Told that she had cancer, Karen Lomas quite naturally wanted to know where it had started. Did she have breast cancer, or perhaps lung cancer?
The trouble was doctors could not tell her, because Karen, 52, is one of the many thousands in this country who develop cancer of unknown primary (CUP) each year.
To put it simply it means the cancer has no obvious starting point — instead it appears as a secondary cancer, most commonly found in the liver, lungs and bones.
Because they can’t identify the type of cancer the CUP originated from, it can be hard for doctors to tailor the correct treatment — hence only 16 per cent of those diagnosed with CUP are alive after a year.
Because they can’t identify the type of cancer the CUP originated from, it can be hard for doctors to tailor the correct treatment
In fact although few people have heard of it, according to the latest available figures CUP causes more than 10,000 deaths in the UK each year, making it the fifth biggest cause of cancer death.
Karen, a mental health occupational therapist from Cambridgeshire, first noticed something was wrong in late 2012 when a small swelling developed on the right-hand side of her neck. To begin with she thought nothing of it.
‘But a couple of months later, I caught sight of it in the mirror and realised it was quite prominent,’ she says.
‘I had no pain, but I thought I’d better get it checked out.’
Her GP was concerned enough to arrange a swift referral and in January 2013 she underwent a mammogram, ultrasound, CT scan and a biopsy of the lump.
‘Two days later, they confirmed that the swelling in my neck was a secondary cancer in the lymph nodes there, but that further tests would be needed to find out where it had originated.’
Secondary cancers (or metastases) occur when cancer cells spread from their original site and lodge elsewhere in the body.
Using blood tests and by studying cells under a microscope, doctors can tell whether the cells match the tissue in which they’re found or if they’ve travelled from elsewhere.
They are still the same type of cancer, no matter where the new tumour is growing – so, for example, breast cancer cells that form secondary tumours in the liver are still classed as breast cancer.
Karen, a mental health occupational therapist from Cambridgeshire, first noticed something was wrong in late 2012 when a small swelling developed on the right-hand side of her neck
‘Although I was obviously worried about the cancer diagnosis, I wasn’t overly concerned that they didn’t yet know what type of cancer it was,’ says Karen.
‘I just assumed that more tests would reveal it so that I could start treatment.’
Karen went back for a battery of further tests in an effort to track down the original site of the cancer, including a thyroid ultrasound as it was the nearest organ, another biopsy and a full-body MRI and a PET scan, which uses small amounts of radioactive drugs to trace cancer cells activity.
But the original source was nowhere to be found. ‘That’s when the first mention of “cancer of unknown primary (CUP)” was made,’ says Karen.
‘I was filled with disbelief that, even after having every part of my body scanned, they couldn’t find where the cancer had started or come from.’
A CUP diagnosis is given when, despite rigorous tests, the primary tumour that led to secondary cancers still can’t be found with certainty.
‘You can identify, say, breast cancer cells in the lung as they match the biopsy from the site of origin,’ says Dr Harpreet Wasan, a Consultant and Reader in Medical Oncology at Hammersmith Hospital, Imperial College London.
‘In the case of CUP, we have only a single biopsy from the organ that has the secondary cancer and nothing to compare it with.’
It is still not known for sure why the primary tumour can’t be found in these cases, but there are several theories.
’It may be that the initial starting collection of cancer cells are tiny (and so undetectable by any scans) and spread quickly around the body, rather than growing locally first into a tumour or lump before spreading,’ says Dr Wasan.
Karen says she was ‘filled with disbelief’ after the diagnosis. She has had cancer twice and battled it with chemo and radiotherapy
‘Another theory is that the initial tumour grows so fast, it can’t maintain its nutritional needs where it starts and so has to migrate to get nutrients to survive, making its origin hard to trace.’
CUP can occur in people of any age but is most common in the over 75s.
All cancers have specific treatment programmes such as chemotherapy, but when you don’t know what type of cancer it is, you can’t tailor a treatment.
