When your Cancer Diagnosis is as Eccentric as you are

Fleur Hull
8 min readMay 25, 2021

I thought I was super irreverent and bold until I met my Uterine Embryonal Rhabdomyosarcoma.

Warning: this post contains graphic medical images and uses words describing bodily functions and excretions

Turning up at my Jewish friends’ home for Shabbat in disco. Because, why not?

In August 2020, when the pandemic was going full-throttle and I’d written the first four chapters of Career after COVID-19, my period started up again. I hadn’t bled since the insertion of the Mirena IUD in 2015, a year after my son was born. The logical conclusion was that the hormones were fading out and it was time for a new IUD.

In mid-October, my super-smart and also hilarious Gynaecologist put a new Mirena in and noticed a nabothian cyst on my cervix. When I asked if that could be the cause of the bleeding she dryly responded, “Nabothian cysts are purely decorative.” Her letter back to my general practitioner noted, however, that adenomyosis might be the reason for further bleeding if any were to occur.

Adenomyosis, as it turns out, is the lesser-known cousin of Endometriosis. It’s the one where the endometrial lining from one’s uterus gets lodged inside the muscle of the uterus and can cause pain and bleeding each month as the lining grows and expands along with hormonal changes.

Drawings of female reproductive organs comparing uterus with Adenomyosis and Endometriosis (credit: Harpers Bazaar)
Adenomyosis vs Endometriosis (picture courtesy of harpersbazaar.com)

My (also awesome and down-to-earth) GP and I kept this in mind as the bleeding continued through November and December. The early weeks might have been explained by my body’s settling in to the new Mirena. By December, however, maybe peri-menopause was the reason. “Nope,” said my hormonal tests and so in February off I went for a pelvic ultrasound, which confirmed the adenomyosis, and also showed a smallish polyp growing near my uterine cervix.

The only real cure for adenomyosis is a hysterectomy, the surgical removal of the uterus. It’s a tough call for a younger woman with dreams of child-bearing, but a no-brainer for me. I have two delightful cherubs at the centre of my universe, and I JUST WANTED THE BLEEDING TO STOP!

Before my Gynae could finish the question, “How do you feel about a…” I told her, “Let’s get that uterus out.” She told me that she’d give me a quick check-up on the table with my legs in stirrups, to confirm the polyp was the small, mucousy mass indicated on the ultrasound, and then we could go ahead and book the hysterectomy surgery date.

As she put the speculum in and looked up towards my cervix, Dr Hilarious exclaimed, “Oh my God!” I didn’t know whether she was marvelling at my magical vagina walls or if there was something sinister afoot ‘down there’.

“That is the biggest polyp I’ve seen in my entire medical career! I’m surprised you haven’t been tripping over it.”

The Polypectomy

On St Patrick’s Day, 2021, my gynae prepared to remove the polyp under general anaesthetic, using a large loop excision of the transformation zone (LLETZ) to remove the cervical tissue. Because I was insanely curious about what had been bouncing around inside me, I asked Dr Hilarious to take a picture of the polyp before it was sent to the pathology laboratory. And because I’d been reading about the downtime after a LLETZ procedure, I asked my doctor when I could have sex again. Her whip-smart reply: “best to wait until you get home from the hospital this evening.”

Finally, after eight months, the bleeding had stopped. I was preparing to accompany my children to stay with their father in another part of Australia for the Easter school holidays, and was excited to be free of the blood burden. But then, five days after the polypectomy, there was blood again. My gynaecologist’s nurse assured me it was normal and that I should come to my post-op appointment after Easter, when I’d returned from interstate.

Two working days before I left, I was touched when Dr Hilarious personally called me to ask how my recovery was going. The phone had rung as I collected my children from after-school activities, planning to pick up fish and chips before heading home for dinner. I asked the kids to be quiet as I spoke to the doctor. Little F, my six year old son, asked, “Is that your vagina doctor, Mum?”

The out-of-body experience began as the chit chat about surgical recovery moved on to my casual enquiry about the pathology results. I kept a poker face in front of the children as I was told that the polyp was, in essence, a 6cm (2.3inch) malignant tumour. It was big but it was mostly out, I was told, and my prognosis was good. I grabbed a paper and pen to write down the name of the extremely rare cancer I had: Embryonal Rhabdomyosarcoma, as well as the appointment time for the following day. My gorgeous gynae was coming in on her day off so I could get the referrals for the oncologist, scans and blood tests before I left on my trip.

