C.U.P – Cancer of unknown primary, is the term used when cancer cells are found in the body but the place where the cancer started (the primary site) cannot be located. In most cases the primary cancer is never found.
A C.U.P. diagnosis means that the primary cannot be located even after thorough tests. The person is diagnosed with some form of metastatic cancer, i.e. cancer that has spread from somewhere in the body. Lack of a primary has profound implications for treatment and ongoing management.
Cancer is a group of more than 200 different diseases. Each type of cancer has different risk factors, such as getting older, poor diet, smoking, excessive alcohol consumption and being obese. These may play a role in C.U.P.
C.U.P. is not a rare cancer. In 2007 C.U.P was announced as the eighth most common cancer in Australia (seventh for women and ninth for men – 2897 new diagnoses) and the fifth most common cause of cancer death for both – 2,334. Relative five-year survival between 1998-2004 was 9.1% compared with breast cancer 87.7% and prostate cancer 85.3%.
There are nearly 3,000 new cases of C.U.P diagnosed each year in Australia. It is more common in men than women and the risk increases with age.
C.U.P. patients are made up of many different cancers. The patients all have different medical histories, different lifestyles and different outcomes. Yet we are all in the same group of ‘cancer of unknown primary’. We are now a member of the ‘C.U.P.’ club, whether we want to be or not.
Current cancer treatments rely on the identification of the primary cancer to target treatment to specific tumour types. Cancer is named after the site where it began, i.e. lung, pancreas etc. This is called the ‘primary’ or main cancer. If the cancer spreads to other parts of the body it is still referred to by the site of the primary, i.e. lung cancer or pancreatic cancer and the distant cancers are called ‘secondaries’ or ‘metastases’.
Just like most people when they are told they have cancer, I was scared and my head went into a spin with all the questions running through my mind. I felt even more stress not knowing where the original cancer came from. I found it very difficult to accept that my primary cancer could not be found. We can put men on the moon, yet the doctors cannot tell me how something so life threatening can invade my body, do damage and leave, without a trace!
Cancer was explained to me as a disease of the cells, which are the body’s basic building blocks. Our bodies constantly make new cells to help us grow, to replace worn-out cells and to heal damaged cells after an injury.
I believe that patients diagnosed with C.U.P need specialist advice and support at an early stage. This should be given by medical staff who are well informed about C.U.P. and understand the sensitive needs of C.UP. patients. The tests and treatment options that follow the diagnosis need to be thorough and extensive and performed without delay. C.U.P. patients feel alone and isolated because they are told they have a cancer that cannot be explained or identified. This can be difficult to live with
Before you see a doctor it may help to write down your questions. Taking notes or recording the discussion can also help. Many people like to have a family member or friend go with them to take part in the discussion, take notes or simply listen.
You may want to ask for a second opinion from another specialist. This may confirm or clarify your own doctor’s recommendations and reassure you that you have explored all of your options. Some people feel uncomfortable asking their doctor for a second opinion, but specialists are used to patients doing this. Your specialist or general practitioner can refer you to another specialist and send them your test results. You can still ask for a second opinion even if you have started treatment or still want to be treated by your first doctor. Knowledge is powerful!!
Although C.U.P is a relatively common cancer, some people with C.U.P feel there is less support and information available for them as it is not known as well as other more talked about cancers such as lung, breast, prostrate.
Normally cells grow and multiply in an orderly way, but sometimes something goes wrong with this process and cells grow in an uncontrolled way. This uncontrolled growth may result in a lump called a tumour or may develop into abnormal blood cells.
A tumour can be benign (not cancer) or malignant (cancer). A benign tumour does not spread to other parts of the body. A malignant tumour is made up of cancer cells. The original organ or tissue where a cancer began is called the primary site.
C.U.P. can be treated using chemotherapy, radiotherapy, surgery and hormone treatment or a combination of these. Treatment will depend on factors that are unique to your situation.
Research by the Cancer Institute NSW on C.U.P. concludes that the ‘findings in their study support the premise that unknown primary cancers are a specific cancer rather than the absence of a primary site. C.U.P. remains poorly understood. More research is required to begin to unravel its complexities for the long-term goal of achieving better outcomes for patients’.
The following terms will be used throughout your cancer journey.
– Biopsy is the most important test. Many different tests are used to diagnose C.U.P. by taking a tissue sample.
– Scans and tests can help your medical team decide on the best treatment plan.
– The stage describes the size of the cancer and how far it has spread.
– Prognosis is a general prediction about what may happen to you. (No one can predict the exact course of your illness).
– Multidisciplinary team refers to the many health professionals who will work together as a team to treat you.
– MRI (magnetic resonance imaging) scan. This test uses a magnet and radio waves to take detailed pictures of an area of the body.
