Partner - 30 years old - likely a rare pleural sarcoma

Hi 

I've kept an eye on this forum for a number of years. Partly due to my own cancer scars (turned out to be nothing) and the fact my father has lived with prostate cancer for a number of years now. 

You are good people. From those diagnosed to family members, I've seen so many posts filled with grace and love in a way I couldn't even summon on my best days. I send you all "internet stranger" love.

But unfortunately, the time has come to make my own post. 

My partner, my fiancé, my entire world since we were 14, has been told she has cancer at 30 years old. 

My partner received a diagnosis of sarcoma, likely liposarcoma (to be confirmed) on Tuesday.

We don't know for sure yet whether it is primary or secondary, but the surgeons and consultants are currently working on the idea that this is a rare manifestation of sarcoma in the pleura. 

5 weeks ago she was doing heavy work around the house and hiking up a moorland, without a bother in the world. 

fAew days later she suddenly had back/shoulder/chest and arm pain. We rushed to A&E thinking heart problems, only for them to find a large left pleural effusion and a white out on x-ray, initially suspected to be a localised fluid buildup because of how sarcoma tumours are often quite low density. Unfortunately they tried to take fluid samples for testing, but in hindsight we now know they were just stabbing her tumour unfortunately, which was very painful for her. 

We were referred to another hospital 5 days later where a wonderful consultant said he wanted this seen to ASAP, and walked up to a lung surgery ward in person to secure a bed and slot for surgery.

She had surgery the next day (12-13 or so days after it all started). Chest drain, part of her lung removed, and discovery of a suspected "bloody and crumbly" tumor mass which was almost all removed through VATS, but not in full due to being "close to an artery" and the surgeons "not really knowing what they are dealing with yet"

750-800ml of liquid drained from her, and I slept in hospital for 2 nights because I couldn't go back home and leave her.  Remarkably she was home within 3 days. She recovered extremely well from surgery and was out breathing my asthmatic self within 5 days!

2 week wait and then we got the biospy results. 

We now await PET scans to know if this is primary or secondary, or whether it has spread, lung function tests and MDT meeting. It's all moving very quickly, which is reassuring.

The surgeon is "hopeful" if the cancer turns out to be a rare case of primary pleura sarcoma that he can operate with full removal. They are talking about being "aggressive with treatment" which is scary and reassuring in equal measure.

My partner is a particularly brave, stubborn and headstrong women - I'd argue she's dealing with this diagnosis better than me if anything. So at least we know she's got a good start should she decide to fight this aggressively.

I don't really know what I'm hoping to get out of this post, other than getting this off my mind. I feel I need to "tell someone" even if no one is listening, if that makes sense. 

Lots of love, luck and care to you all. Thank you. 

Parents
  • Update - weird day, not sure what to think. 

    The bad news:

    They've managed to identify the type of liposarcoma - it is exceptionally rare. Is in case reports in literature can be counted in single digits rare.

    Primary pleural Myxoid pleomorphic liposarcoma 

    It is highly aggressive in case reports. Recurrence and metastatic in more than 75% of reported cases. But as the surgeon stressed and my data analyst head says - fewer than 20/30 people having this diagnosed ever reported isn't really enough to draw a conclusion. 

    There isn't anything in the way of a defined treatment for this. Surgery and completely resection is still the gold standard but they don't seem to know if adjuvant (chemo/radio) is useful.

    Because she had a pleural effusions there is a likelihood of tumour seeding now, but the surgeon and oncologist wasn't even convinced that has high likelihood for now.

    The good news:

    No change in chest x-ray. 

    PET showed no evidence of metastases or cancer elsewhere outside of the tumour site. 

    In fact, while the PET scan did show some "lighting up" at the sight of the tumour it didn't even strongly suggest malignancy.

    The surgeon suggested while unlikely, it might be the case that what did highlight on PET could be benign post surgical scaring and the tumour has already been removed.

    CT scan still suggests it's operable.

    Right now the MDT and surgeon are still working with curative intent. They believe full resection of any remaining tumour remains possible.

    Surgery will be tough due to the location. It'll be a case of thoracic, cardio, neuro and plastic surgeons all working together. And she will likely be sent straight to ICU afterwards. She's tough though, she'll be alright with surgery.

    We managed to get an MRI today which will hopefully confirm it is operable, which is good.

    We're probably looking at surgery in 2-3 weeks now. 

    Chemo/radio afterwards will be explored and from her perspective, we will probably push for it because the potential results outweigh the negatives of "wait and see". 

    So really not sure how to feel right now. No metastatic disease was good news, ultra rare cancer and medical staff scratching their heads is pretty concerning though.

    All the best everyone 

Reply
  • Update - weird day, not sure what to think. 

    The bad news:

    They've managed to identify the type of liposarcoma - it is exceptionally rare. Is in case reports in literature can be counted in single digits rare.

    Primary pleural Myxoid pleomorphic liposarcoma 

    It is highly aggressive in case reports. Recurrence and metastatic in more than 75% of reported cases. But as the surgeon stressed and my data analyst head says - fewer than 20/30 people having this diagnosed ever reported isn't really enough to draw a conclusion. 

    There isn't anything in the way of a defined treatment for this. Surgery and completely resection is still the gold standard but they don't seem to know if adjuvant (chemo/radio) is useful.

    Because she had a pleural effusions there is a likelihood of tumour seeding now, but the surgeon and oncologist wasn't even convinced that has high likelihood for now.

    The good news:

    No change in chest x-ray. 

    PET showed no evidence of metastases or cancer elsewhere outside of the tumour site. 

    In fact, while the PET scan did show some "lighting up" at the sight of the tumour it didn't even strongly suggest malignancy.

    The surgeon suggested while unlikely, it might be the case that what did highlight on PET could be benign post surgical scaring and the tumour has already been removed.

    CT scan still suggests it's operable.

    Right now the MDT and surgeon are still working with curative intent. They believe full resection of any remaining tumour remains possible.

    Surgery will be tough due to the location. It'll be a case of thoracic, cardio, neuro and plastic surgeons all working together. And she will likely be sent straight to ICU afterwards. She's tough though, she'll be alright with surgery.

    We managed to get an MRI today which will hopefully confirm it is operable, which is good.

    We're probably looking at surgery in 2-3 weeks now. 

    Chemo/radio afterwards will be explored and from her perspective, we will probably push for it because the potential results outweigh the negatives of "wait and see". 

    So really not sure how to feel right now. No metastatic disease was good news, ultra rare cancer and medical staff scratching their heads is pretty concerning though.

    All the best everyone 

Children
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