicists and engineers. But however sophisticated this becomes there are parts of the body e.g. the pancreas which are inherently difficult to image.
- Better epidemiological data through better recording of CUP patients. The true incidence of CUP is understated because there is little agreement on its definition and clinicians are keen to assign a primary where possible. The coding for CUP used in hospitals and cancer registries is imprecise. It is based on coding at arbitrary time points in the diagnostic pathway.
- Better statistics. Arguments about funding and treatment priorities tend to be won by the evidence. In the CUP field the statistics are ambiguous and unreliable. We need to be able to quantify, and make distinctions between, metastasis of undefined origin, provisional CUP and confirmed CUP in Hospital Episodes Statistics that provide the source data for Cancer Registries.
- Publication by CRUK, NCIN and other opinion forming organisations of CUP data in the cancer "league tables". CUP is the 4th commonest cause of cancer mortality in England and Wales yet this fact is sometimes “hidden” in national statistics. It is difficult to argue for research funding – often related to incidence/ mortality by research funders, pharma etc. when CUP does not appear in the "league tables". Sadly, CUP is in the top 10 cancers for incidence and top 5 for mortality. In 2011 CRUK and NCIN included CUP for the first time. We must ensure that these data remain recognised. If we look at other countries CUP is featured. In Australia there is an excellent publication Cancer in Australia an overview 2010 published by the Australian Institute of Health and Welfare http://www.aihw.gov.au/publications/can/56/12138.pdf . Looking at this one sees in Australia CUP incidence as the 9th commonest for men and 7th for women in 2007 with overall mortality as the 5th commonest in 2007. It is interesting, and of concern, that the Australian statistics include a projection of CUP showing an increase in Incidence and Mortality for 2010.
- Clinicians and researchers to specialise in CUP. The medical profession tends to resist change and perpetuate orthodoxy. As Siddhartha Mukherjee in "The emperor of all the maladies: a biography of cancer" states: the lesson of the last 150 years in oncology is that orthodoxy was repeatedly wrong. As more clinicians become aware of CUP, following the NICE Guidelines, we need to encourage specialist medical expertise in CUP, as opposed to generalist knowledge. Better pathology is needed to recognise and validate molecular profiling for CUP and more targeted therapies need to be researched for this diverse group of patients.
A papyrus from ancient Egypt in 1500 B.C. indicates that cancer was treated by cauterization to destroy tissue with a hot instrument. Surface tumours were surgically removed much as today. Source: about.com.cancer.
Join others affected by
CUP to share information
& gain support in a moderated forum
Ron on the 130 km Etape Caledonia. With son Jimmy they raise £2,000 for Jo's friends through JustGiving