– A CT scan is able to detect and localize changes in the body structure or anatomy, such as the size, shape and exact location of an abnormal growth, a sizeable tumour or a musculoskeletal injury.
– PET Scan (positron emission tomography) scan uses low-dose radioactive glucose injected into a vein to measure cell activity in different parts of the body. A scan is taken a couple of hours after the injection. Areas of cancer usually absorb more sugar than surrounding tissue and show up on the scan.
PET scans provide information to help physicians:
• Locate the site of the cancer
• Determine the size of the tumour
• Differentiate benign from malignant growths
• Discover if the cancer has spread
• Select treatments that are likely to be appropriate
• Monitor the success of therapy
• Detect any recurrent tumours
A PET scan is very different from an ultrasound, X-ray, MRI, or CT scan. A PET scan allows the physician to distinguish between living and dead tissue or between benign and malignant disorders. Since a PET scan images the biology of disorders at the molecular level, it can help the physician detect abnormalities in cellular activity at a very early stage, generally before anatomic changes are visible.
• Combined PET/CT Scan
Positron emission tomography (PET) and computerized tomography (CT) are both state-of-the-art imaging tools that allow physicians to pinpoint the location of cancer within the body before making treatment recommendations. The highly sensitive PET scan images the biology of disorders at the molecular level, while the CT scan provides a detailed picture of the body’s internal anatomy. The PET/CT scan combines the strengths of these two well-established imaging modalities into a single scan.
Alone, each imaging test has particular benefits and limitations but by combining these two state-of the-art technologies, physicians can more accurately diagnose, localize and monitor cancer, as well as heart disease and certain brain disorders
Symptoms of C.U.P. are different for each person and will depend on where the cancer is found in the body, where it be the primary site or where the secondary cancer is found. Some people with C.U.P have few or no symptoms; others have a range of symptoms that may include:
• shortness of breath
• bone pain and/or back pain
• swelling and discomfort in the abdomen, feeling sick (nausea), fluid collecting in the abdomen (ascites)
• yellowing of the skin and eyes (jaundice)
• swollen lymph nodes in the neck, underarm, chest or groin
• looking pale, feeling tired and breathlessness due to a lack of red blood cells (anaemia).
You may also have general symptoms such as unexplained weight loss, no appetite or feeling extremely tired.
Staging means determining how far the cancer has spread. This information helps doctors plan the best treatment for you. Staging systems are different for every type of cancer. For most cancers there are four stages. The stage is worked out according to the size of the cancer, growth into nearby organs, and spread to lymph nodes or other organs in the body.
Stage 1 is an early cancer and stage 4 is an advanced cancer.
With C.U.P, the cancer has spread beyond the primary site and must be at least stage 2 or higher.
Prognosis means the expected outcome of a disease. The doctor most familiar with your situation is the best person to discuss your prognosis with, but it is not possible for any doctor to predict the exact course of the disease.
Although most cancers of unknown primary can’t be cured, treatment can keep some cancers of unknown primary under control for months or years. In other cases, treatment can relieve symptoms such as pain and help to improve quality of life.
Treatment can help control the symptoms of C.U.P but it may not cure the disease.
There are many types of treatment for C.U.P. including chemotherapy, radiotherapy, surgery and hormone treatment. Your treatment will depend on the type of cancer cell, the suspected primary site and the location of the secondary cancer(s). It will also depend on your general fitness.
Chemotherapy treats cancer with drugs. Most chemotherapy drugs cause side effects, such as nausea, vomiting, fatigue, hair loss, mouth sores, loss of appetite, diarrhoea, and increased risk of infections. Side effects depend on what kinds of drugs you are given.
Radiotherapy treats cancer by using x-ray beams to kill cancer cells. It is painless but radiotherapy may cause burning on the skin, scarring or shortness of breath. Other side effects include tiredness and nausea.
Surgery may be used to remove lymph nodes in the neck, underarm or groin.
Hormone treatments are sometimes used to treat C.U.P. by slowing the growth of the cancer or shrinking it.
The three main forms of Carcinoma cancer are:
• Adenocarcinoma – Most people with C.U.P. have cancers that develop from glandular cells, which are found in many organs of the body. Common primary sites for adenocarcinomas include breast, colon, prostate, stomach, pancreas, liver and lung.
• Poorly differentiated carcinoma – There is enough detail to tell that the cells are cancerous but they may look too abnormal to classify further under the microscope.
• Squamous cell carcinoma – This is my type of cancer!!!
These cancers look like the flat cells that are normally found on the surface of the skin or the lining of certain organs. About 5% of people with CUP have squamous cell cancers. Common sites include the head and neck area, skin, oesophagus, lungs and cervix.