So doctors end up using a chemotherapy that has a wide application, and can be used to treat a number of different cancers.
‘In addition to this, secondary cancers are always hard to cure, even when we know where they originated, as they are generally more aggressive and eventually unresponsive to treatment,’ says Dr Wasan.
Dr Wasan, with the help of CUP teams throughout the UK, has just completed a nationwide trial, examining outcomes on patients to assess which chemotherapy drugs are most effective, and is trying to identify the best test for finding where the cancer started, such as genetic profiling of the tumour.
Another issue for those with CUP is that whereas there are specific medical staff or support staff for breast cancer, say, or prostate cancer – for CUP often there isn’t.
All cancers have specific treatment programmes such as chemotherapy, but when doctors don’t know what type of cancer it is, they use a chemotherapy that has a wide application
‘There’s a woeful lack of specialists to help people cope with the concept of the unknown diagnosis,’ says Dr Wasan.
Guidelines from The National Institute for Health and Care Excellence (NICE) say that every hospital with a cancer unit should have a CUP team, including a CUP specialist nurse. However, Dr Wasan says this is not always the case.
‘It’s a distressing and confusing diagnosis and patients need clear explanations and specialist support to understand that this condition is relatively common,’ he says.
The condition also has few survivors or champions to fundraise for more research, ‘so it’s a neglected area,’ he adds.
When Karen began her first round of treatment there was little in the way of specialist help available.
‘Aside, from my doctor, the charity (Jo’s Friends, the only designated charity for CUP) was my only source of help and information,’ she says.
‘I didn’t have a specialist nurse, as I didn’t fall into any cancer category.’
She was started on a broad range chemotherapy called ECX.
‘I had to start treatment as the cancer in my neck was growing,’ says Karen.
Illustration of lymphocytes attacking a cancer cell. One issue for those with CUP is that there often isn’t any specific hospital staff or support staff to treat the condition
Twenty sessions of daily radiotherapy followed and it seemed to have done the job. ‘It had destroyed the lump and I appeared to be clear of cancer everywhere else,’ says Karen.
‘I had scans of my torso every three months to be sure.’
But in June 2015, Karen started vomiting. Her GP suspected a stomach bug and gave her rehydration drinks, but when she was still vomiting two days later, she was sent to AE.
A chest X-ray and ECG (a test to measure the electrical activity of the heart) showed that she had pericardial effusion (a build-up of fluid around her heart) and she was taken into hospital to have it drained off through a tube in her chest.
‘I felt instantly better when the fluid was drained off,’ says Karen.
‘However, the fluid was sent off for testing and it came back showing that it was cancerous and had been caused by a secondary cancer in the lymph nodes.
‘I was told that I would need more chemo urgently.’
We have small amounts of fluid in many body systems, such as around the heart and lungs, acting as a lubricant explains Dr Wasan.
‘But cancer can clog up the system — the fluid can’t flow freely anymore and builds up.’
A routine scan in April showed that the cancer has come back again, and this time, it appeared in her stomach as a small tumour. Karen is now in her third round of chemotherapy
Karen’s chemotherapy was much harder this time round.
‘I was sick a lot and developed terrible blisters on my feet so I couldn’t walk. I was off work for over three months, having never taken a sick day in 20 years.’
The treatment worked again —there was no sign of cancer anywhere in her body — and Karen returned to work in January this year.
But a routine scan in April showed that the cancer had come back yet. This time, it appeared in her stomach as a small tumour.
‘I’ve had more tests and biopsies on this latest recurrence and it’s still not revealing where it’s originally from,’ she says.
Karen is now in her third round of chemotherapy.
‘I’m past caring where the cancer has come from these days – I’m now more concerned with being treated successfully and managing my symptoms.
‘The doctors have been amazing, but more research needs to be done — the diagnosis is not only hard on the patient but also on the doctors because they want to be able to treat you as best they can.’
Cancer of Unknown Primary Foundation