Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello everyone,

I'm new to this forum, my diagnosis is very recent.

It took me 4 month from initial complain about a lump on my forehead to ultrasound guided biopsy to get the diagnosis: right iliac fossa mass, ASPS, stage IV.

I'm 33 y.o female and I live in Vancouver. I've had a leg , and lower back pain for the past 4 years, I initially thought it's sciatica and was treated with steroids, Pilates and massage. I did the MRI only when I noticed that the lump on the head doesn't go away. Since then I had CT scans, MRIs, bone scan, and ultrasounds. Waiting for a PET scan next week.

My treatment with Pembrolizumab will start in a couple of weeks. No surgery scheduled yet.

Any recommendations / suggestions are welcome.

Cass

Hello Cass

Welcome.
As your dx’d is new I’m sure you have a whole host of questions and worries , about ASPS.
Be assured there is lots of information available , namely one that ASPS is typically slow growing . With that being said utilizing scans to keep tabs on the existing tumors and with that info planning on eliminating them.

Our family started our journey in August of 2012. Our son was 32, married with a 4 year old daughter. He is now 42 and has been given the opportunity of watching his daughter become a teenager .😊

Even in 2012, there was a lot of info to be utilized to help us in our decisions for various treatments of this incredibly rare sarcoma.
This forum was and has been a god send to us .

How big is the primary?
If I might ask , what does your team hope to find out with the PET scan ?

Hi Debbie,

This forum is very helpful, I'm glad I found it. I'm happy to hear about your son, I'm sure he is a great father.

Here is what was written in the papers before the retroperitoneal biopsy:

"A well-defined lytic lesion with an epicenter within calvarium of the right frontal bone is present. This lytic lesion measures 1.2 x 1.8 x 1.5 cm. The lesion is predominantly hypodense with foci of higher density/bone within it. There is a small soft tissue component extending out into the scalp soft tissues. There is no breach through the inner table of the skull or into the intracranial vault. There is no periosteal reaction, and no underlying fracture. No additional bony lesions are identified within the remain calvarium.

All Exams

Intracranially, the midline structures are central. The fluid-filled ventricles, CSF cisterns, and cortical sulci are of normal caliber for patient age. No mass, hemorrhage, or acute infarct is seen. The posterior fossa structures are unremarkable other than some dense calcification arising from the right tentorial leaflet near to the midline which has a benign appearance. The imaged paranasal sinuses, mastoid air cells, and orbital contents demonstrate no concerning abnormalities.

Abdomen and pelvis:

The liver enhances appropriately. The gallbladder, pancreas, adrenal, and kidneys enhance appropriately. No solid organ masses are detected. The spleen is a little prominent measuring up to 13 cm in longest dimension. There is fecal loading within the large bowel which is normal in appearance otherwise. The appendix and small bowel are normal. The stomach, and duodenum are unremarkable. The midline vascular structures enhance appropriately. No upper abdominal lymphadenopathy. No inguinal or pelvic sidewall lymph nodes.

A heterogeneous soft tissue mass is present arising from the right iliopsoas region which appears to arise from the iliacus muscle. It measures approximately 6.5 x 4.7 x 6.8 cm. On CT there does not appear to be definitive bony involvement of the iliac bone adjacent to this mass, and there are no additional soft tissue lesions. The mass does have some visible draining/supplying blood vessels in the retroperitoneum and adjacent soft tissues.

The diffuse marrow infiltrative process noted on the MRI is not readily apparent on this CT. There is however a lytic lesion present within the right L1 posterior vertebral body extending into the pedicle and transverse process. Additional small lytic abnormality is present within the left L4 vertebral body extending into the pedicle. A similar lytic lesion is present within the right posterior T8 rib.

No lung base nodules.

IMPRESSION: Dominant lytic bony lesion in the right frontal calvarium, as well as lytic bony lesions within the right posterior 8th rib, and involving the vertebra of L1, and L4. Suspicious soft tissue mass arising from the right retroperitoneum. A translated MRI report from Europe suggests abnormal bone marrow signal as well although this is not appreciated on CT. A primary retroperitoneal soft tissue mass with bony metastases certainly needs exclusion and is the most unified diagnosis. Differential considerations would include primary sarcoma, but also additionally lymphoma, vascular malformation and despite the young age myeloma/plasmacytoma. There is also the potential the bone lesions are a separate process with some considerations including hemangioma or LCH. An MRI of the pelvis with gadolinium may provide additional information. If deemed appropriate, the right retroperitoneal mass would be amenable to ultrasound/CT-guided biopsy."

Best regards,

Cass

Hi Cass,
as I understand the biopsy was taken from the suspected primary tumor in the abdomen, from the right iliac fossa. Who is your oncologist? Dr.Simmons is Ivan's oncologist there and we like her so far, and the decision to start Pembrolizumab is a very reasonable one. We had to fight to get Ivan treated by it 4 years ago off label, and it is still not approved for ASPS and I am proud to see that his case is now used as a precedent for the insurance to cover this treatment. It is like 12K a month.
We have extensively covered the possible ways to improve the response to immune checkpoint inhibitors, such as adding more insoluble fiber to your diet to improve the microbiome (we currently do not recommend the probiotics as they may actually negatively affect the richness of the microbiome you host now), moderate physical exercise to maintain and may be even increase the muscle mass, ensure that you sleep well and reduce stress if possible actively avoiding any unnecessary stressful situations for few days before and after the treatment. The example might be not scheduling any exams if there are some in your life, not planning any dental work or cosmetic procedures. Avoiding crowds where you can catch some seasonal cold. Trying not to get into potential allergic triggering situation and if you get an allergy to the pollen as some plants are starting to bloom, use the saline nasal spray to wash it out often. Dressing appropriately to the weather. All of this is aimed on supporting your immune system and trying not to derail the possible immune response after the brakes from the immune system will be taken down by the PD-1 blockage using Keytruda. If the treatment is still 2 weeks out, it is a good time to organize things around that.

