One Friday morning in early May, 17 years ago, I was rushing to get to work as usual. Trying to avoid the daily unflattering sight of myself in the bathroom mirror as I emerged from the shower, I could not avoid my attention being grabbed. Seemingly overnight, a large growth had emerged on my left chest wall. It was hard to the touch. Not good, I thought, carrying my habit of understatement into my private unspoken realm.
I have a genetic condition called neurofibromatosis type 1 (nf1). Nf1 causes tumours called neurofibromas to grow along the soft tissue surrounding the nerves. The tumours themselves are usually benign but even so can cause problems. They rarely become cancerous – each person with nf1 has an 8-13% chance of neurofibromas undergoing malignant change. When this happens, it is called a malignant peripheral nerve sheath tumour (MPNST) and is a type of sarcoma. They are also known as neurofibrosarcomas. MPNSTs can occur in people without nf1 but the lifetime risk is 0.001%.
I had read that prognosis is often poor following an MPNST diagnosis. Knowing this, I made my way to work and when my GP surgery opened, I phoned them; I got an appointment the same morning. The GP I saw phoned the oncology department at a local hospital whilst I was still in the consulting room. I was given an appointment on the spot for the following Tuesday afternoon. The speed of response was simultaneously impressive and terrifying.
An oncological odyssey
What followed was a rollercoaster odyssey of short-lived relief, followed by escalating jeopardy, monumental treatment decisions, adaptation, acceptance and survival.
The first scans and biopsy indicated that the tumour was benign, but at the next appointment the consultant qualified this, saying there was no malignancy “that we’ve found”, and that the tumour was in a “very awkward place”, rendering surgical excision potentially riskier than leaving it in place. A period of ‘watchful waiting’ followed.
A couple of months later I was hit one night with severe pain. I had to get up and walk to a nearby petrol station to buy painkillers at around 2am. Later that morning I went to Manchester for Pride weekend. There was nothing realistically I could do over a weekend so focused on enjoying the weekend and parked contacting the hospital until after the celebrations. A possibly ill-advised combination of joyously camp but lethal cocktail shots on Canal Street, paracetamol and ibuprofen gave me some distraction from the pain.
The hospital continued to favour conservative treatment due to the risks of surgery but when the pain returned and became more persistent, I asked if a referral elsewhere might be advisable. I was referred to a peripheral nerve surgical team in London and the tumour was successfully removed, a year after that first GP appointment. The histology report confirmed that the 15cm mass removed was a high-grade sarcoma, an MPNST.
I was followed up in London initially where radiotherapy and chemotherapy were proposed. I asked the first clinician I saw about my prognosis. “Are you sure you want to know?” he asked. “Yes!” I confirmed. He said that we do see some sarcoma patients surviving for ten years now but close surveillance would be necessary to detect any recurrence or spread as early as possible.
MPNST tends to metastasise to the lungs so the first of many chest scans was done. I was away on holiday when I got the results via a phone call with the consultant. The report has come back “not normal” she said, as I was standing on a pretty west Wales beach, the weather, at least, benign. “There appear to be growths in your lungs” I was told, “they may be a normal part of your condition, but there’s a larger one we’re concerned about, we need you to come back in to agree what to do next.”
Calm before the storm
My follow up care was transferred back to Birmingham, where I live, ongoing treatment in London being impractical. Thankfully a further scan showed no sign of the large growth on my lung, its fleeting appearance a mystery to this day. A course of radiotherapy to help prevent local recurrence followed, scheduled daily first thing in the morning so I could get to work straight after. I had no side effects and chemotherapy was discounted as there was no real evidence for its effectiveness in my circumstances.
I had regular follow ups in the months that followed, and all appeared to be well. That is until that damn bathroom mirror grabbed my attention again. A small lump had appeared on my chest wall close to the operation site. It was the weekend, so again I had to wait for a couple of days, during which time I saw four more lumps appear on my chest and upper left arm. These were swiftly excised and confirmed as MPNST recurrences. Within weeks of this surgery a further lump had appeared. Things were about to get more radical.
Your arm or your life
I was presented with two options: to have my left arm, shoulder and a significant section of my chest wall amputated, giving around a 10% chance of survival; or no surgery and chemotherapy in the hope of giving a little more time waiting to die. My decision was instant; the surgery it would be.
A full body PET scan showed that there was no clear evidence of metastases or further local recurrence and so the surgery went ahead, now nearly two years after that fateful first encounter with destiny in the bathroom mirror. The procedure was, to say the least, not routine. It was a collaboration between orthopaedic, oncology and thoracic surgical teams. I gave my consent for it to be filmed for training purposes.
It went well. Nearly two weeks later I left the hospital to start a long period of recovery and adaptation, staying with a wonderful friend to whom I am forever grateful. I say long, but I did insist on cooking us both a meal from scratch within a week or two of my discharge – cooking is one of my big pleasures in life and I was determined not to let that go.
Thank you NHS
From that point forward, follow up scans and examinations showed that all was well. The only set back was a local recurrence that showed up four months after the amputation and was immediately removed. Finally, nearly 13 years after the first tumour, I was discharged. It had been more than ten years since the last tumour, and I was considered cured.
I was asked to fill in a discharge feedback form, barely A5-sized, as I remember. I gushed praise as concisely as the space allowed, handed in the form and got the bus back to work.
In its 75th anniversary year, I cannot thank the NHS enough. The brilliant human beings who demonstrated such mind-blowing skill, dedication, compassion, and care ensured I am alive today. I know and accept it’s still possible that further problems can occur, especially given my nf1 diagnosis.
For now though, all appears to be well; I’m still surviving sarcoma, so far. I can’t ask for much more than that.
If you, or someone you know is affected by nerve tumours and needs support, please contact Nerve Tumours UK.