On with the show

The kids and I hopped in the car and I texted my partner, Big W, as I knew I couldn’t tell him in person when we got home, with the children within earshot. He’s got a medical background, and I was counting on getting his insight into this strange sarcoma when we had time to speak later on. I can still see myself walking into the fish and chips shop with the word ‘cancer’ sloshing around in my brain and heart, going through the motions of paying for the food and driving home, my thoughts racing and imagining how cancer might impact me and the people I love.

Big W greeted me with a huge hug when I arrived home with the fish and chips, and I was careful not to display excess emotion in front of the kids. When we finally sat down to discuss the diagnosis, I was surprised and confused to hear Big W ask which pathologist had looked at the slides of the polyp. His analytical and enquiring mind was questioning the diagnosis itself, because of the unlikelihood of such a finding in a patient like me.

The cancer diagnosis

When we met with my gynaecologist the next day, she confirmed that a senior gynaecological pathologist had looked at my slides, and that information, as well as seeing the photograph my gynae had taken of the polyp (pictured below, NSFW), convinced him that it was actually cancer. In a very strange way, it was a relief from the uncertainty that had messed with my mind overnight. I’ll write more about the feelings associated with a cancer diagnosis in another post.

The polyp (and IUD) that was removed from my uterine cervix and subsequently identified as adult embryonal rhabdomyosarcoma. The ruler measures distance in cm.

What is this rare cancer?

A quick google search will tell you that embryonal rhabdomyosarcoma (ERMS) is a rare cancer of the body’s connective tissue. The malignant cells resemble the primitive developing skeletal muscle of the embryo. ERMS tends to occur in the head and neck area, bladder, vagina, or in or around the prostate and testicles. Although rare as far as cancers go, it is the most common soft tissue sarcoma occurring in children. Adults are more likely to have faster-growing types of ERMS.

My tumour also shows some overlap with mullerian adenosarcoma, but at this stage my treatment plan is based on the major diagnosis of ERMS.

How rare is it?

My oncologist could only find seven other cases of this type of cancer in a woman of my age written up in medical journals. No doubt there have been others throughout history that haven’t made it to the journals, but for now I’m feeling like my tumour is as wacky as I sometimes like to be. It’s kinda cool, although the downside is there is not much data on what treatments work for a case like mine. I have to expect that I may be ‘over-treated’ because of the lack of evidence for any particular plan or protocol.

What causes ERMS?

Like many cancers, it’s hard to know what the genesis of my tumour is. ERMS tumours located in the uterine cervix may be caused by a genetic mutation called DICER1. The pathologists here in Perth have sent my slides to a world-expert in the DICER1 syndrome, based at McGill University in Canada. I’m also booked in to see a genetic counsellor to undergo blood testing. If the tumour and my blood results are consistent with the DICER1 syndrome, members of my family may be encouraged to undertake testing to ensure they are not at risk of developing ERMS.

If I don’t have DICER1, then the development of this tumour will be considered idiopathic.

Treatment and prognosis for my adult ERMS

I had MRI, CT and PET scans taken and there was no sign of major nodules or growths beyond my pelvic region. One month after the polypectomy, I underwent the following surgical interventions, conducted by my gynaecological oncologist:

  • Midline laparotomy and radical hysterectomy, which means my uterus and cervix was removed via open surgery with a vertical cut from my belly button down;
  • Bilateral salpingo-oopherectomy (removal of both ovaries and fallopian tubes);
  • Omentectomy (removal of my omentum, a flap of fatty tissue that hangs over the intestines and can sometimes be a magnet for cancer spread; and
  • Removal of three lymph nodes that were slightly enlarged.

Cancer cells were found in the lower part of my uterus and cervix, but fortunately not in the other organs.

The good news for me is that DICER1 tumours in adults have a relatively favourable prognosis compared with typical ERMS found in children. My cancer has been staged at 1A because of the high likelihood that most, if not all, of the tumour was removed in the polypectomy and radical hysterectomy.

The other great thing about having a crazy rare cancer is that the science and research about this is endlessly fascinating to me. It’s keeping my mind focused on this curious adventure, enabling me to observe the process without engaging too heavily in the painful thoughts around that big word, Cancer. No doubt there will be tough times and I’ll try to stay honest and raw about how this process affects me and those around me.

Today I commence chemotherapy and the medical team is still assessing whether I will also require radiotherapy. I’ll write more about the surgery and chemotherapy, as well as general thoughts on this journey, along the way. Thanks to everyone for their love and amazing support this far.

You can follow me on my socials, by heading to my Link Tree: https://linktr.ee/fleurification.

--

--

Fleur Hull

Marketing Manager for start-ups, author of best-selling Australian career guide. In 2021, I survived Embryonal Rhabdomyosarcoma, a rare cancer.