Hi Olga,

Yes, you are right about the biopsy. Dr.Smrk is taking care of my treatment. So far I don't know about the costs. Thank you for pushing them to use this treatment. I sincerely hope it will help me too.

I'm still wondering what is considered as moderate exercise. I do Pilates 2 x week and freestyle swimming 2 x week about 1km. Is it too much or not enough? Do I need to be careful about cardio? Pilates activates lymph movement, is that ok?

I also came across the idea that Turkey tail helps to improve immune system. I have not asked my doctor about it, but would be curious to know.

Thank you for your advices, I really appreciate it. I've added more fiber to my diet and I'm trying to minimize the stress although I have to admit it it's been really hard considering what's happening in the Ukraine.


Best regards,

Cass

Hello again Cass,

The scan reports gave a very detailed description of all the maladies to watch from here on out . Scans are certainly our best tool in which to keep ahead of the game .

It’s interesting that there appears to not be any lung tumors ..
Which is good news . Which brings me to the the skull bump .

It’s good to note that not all images maybe ASPS, but should be treated as such in that it’s important to have a plan to eliminate them at their smallest size .

Was the biopsy sent in for a molecular
analyzation?

Here’s an article specific to findings of targeted proteins of ASPS, found during a group molecular study .
https://academic.oup.com/ajcp/article/1 ... 30/5102942
In thinking about the proposed PET scan, it appears that it can be used in immunotherapies to track infusion reactions? Inflammation?

Typically ASPS has a low metabolic composite so PETs don’t light up unless the tumors are of a significant size ..over 1cm maybe.

They are known typically,as an indolent sarcoma.

Having the PET scan scheduled gives them a baseline prior to starting Keytruda to compare to your reaction while on Keytruda, possibly.

Cass,
hi again. The war in Ukraine is stressful indeed but you should make a conscious effort to channel your anger into some positive actions, it helps.
re. If Pilates activate lymph movement - its excellent as you need the immune killer cells to reach all the areas of ASPS metastases.
re. If Turkey tail helps to improve immune system - some components of immune system are acting to protect tumors as the tumors use the signaling to use them. Therefore I am not able to say if Turkey tail is working at all or working to strengthen the immune cells sub-type that is beneficial to strengthen or otherwise, the sub-type that is needed to be blocked
re. If yours is a moderate exercise. Your training plan looks good, perhaps try to incorporate more outdoor activities to get more fresh air.

I was ask by my oncologist to stay away from turkey tail for the first 3 month, so that we have clear picture.
I was referred to fertility clinic. Never in my life I thought that I would have to go through egg preservation. It's hard to think about kids when you don't even know you life expectancy.
I was shocked and terrified by the amount on the hormonal injections that needs to be done. It postponed my ASPS treatment by a week.
Very stressful. I hope it's worth it.

Hello Cass,

Thank you for sharing a most difficult situation that you are dealing with . My heart goes out to you .❤️

We as patients are truly not sure of ASPS’s response to hormones , whether it’s hormone driven or hormone suppressed .

Was your biopsy pathologically studied ? For molecular composition?

Not knowing how immune therapies and or future treatments can affect a woman’s body , I’m sure you’ve made the right choice a this juncture.

Do continue to boost your immune system through diet and exercise to reach a good bases for your body to work with the Keytruda.

Love ,

Hi again Cass,

If I might ask, was Human chorionic gonadotrop used in your procedure ?

Hi Debbie,

Thank you so much for your support.
Yes, they will be used. I will need to do shots of Gonal F 225, Menopur 225 and after the first week I will need to add Cetrotide+.

Cass

Cass,
I am not sure how all of this fertility boosting treatment fits into immunotherapy treatment. Are you planning to have kids any time soon, or ever? The eggs harvesting is usually done before of the cytotoxic therapy as it can damage the fertility, but if the immunotherapy damages the ovaries is not known (at least I am unaware of that). Some of our patients decided not to have kids. I mean it is ultimately up to you to decide, if you want to go trough this.

Surprisingly I found a very decent article that is out of there that discussed the issues of getting pregnant and immunocheckpoint inhibitors.
they say - I quote:

Although such a strategy could be pursued in a metastatic setting, it is better to avoid a delayed therapy initiation in favor of a fertility-preservation strategy, especially in high-burden disease.

Checkpoint inhibitors, fertility, pregnancy, and sexual life: a systematic review
https://www.esmoopen.com/article/S2059- ... 6/fulltext

please read it carefully and discuss with the oncologist and your family. As I understand, there is not enough data re. issues esp. in women, more info re. men problems.

Hi Olga,

Thank you for the article. I've decided to go ahead with egg preservation. I hope it will only delay the treatment for about a week.

Going for MRI and PET scan tomorrow.

Best regards,

Cass

Good luck with the scans and with the procedure, stay strong. I will now move your topic to the area where are the patients updates located, to keep it organized.