Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi all
Our family has taken 2 regiments thus far for Joshua's brain tumors and to date have been pleasantly surprised at the side effects or let me say the lack of side effects.

However please approach with caution as any chemo can have its late effects and or have inside biological effects on the patient.

Even though ASPS is slow it is a tuff cancer to kill--as are most cancers and to date there isn't a lot of information on what works on ASPS


http://scienceblog.cancerresearchuk.org ... ozolomide/

Hi all,

Joshua's neuro ablation was completed without any neurological issues on April 1st. No, really on April 1st

We've scheduled a Mri for the 27th to view the results.

Joshua has had to be on continuous doses of dexamethasone since December 18 of I believe 12mg a day broken down into every 4 or 6 hours of course then ending on 2mg a day by April 1.

We began with 24 mg for 4 days and are currently on 6mg? We hope to end after 22 days or before on 2 mg.
So far the symptoms of the neuro ablation with every of decrease dose has been uneventful and we hope to keep going that route.

Also in February and March , prior to the neuro ablation procedure in hopes of keeping further metss from appearing in the brain. They were finished on March 17th. We took 250 mg of temador for 5 days each month .

Our Ct scan of the chest was performed on April 8th and I am sorry to say that the lung tumors are progressing in spite of our 2 courses of temador.
Granted we are on an oral fairly tolerable medicine and I am thankful for that. Also we have been on only 2 doses as well.

The largest of our lung tumor at this moment is 2.6cm x 3.1cm. Up from 1.8 cm x 2.0 cm in October . I am trying to consolidate the measurement of the other tumors to report as they have followen off the current report as Josh has over 150 notable areas to see as a result of the lung surgeries in Germany. Also not having those 150 tumorsat I am so thankful for

Temador has thus far been studied with brain cancers and clinical trials on the lungs.

Apparently some tumor types of cancer have been known to be " protected " by the dexamethasone so as to prevent the chemo, Temador , from entering the cancer cell and performing the DNA destruction.
The protection with dexamethasone begins as the immune system is suppressed /compromised and then consequently the dexamethasone spars grow in the process of "helping the body " to combat swelling in our case . Some cancers or possibley the variety of ASPS Josh has , can then take advantaged and grow. We may of been taking care of micro mets, again speculate, but it is time to take care of the larger tumors as they are the real issue now.


The growth talk is all speculative of course and as we have found out on Friday our news, we are spending the weekend figuring out which way to go from here and which questions to ask what doctors .
We need the steroids for another 10 days or so and we are due to take another dose of Temador on Monday night.
We will be talking to our Neuro surgeon and getting his angle on an accelerated DECREASE in the dexa and talking with our Oncologist who is in charge of the chemo to what are choices would be Monday.

The good news is both doctors are in charge and on our most current board of doctors in Joshua's care and talking continuously to each other.
From the get go we have been the search and destroy family and we mean to stay the course as long as humanely possible with our doctors hopefully on board.

Also we will be re-approaching an ablative doctor to see if that is an option.

The growth of the lungs leads us to believe that the environment or combination of drugs needs to be stopped so as to discontinue other unwanted growth(s).

Will update when able.
In the mean time please include us in your prayers for guidance and healing .

Got to go . Grand daughter awakening from sleep over. This grandma NEEDS to go play and spend time with her before parents want her back. Josh and his wife will NEED her later today too.

Till next time

Much love
Debbie and family

Questions and thoughts needed

Drug interactions and tumor progression
Trying to wrap my mom brain around the summary of this section as it mentions the hepatic region but I know the liver plays a big part in any medicine intake?

http://www.cureasps.org/forum/viewtopic ... d+pitfalls

Deb, it looks like there might be a drug interaction between dexamethasone and Temodar (temozolomide is generic name) (or other chemotherapy drugs) decreasing their potency - may be there is but it is not certain. But unfortunately it is absolutely necessary to decrease the brain swelling after the brain surgery or LITT ablation. Failure to decrease the swelling could cost the patient his life or damage his brain causing loss of function and disability. I have not seen any published information re. Temodar being an active chemotherapy agent for ASPS or other sarcomas except gynecologic leiomyosarcomas, and our group experience with it is very limited.

Hi Olga
Thanks so much for the response. I truly have NOT found , thus far, any documentation of an APS patients having taken temador to date .
We know that with the advent of imaging and chemo there will be something soon.

So here we are as a testament for possible success .

Found this article to begin to dissect temador and its possible benefits on ASPS.

http://www.chemocare.com/chemotherapy/d ... S2vT0ko6Uk


Let me reflect where we are with positive tissue biopsied after surgical removal, saying we have ASPS .

8/31/2012--primary left leg, thigh
Mitotic rate of 6/10, tumor over 6.5cm DX ASPS

January 2013- over 70 tumors
August 2013--over 70 tumors

Germany biopsy of tumors detected positive for ASPS

August 2014--Left lung showed 20% increase of tumor to over 2cm. If Asps then vascular "highway if you will" could of been established.


To date with no biopsies to document ASPS traveled other than from the primary to lungs.
Had a virus on Thanksgiving? Coughing fits low level and then 3 weeks later brain MRI revisited showed 3 lesions 1.1cm, 4 and 5 mm on brain detected only as a shadow in August?

