Ellie from England - Dx Sep 2016 at 12 yrs old

[Olga]

If the pneumothorax happened in the lung apex, at the very top, it is most probably typical adolescent type one, just the lung tissue is to thin. If it is elsewhere, there are might be other sarcoma or treatment related reasons. We had both type of cases here. Post us what they found.

[Disco76lover]

Ellie had a VATS procedure yesterday to fix the pneumothorax. Before the op, the surgeon said he had looked at the scans and can see her lung mets are reducing. He advised they are tiny and wouldn’t be able to see them during surgery. He doesn’t think any of this is causing the pneumothorax.
The operation went well and the surgeon confirmed it was caused by a bleb at the top of her lung. This has been stapled and a talc added to the lung to prevent it happening again.
He said it is common in long, very thin people like Ellie. Hopefully now, her recovery will be quick. She is on HDU and being looked after by an excellent thoracic team as well as fantastic nurses. Thank God for our NHS.

Ellie’s oncologist is keeping her off cediranib for another week until she heals and then is putting her back on it.

[Olga]

Awesome news, good luck to Ellie to recover and stay safe during the virus outbreak, always wear mask in public spaces and wash your hands properly

[D.ap]

Melanie
That is great news.
Hugs to you .

[Disco76lover]

Thank you both. Ellie’s oncologist mentioned that it’s not uncommon for people to have to come off their meds for a couple of weeks but I just hope that it doesn’t affect how well they’ve been working. She doesn’t seem to think it will.

[Olga]

If the drug is working, it will start to work again as soon as she resumes, and I hope you have already refilled the meds for couple of months

[Disco76lover]

Hi Olga, the oncologist is wanting to start ellie back on the drug this week. We have been given our next three months worth of meds.

[Disco76lover]

Ellie had her 4 monthly lung scan in June. I am pleased to report that some of the lung mets have reduced, one can’t be seen anymore and one other may have very slightly grown. However, the oncologist said it could be due to Ellie’s breathing at the time, which is why it may look very slightly bigger. She is not concerned at all and is very happy with the scan. Ellies lung continues to heal from the unrelated pneumothorax.

This was a reassuring result, especially with Ellie being off the cediranib for 2 weeks and going through a lung operation for the pneumothorax.

We are back at the hospital at the end of this month for the 12 monthly brain mri scan. The oncologist has advised she is not concerned at all and is doing it to reassure me.

[D.ap]

Melanie,
Wonderful news!
So very very happy for you a Elle and family
.
Love,
The Pearson’s 💞

[Disco76lover]

An update on my daughters treatment. Ellie had her yearly brain mri scan today and I’m happy to report it was all clear. The radiologist has also reported on her ct lung scan in June. It was much better than we thought as the oncologist advised many of her mets have reduced quite a bit. One has cleared altogether and the rest have reduced on average by 1 or 2mms each. None of them are over 1cm. We are very relieved and happy that cediranib is still doing the job it needs to.

Ellie’s primary tumour in her calf was removed a couple of weeks after diagnosis in Sept 2016 measuring around 3cm. Full body and bone scans were done and all clear except for one spec on her lung. Fast forward three months later and both lungs had metastasis.
She started on cediranib in May 2017 and has seen her lung mets reduce.

[Olga]

Thank you for an update. Some of our patients had (and are still having) a very remarkable and long term sustained response to cediranib. Hoping for the same for Ellie. Sometimes the resistance may develop in single/separate mets only and they might be destroyed by the local treatments such as ablations.

[Disco76lover]

Thanks Olga.
Am I right in thinking that once the primary has been removed, the mets can’t reproduce? The ones that Ellie has in her body now, will have come from the primary and it’s these we are monitoring? With no primary, there can be no new mets, due to it being removed and it’s the ones she has that we are treating and closely monitoring?

So many thoughts and questions in my head!

[Olga]

Melanie, most sarcomas and especially ASPS disseminate very early. The cells enter the blood stream and float in single cells or clumps of few cells. Then they get trapped somewhere and attach to the surface, usually in the lungs small peripheral blood vessels - this is why the first mets in ASPS are usually in the lungs. They can stay dormant, sleeping for a long time. Then under the influence of the patient own growth factors, at the certain time they start to grown in - this is why after the primary surgery we often see the increase on the lung mets, as the surgical trauma stimulate the circulating grow factors in order for the body to heal.
It takes awhile for ASPS to grow its own blood supply initially this is why the small ASPS mets grow very slow, till the feeding system is in place (usually till the size is 20 mm, but in the brain it seems to go faster).
It is hard to say if there are already dormant mets in other organs disseminated before the primary was removed. This is why the life long surveillance is done to watch for the sudden wake ups. It could also be expected that the lung mets have the same propensity to disseminate as the primary, because the brain mets usually come after the lung mets in few years (if ever, as not all the lung mets patients get the brain mets). Cediranib affects the blood supply of the mets even small ones that might go under the detectable size so you can hope for no new active mets elsewhere to.

[Disco76lover]

Thank you Olga.
Please can you explain what you mean when you say cediranib affects the blood supply? I’m hoping that any mets that are undetectable, elsewhere in the body, are being zapped by the drug. 🙏😊
Thank you for sharing your knowledge Olga.

[Olga]

Cediranib is not the cytotoxic drug (does not kill the cancer cells directly) but makes it impossible for the dormant cancer cells to grow the blood supply, so they starve and die.
Cediranib is a potent inhibitor of vascular endothelial growth factor (VEGF) receptor tyrosine kinases.
This is what Canadian medical info site says:

Pharmacodynamics
Cediranib is a once-daily, orally available, highly potent and selective VEGF signalling inhibitor that inhibits all three VEGF receptors. The preclinical profile of Cediranib indicates that it has the potential to be the 'best in class' VEGF signalling inhibitor. Phase I data indicate that Cediranib is generally well tolerated, with the most common dose related adverse events being diarrhoea, hoarseness, headache and hypertension.

Mechanism of action
Cediranib inhibits vacular endothelial growth factor (VEGF) receptor tyrosine kinase (RTK). By forming a blockade at the VEGF receptors, Cediranib limits the growth of new blood vessels, which are essential to supporting tumor growth. Thus, lacking sufficient blood supply, tumor cells become starved for nutrients, slowing or halting growth and potentially improving the efficacy of other treatments. Preclinical evidence indicated that the drug had a high affinity at these sites, and was well tolerated and efficacious in animal studies.
https://www.drugbank.ca/drugs/DB04849