Raising the profile of Granulosa Cell Tumours with the Survivor Sisters!
By RareCan Team on 19th January 2023
RareCan Member Linda was diagnosed with Adult Granulosa Cell Tumour in Jan 2007. Given the rarity of her cancer diagnosis, she is passionate about creating awareness and believes that research is needed to establish more targeted drug options, rather than having to piggyback breast cancer or prostate cancer drugs. As Linda says, ‘You need to be in it to win it!’
Having a rare cancer, which you quickly learn that your clinicians don’t know much about it either, can be very isolating and leaves women feeling fearful and alone.
So what is Granulosa Cell Tumour? Generally referred to as GCT, it accounts for around 2-5% of all ovarian cancers. It is one of the few cancers which actually starts in the ovaries rather than in the fallopian tubes. It has two specific subtypes, Adult and Juvenile and although most are separated by age at diagnosis; eg Adult >30 and Juvenile <30, this is not always the case. GCT is often diagnosed in stage 1 and has a good prognosis for 5-10 year survival. It’s considered a slow growing and indolent disease, with a habit of recurring many years later and so requiring long term monitoring. The prognosis for recurrent disease is much poorer with around 80% eventually succumbing to recurrent disease.
While there are several support charities for ovarian cancer, such as Ovacome, Target Ovarian Cancer, Macmillan etc, their main focus and membership is often for high grade serous, the most common, and deadly ovarian cancer.
GCT is rarely suspected pre-surgery, often because CA125, the standard blood test for ovarian cancer, is not a marker for GCT and will show within or close to normal limits, and also because imaging needs to improve to give clearer pictures of the ovarian cysts or masses. For younger women in particular, they are often misdiagnosed with IBS, until they present at A&E with acute symptoms. Diagnosis is generally from histopathology post-surgery and is a surprise to all involved! Sometimes further staging surgery is needed, especially if the “cyst” was cut or ruptured during initial surgery.
Many Gynaecologists will never have seen a case of GCT or maybe only seen 2-3 cases in their working years. They then rely on outdated information which describes GCT as low malignant potential and unlikely to recur for many years. Women will be signed off from care post-surgery with no further follow up and are often never referred to an oncologist.
There is currently no standard of care or follow up for the varying stages. While stages 1C upwards are a higher risk for recurrence (around 40%), this is not generally understood.
Surgery is considered the mainstay of dealing with recurrences with GCT known not to respond particularly well to chemotherapy, due to its slow growing nature. Endocrine (drug) therapies are more widely used now, but usually to maintain stable disease rather than prevention or cure. Currently, recurrent disease is not curable.
Because GCT behaves very differently to other epithelial ovarian cancers, it is often excluded from clinical trials and Pharmaceutical companies are not so invested in trials for low numbers of participants. Too many of our women receive a poor standard of care due to lack of information and no clear guidance on the disease. Research is needed to establish more targeted drug options, rather than having to piggyback breast cancer or prostate cancer drugs. Even new medications for ovarian cancer are not generally available to women with GCT as they weren’t included in the initial trials, so we have no way of knowing if those could potentially be helpful. It feels as if every step or treatment option has to be fought for, with many of us coming to our medical oncologists with information gleaned from our GCT FB groups to ask for specific treatment options to be considered.
I spotted a post on Facebook from RareCan, which caught my eye as they were looking for people with a rare cancer diagnosis to register with them. While at that point I didn’t fully understand what the end game was meant to be, it showed enough to encourage me that RareCan was looking to assist research into rare cancers.
Once I had spoken to the team from RareCan about their work, it was particularly encouraging to find that they were working on both sides of the tracks, so to speak. RareCan are in contact with researchers and pharmaceutical companies who are interested in setting up a trial or a piece of research into a rare cancer.
Those with a rare cancer diagnosis can register with RareCan, helping them to show the researchers that there is access to numbers, which is key for research and trials. I particularly liked that they also help screening potential participants and provide support through the sign-up process, which can involve lots of paperwork and questions!
While there is no guarantee that we will immediately get access to a trial or new drug for GCT, by being members of RareCan, could very well help our cause to progress this area – you need to be in it to win it!!
The hope is that if we can encourage as many women as possible with GCT to register with RareCan, in conjunction with pressure from our medical oncologists, they can make the link between us and trials for potential new drugs or for investigating the use of currently available drugs for ovarian cancer, which have not been previously available to GCT patients.
There is some research currently happening and our FB group is working with Hudson Institute of Medical Research in Melbourne, Australia. If they are successful in finding potential drugs for GCT, then I would hope that RareCan will be able to assist in getting trials set up in the UK for our group members.
We have set up a closed Facebook group purely for women in the UK and Ireland called “UK GCT Survivor Sisters!” where only people within the facebook group can see the activity within there. We welcome all ladies with a diagnosis of Granulosa Cell Tumour, whether recent or many years ago. Any mother of a child under 18 with GCT (usually juvenile GCT) is also welcome to join. We currently have close to 180 in our membership.
We are also linked to a worldwide group called “GCT Survivor Sisters!”, with members from all corners of the world, currently numbering just over 1800! The groups are extremely supportive and informative with a great deal of information sharing of treatment options and new ideas. Only women with the diagnosis are allowed to join and screening is an important part of the admission to the group. With no other family members or randomers included, it’s a really safe environment to discuss personal issues and voice fears or concerns, which we all have from time to time.