It's complicated.
Once again I have the odds stacked against me. My world turned upside down on Tuesday ; I received the pathology from the soft tissue tumour removed from my left thigh 3 weeks ago.
A rare and more aggressive malignant sarcoma was found inside a large but slow growing benign tumour , I have had that for many years. It was a shock ; even my Specialist was surprised. I guess no matter how prepared I was, it was still an unknown quantity.
In fact, my Specialist surgical oncologist (he is a Sarcoma specialist too) Dr told me the day before I was discharged - he didn't have the pathology results but it had all looked good and he didn't think there was anything to worry about.
Wrong.
This last week I’ve exhausted numerous articles and research results available on the Internet regarding my latest diagnosis Malignant peripheral nerve sheath tumour [MPNST].
WHILE it is rare 1:100,000 - do the maths - only approx 230 in Australia would be affected, it is more common in individuals with Neurofibromatosis 1(a genetic condition I was born with that affects 1:3000 births) with anywhere from 5-10% risk (so people with NF make up 50% of the subset of people who develop a MPNST).
Twelve months I noticed some changes in my thigh lump - more numbness and nerve pain. I'd just finished the 12 weeks of weekly chemotherapy, so I finally went to see my GP about it . I asked for and had an ultrasound. I was then referred to a local surgeon and I waited 3 months to see him - he sent me for an MRI and then a month or two later I went back for review.
HE said he wouldn't operate because it was too close to nerves & major blood vessels etc - he said there was no hurry to do anything because the MRI was fine - just wait to see my Sydney specialist Dr (Breast Cancer , sarcomas & benign tumours) .
It has taken almost a year to get to this point.
I missed seeing Sydney Specialist DR in November because Joel had his hernia repair the day before. I had to wait a further 2 months to see my Dr again because this DR was so busy. I didn't know the gravity of waiting.
Recently, I discovered through my own Goggling and reading that an MRI cannot always tell if these tumours contain malignancy. Two weeks after I saw my Breast cancer surgeon (originally for review of BC) in January - I was booked into hospital to have the thigh tumour removed and 2 other benign ones.
It is not related at all to Breast Cancer but as I mentioned it is associated strongly with Neurofibromatosis (NF1).
Though the margins (malignant tumour free tissue) were clear it was small margin. The tumour was identified as stage 2 (1 being best - stage 3 worse ; stage 4 means it has spread elsewhere in the body). There is so much more to it and I not sure what else to say about it, and it would waste too much of my energy trying to explain it .
I feel fine apart from residual swelling in my leg and an infection - where my body decided it didn't like the dissolvable stitch knot. The first antibiotics were not working so I was ordered stronger ones. Much improved.
The initial treatment plan - was to have 6 monthly, full body PET scans for 3 yrs and see my surgeon regularly. This has changed now and I'll have a CT scan in 3 months.
At this stage (stage 2) chemotherapy is not used (Lots of controversy over how effective it is vs the high risks) UNFORTUNATELY - they mostly use the same chemo I've already had so I cannot have it again because I've maxed out my lifetime's limit - it is so toxic and can cause heart damage amongst other side effects.
My Specialist mentioned radiation however his opinion was the risks of having radiation is higher and outweigh any benefits - than the risk of local recurrence (tumour growing back in my thigh) . He will be keeping a close eye on it anyway and if it regrew locally it would be surgically removed - eminently treatable. Still - rest assured I'm getting a second opinion about that from a Radiation oncologist. I have to for my own peace of mind.
Being in limbo is terrifying. I’m lucky to have love poured on to me by such a big community of people, (family and friends included), last time and now.
The risk of distant metastasis (Cancer spreading to other parts of the body [mostly the lungs]) is the same with or without radiation. It's tricky, nothing can predict when,where or whether it will spread or not. The risk is not so much immediate but mostly within 1-2 yrs, up to 5yrs (or more).
The prognosis has not even been mentioned. (I must stay away from Google).
Why ? because is never an easy question to answer.
