Rare cancers- who cares?
Note - updated on 9 June 2015: You may have seen yesterday’s headline Deaths from less common cancers on the rise, revealing the worrying increase in deaths from less common cancers. We initially brought your attention to rare cancers here in March 2015, which talks about what a rare cancer is and why research is important if hardly anyone has these types of cancer. We have also written about about forgotten cancers, why we should remember them, and how Worldwide Cancer Research is doing just that.
Just what is a rare cancer? And if they are so rare, why do we even need research. Should we not focus strained resources purely on the bigger killers- bowel, breast, or prostate cancer perhaps? Rare Diseases Day 2015 got us thinking about some of these questions, and here's what we found out.
A quick straw poll reveals slight confusion over the definition of a rare cancer. It’s definitely a type of cancer which affects very few, that much is sure. But how rare does it have to be- to be considered rare?
How rare is rare?
It seems the consensus is still out. In the US, a cancer is considered rare if fewer than 15 cases per 100,000 are diagnosed per year. In Europe it’s fewer than 5 or 6 cases per 100,000.* Compare this to over 100 males in every 100,000 in the UK who are diagnosed with prostate cancer or 125 per 100,000 women who will develop breast cancer.** That’s definitely more people, and that means much greater impact right?
But look closer. With least 198 different types of rare cancer so far identified, Rare Cancers Europe estimate that in total they account for over 1 in 5 of all cancers diagnosed every year. That's over 500,000 new cases in Europe alone. Not so rare now.
Another thing. With ongoing advances in science revealing the ever-increasing molecular complexity of cancers, what was once one cancer, might now be thought of as many different subtypes, all with different characteristics, treatment profiles, frequencies. What was a very common cancer becomes a collection of less common cancers. Think of breast cancer, and the many different subtypes we now know exist. Triple-negative breast cancer, for example, accounts for just 15 to 20 per cent of all breast cancer cases and has fewer treatment options than HER2 positive breast cancer, a more common type.
So perhaps it might be better to forget about statistics altogether- instead think about what rare cancer means to patients. Does it affect an unusual part of the body perhaps, is it an unusual type, does it require different or special treatment. But some might say this is still semantics.
In essence, no matter how they are catalogued or defined, there are some very definite and very similar challenges that many patients with rare cancers must deal with. According to Rare Cancers Europe, these patients often have a late or wrong diagnosis, and they can be faced with a lack of access to appropriate therapies and clinical expertise in their area.
And the reason boils down to knowledge. If it's rarer, chances are it gets less attention, less overall funding. In turn research progress can be slower, knowledge remains scarce and treatments slow to develop. Fewer patients can mean it's harder to run clinical trials, and a lack of commercial interest in developing new therapies.
And that's not all. The UK’s Rarer Cancers Foundation gets right to the heart of what all this means:
The most important feature of a rarer cancer is the fact that the patient feels isolated. There may be few survivors, or no available support network.
Ignore everything above, this statement alone shows exactly why we have to keep funding research into rare types of cancer, however we ultimately want to define them.
If you would like to find out more about our work with rare cancers
Search projects we are currently funding.
Read what some of our researchers recently had to say about their work in rare cancers.
* Statistics from Rare Cancers Europe website.
** Latest statistics from Cancer Research UK