18 October 2014

CANCER - What is cancer of unknown primary?






When Jane Barrett was diagnosed with a common but little-
known cancer, she struggled to find information or support

What is cancer of unknown primary?
Ribbon days, pin days, moustache months – cancer awareness in Australia has never been higher. But imagine being diagnosed with the sixth most deadly cancer in the nation, only to discover there are no "days" for it, no specialists dedicated to its treatment, no support groups or networks. In short, your cancer is invisible, and so are you.

Jane Barrett found herself in this lonely situation when she was diagnosed with cancer of unknown primary (CUP).

People with CUP have been diagnosed with some form of metastatic cancer – a cancer that has spread from somewhere else in the body – but the primary cancer cannot be located, even after thorough testing.

The reason for this is not known. It could be because the body has already rid itself of the primary by the time of diagnosis; it may be too small to be seen; or the cancer may have spread very quickly. Because of the advanced stage of the disease at diagnosis, the median survival rate is a little under a year, although individual cases can vary considerably.

Barrett's symptoms first appeared about two years before her February 2009 cancer diagnosis, but they were so vague – a cough now and then, a hoarse voice, a tired throat – that when preliminary testing turned up nothing worrying, she dismissed them. However, after experiencing tiredness and shortness of breath while on a walking holiday in New Zealand, Barrett went back to her GP.

This time they did a fine-needle biopsy and a specialist consultant gave her the results.

Devastating diagnosis

"He said: 'You've got a metastatic neck cancer and from the cells, we can't tell where the primary has been'," Barrett says.

Not being able to identify the main cancer was a significant blow, because it is the primary that guides treatment.

"I just couldn't grasp how you couldn't have a primary," she says. "I walked out of there and I just didn't know what to do. It made no sense. There was no mention of the concept of 'cancer of unknown primary'."

Doctors removed 70 lymph nodes from Barrett's neck and discovered the cancer had also spread into the surrounding tissue. The major surgery, combined with six weeks of radiation treatment, left her with debilitating side effects, including tiredness, face and neck lymphoedema (fluid retention and swelling), loss of taste and partial numbness of her neck, head and shoulder.

Searching for answers

"It was only after radiation, when I started to feel somewhat better, that I started to look into CUP," Barrett says. "And I think then the desolation really hit me. In Australia, there was just nothing. No information or support. And I couldn't find others out there, like me, who had this disease. So there was this feeling of absolute loneliness and despair."

Drawing on her professional background as a health educator, Barrett began to pull together the slivers of information she could find.

The latest Australian Institute of Health and Welfare figures show that CUP is the eighth most common cancer diagnosis in the nation – the seventh most common for women and the ninth for men – with a total of 2897 people diagnosed in 2007. CUP is the sixth most common cause of death from cancer, following lung, bowel, prostate, breast and lymphoid cancers.

"So this is not a rare cancer. Its impact is huge," Barrett says. Through her research, she concluded CUP was a cancer defined by the word "lack".

She says she found there was a lack of a clear definition, of dedicated specialists, of research and clinical trials, of clinical guidelines and models of care, of patient information and support, of a voice for CUP sufferers, and of an understanding of CUP in the general population and the health sector.

No specialists

Gillian Batt, director of Cancer Information and Support Services at The Cancer Council of NSW, says that when Barrett approached the people at her organisation and told them her story, they too were stunned at the lack of information surrounding CUP.

"Quite often people are not even told they have CUP – they're just told: 'You've got cancer but we don't know where it started'," Batt says. "So they haven't got a label. They've got no club to belong to and there are no specialists. You usually end up being seen by a specialist for where your secondary cancer is presenting. It would be farcical if it wasn't so dreadful."

The Cancer Council has now produced a free patient booklet on CUP. It is also doing research with patients to find out what support is needed and there are plans to introduce online and telephone support groups.

A lack of specialists dedicated to CUP had also seen it slip between the cracks when it comes to medical research.

Barrett was overjoyed when internet contact with a CUP foundation in the UK finally put her in touch with Professor David Bowtell, head of the Cancer Genomics and Genetics Program at Melbourne's Peter MacCallum Cancer Centre.

Bowtell and the team at Peter MacCallum are tackling CUP on several fronts, including creating a diagnostic test based on the molecular appearance of the cancer cells, rather than where the cells are thought to come from. It is hoped the test will be generally available later this year. The centre is also developing a program of psychosocial support for patients with CUP and their families.

Bowtell says one of the lessons learnt from the very successful breast cancer awareness, research and support campaign of recent decades is the importance of patient input.

Barrett's involvement with Cancer Voices NSW, an organisation that ensures the voices of cancer patients are heard at the decision-making level, has now led to her involvement as a consumer representative for a new research project being undertaken at the University of New South Wales.

Associate Professor Claire Vajdic says the project will look at the diagnostic and treatment paths of people with CUP.

"It will provide information with which we can inform patients about what to expect," she says.

Back to the battle

After more than two years of being cancer-free, in April Barrett was diagnosed with cancer in her thyroid and the lymph nodes in the opposite side of her neck. Tests so far show the cells are non-specific. She is undergoing more surgery.

"The uncertainties of CUP, which never really go away, are back in full force. We need more research, support and information about this cancer," she says.
Source: http://www.bodyandsoul.com.au/

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