My decision-making skills will be put to the test in the next few weeks.I already know the gravity of receiving a life-threatening diagnosis barely 21 months ago. Previously watching my husband go through it too - almost 4 yrs ago. He still needs followup for a further six years.
Anyway, I had more concerns so I asked my local GP for Chest CT scan on Thursday - to especially check if there was anything else I SHOULD know or worry ABOUT. Hoping, there was nothing to worry about and it would reassure me for the time being.
I took the form to Imaging Place straight away. The silver lining of having so many heart scans , ultrasounds, biopsies etc at the one place over the last 21 months - was they knew me well.I handed the form to the receptionist , without a word , she read it and looked at me and said
" I suppose you want this done as soon as possible"
First she said "Can you come back at 3.30pm ? (it was just after 12.40)" I said "Yes" - then a guy came out , she asked him something and said they would do it right away. I was whipped in there within 5mins, out in 15 mins.
The kicker - I picked up the results at 3pm and there were certainly some things to worry about - 3 different things that indicated a need for more investigations. I went straight back to my GP, however she has gone on a month's holiday. I was able to see another GP who ordered the ultrasounds immediately.
Friday, I had an ultrasound of my sternal notch - The Sternal notch can be found at the base of the neck in between the two clavicle bones. There was some kind of lesion seen on the CT near my thyroid /parathyroid . It may be a benign neurofibroma or something else. My Sydney Dr felt it was unrelated to breast cancer and MPNST - who knows ?? He said because I have NF I would likely have them throughout my body. Most likely I will need a biopsy of the lesion.
.
Monday, I am having a liver Ultrasound because they found one existing cyst, and 2 small unknown spots / lesions on my liver.
I also have some small nodules in/on my lungs. My Specialist said these should be nothing to worry about, lots of people may have them ...are you kidding ? I am more worried about these than anything.
The bottom line - my Sydney Specialist said - that the spots and nodules are too small to be defined or determined exactly what they are - even on a PET scan they likely wouldn't show.
PET scans can detect cancer in its early stages, help to monitor cancer treatment and check if the cancer is coming back. DR said (by phone)- I basically have to wait 3 months and see if they grow or change and have another CT scan / PET scan if they have.He tried to reassure me he would keep on top of everything.
It should be noted that with Breast Cancer (post treatment) they DON'T do any followup and routine scans unless you have symptoms.
Anyway, I made an appointment with a medical Oncologist , at The Oncology centre, for next week (they fly in/fly out from RPA , Sydney) to discuss all of the scans/ultrasound findings hopefully.
I know the
Every day I wake up and chose how I wish to live the day - in fear or with hope. I am just exhausted thinking about it all.
I not sure what else there is to say - of course I am terrified . The breast cancer history complicates it and so does the sarcoma and Neurofibromatosis. Fear is stopping me from feeling 100% confident with the doctors’ recommendations. Plus the rarity of MPNST.
I already had a sense that - this was not to be EASY. I can't dwell too much on the what if's. It would waste too much of my energy .
I need all my energy to focus on being positive and getting enough information to make informed decisions.
Right now I feel well apart from a lack of sleep , due to worry. My memory is shot.
I can't run YET (Dr's orders] but I am walking no problems at all. I paddled (dragon boating) 8km Thursday night and over 5km this morning. I have a lot more numbness and nerve pain/spasms in my lower left leg (the nerve associated with the tumour was related to my lower leg) but that was expected.
I feel grateful about a lot of things that went right this week despite everything awful , the kindness of people in my medical circle and elsewhere the last few days. The 3 different people/places who let me get my scans/appointments without much delay and that the Breast Cancer Support Nurse (who I paddle with too) was there for me and is going in to bat for me (I should say Paddle) . She is on my TEAM.
My husband is by nature a very calm and measured man.We have endured quite a few hurdles in our 24yrs. He remains grounded even though we have been rocked to the core with this news. He
I love the mantra it ain't happened till it's happened.
♥
