The hidden challenges of a rare cancer diagnosis

Written by Linda Langdale

What does a rare cancer diagnosis feel like? Isolating? Confusing? Both of these and more! In January 2007, I was diagnosed with Granulosa Cell Tumour (GCT), a rare type of ovarian cancer – accounting for 2-5% of all ovarian cancers. I was given no information at the time, just that it could possibly reappear, but that was unlikely; if it did, it would be in 20-30 years’ time and require a simple operation. I wasn’t told it was an ovarian cancer, my staging, or whether I should see an oncologist. Of course, I went home and googled GCT, but everything referred to cancer, recurrences, medications, even chemotherapy, so I couldn’t align this with what I had been told, believing my diagnosis was something different. But of course, it wasn’t! 

My surgeon understood that GCT needed long-term follow up with a hormone-based blood test called inhibin. Inhibins A and B are secreted by the granulosa cells in the ovaries as part of the follicle stimulating process of the menstrual cycle and fluctuate accordingly. For women with no ovaries, levels should remain extremely low, making it a useful test to detect potential recurrences. Unlike CA125, the standard blood test for ovarian cancer, Inhibin is a more relevant marker for GCT. Inhibin tests are usually requested by a consultant, not a GP. 

Monitoring required inhibin tests every six months. With any cancer, you turn to your consultant and trust they understand and know what the test results mean. Sadly, this isn’t always the case. 

The first hurdle is the bloods have to be frozen at the hospital before being shipped to either Sheffield or Hammersmith/Charing Cross Hospital pathology laboratories. If the wrong-coloured tube top is used (some hospitals use red, others use gold), they get rejected. If insufficient blood is taken, again it is rejected; the bloods need to be assayed twice using the old ELISA methods instead of high-tech spectrometry (spinning the blood and comparing readings against known parameters). Then there is the indeterminable wait for results. Usually about six weeks, but frequently it can be 12 weeks or more!  

I would get my blood test done and about 6-8 weeks later would receive a letter from the consultant advising all was fine. No copy of the blood results, no numbers – just “all is fine.” Until June 2010, when the letter read; “Your inhibin b is 273 which is a little higher than expected. Please make an appointment to see me.” I can still remember reading that letter repeatedly, as I digested the implications of those few words. Was this a cancer after all? Did this mean I now had cancer? – it had returned?….and we all know what a recurring cancer signifies.  

Yes, it had returned and I was moved on to the care of the Royal Marsden. I have spent the ensuing 15 years fighting the beast - including 3 further major surgeries, 5 different medications and combinations of meds, 6 cycles of carboplatin/paclitaxel chemotherapy twice over, 3 different clinical trials, carboplatin/Caelyx chemotherapy and finally weekly paclitaxel chemo, which is my current regimen. 

A major part of my care has been monitoring inhibins, to see if any of the treatments were impacting my disease load. But how can you make decisions when results aren’t back in time for your clinic appointment? This is a common problem for those with GCT, and distressing when trying to determine if your cancer is returning, if a treatment is working or failing or whether a new course of action is needed. Many women travel long distances for their clinics only to be asked for another blood sample and to return 3 months later. Some have gone a year before getting an inhibin result!  

Results also need to be understood. Simple for post-menopausal women – inhibin b should be <10 and inhibin a <2. But often, patients are told their results are “fine” due to confusion between pre and post menopause levels. I was once told by a registrar at the Marsden that my inhibin b of 400+ was fine, when it’s actually really high and had climbed steeply since my previous test, indicating advancing disease. That was when I realised that self-advocating and understanding my rare cancer was really important to ensure that I was fully conversant with what was happening. 

I now run a closed FB group, UK GCT Survivor Sisters! The group is really supportive and informative, sharing information about inhibins, treatments and self-advocacy to ensure members are conversant with what to look out for. Knowledge is power, and many ladies have confirmed that this additional knowledge has empowered them to discuss their treatment and options more fully with their clinicians and feel more in control of their journey.  

Together with Ovacome, the Ovarian Cancer charity, we [UK GCT Survivor Sisters] are calling for laboratories to deliver inhibin results in a timelier fashion and for better training in interpreting results and delivering these to patients.