August 2014-December 18. Brain lescions 1.1cm,4 and 5mm

December 2014-- 3 lesions in brain found with MRI contrasted

Regardless of DX we encountered swelling and slight neurological issue and those are life threatening whether cancer or not.

Comments and questions requested

Love
Debbie and family

Josh writes back in 2013


"My new onc had my excised primary tumor from last Sept. sent to Caris Target Now in Arizona for analysis, and the report came back with the following "agents of possible benefit":
*fluorouracil, capecitabine, pemetrexed
*gemcitabine
*temozolomide, dacarbazine"


The article

To be discussed

Temodar
http://www.chemocare.com/chemotherapy/d ... S2vT0ko6Uk

* "•Treatment of anaplastic astrocytoma and glioblastoma multiforme (GBM) brain tumors"

* These conditions in the brain are, as I understand it, primary brain tumors.
They begin in the brain to grow and must be dealt with aggressively if they can't be surgically removed with good margins.

So they , the metastic tumors, are surviors of the bodies defenses and get past the brain barrier to reside in the brain and grow.
They are tough to combat and are stage IV aggressive and SHOULD not be taken as a wimp tumor??

Joshua's brain lesions, as I like to refer to them, went from 4mm to 1.1cm in 3 /12 months Two others popped up on right side of 4 and 5 mm

To update everyone

Josh has completed another doseage of Temodar April 13 thru April 17 of 250mg a day
We've managed with very little nausea side effects while still taking zofran as antinausea aid

Weight is still good at 130lbs

Still on 2mg of dexamethasone till after MRI

We are scheduled for an MRI this Thursday along with our consultation with our neuro surgeon to see how successful the Neuroblation was back on April 1.

We are in hopes that the Temodar has kept the right side stable as well as the hoping the "ill defined" images on both the right and left side of Joshuas brain are staying that way.

Will update when able
Well wishes and love to all

Debbie

Our brain metastasis to SRS to temador journey began in August 2014 after a followup brain scan was performed from our baseline scan of 2012 in which the scan of the indicated bilateral lescions. Possibley MS.
We missed our follow up appointment from the August scan and finally matched up schedules with the neurologist on December 17. Her report from the radiologist made no mention of shawdow in the August report, however her eye was able to see "a shawdow". The followup scan showed a 1cm on left side and a 4 and 5 mm on right side of Joshua's brain. We were off and running to find answers and treatments.

This write up explains the course we choose as we had to come to some agreement on brain treatment as the left side looked to be in a very dangerously situated place in the Joshua's brain. Josh could very well come out of surgery paralysed.

A tumor that had grown from " a shadow to 1.1cm in a little over 3 1/2 months was not to be ignored.

We were told by the neuro surgeon that the left side was in a very dangerous place.
So our decision was to try SRS, instead of whole brain radiation or surgery, on the 3 tumors.The one on the left side was 1 cm and the right side tumors were 4 and 5 mm .
All in 3 treatments. 30 gy over 3 fractions/ treatments.* 10 gray x3

We had been strongly advised ,prior to brain mets ,to get on some kind of systemic treatment as Joshua's lung tumors had grown over 20% from 2013 to 2014. A tumor analysis in 2012 of the primary, had indicated that Josh was a good candidate for several chemos including temador. Temodar was a good candidate for possible micromets of the brain because it crosses the brain barrier. We were hoping that it would serve for the lung tumors as well.

Combining stereotactic radio surgery and systemic therapy of Temodar for brain metastases: a potential role for temozolomide explains our management plan set forth by our medical oncologist-

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3414728/

On one hand it was suggested that we perform SRS along with temodar but we held off treating the brain tumors with systemic treatment for over 40 days AFTER SRS.


August 31,2014 MRI shadow on left side

December 18,2014 ---1.1 cm left side,5 mm and 3 mm right side MRI showed severe swelling. Began dexamethasone *

December 24,26 and 29--a total of 30 fractions with 10gy on each day.
Early morning circadian rule of treatment
http://www.cureasps.org/forum/viewtopic.php?f=8&t=557

January 29th-- 1.3cm MRI, 4 weeks post SRS

February 16-- 250mg of Temador x 5 days- first dose--

February 28- **1.6cm -MRI, 8 weeks post SRS
** There is a possibility of a pseudo image suggesting progression during the Mri where tumors appear enlarged. This is when SRS and Temodor are combined and or are concumbant treatments are performed. We had added temodar rather than combined the two

March 13------ 250mg of Temador x5 days- first dose--last dose March 17

March 24 ----- 1.3cm MRI

April 1-- Neuro ablation of left side tumor- in at 11ish out and awake by 4 the same day
April 1 -- bumped dexamethasone up to 24mg
April 2-- home and walking

April 13- 250 mg Temador x 5 days-first dose--last dose April 17
April 13 - dexamethasone 8mg
April 27 - dexamethasone 2mg till MRI on May 7th

Its important to note that Temodar from day one of 250mg has on days 7 -10 nadir effect and on days 22-28 it looses its effectiveness and cells are starting to repair once again. Both the good and bad cells.

Nadir: Meaning low point, nadir is the point in time between chemotherapy cycles in which you experience low blood counts.

Onset: none noted
Nadir: 7-10 days
Recovery: 22-28 days

* dexamethasone and temodar possible reaction /creating substandard effects on cancer tumors by combining the two.

http://www.cureasps.org/forum/viewtopic ... 1009#p7763

To follow
May 7th--MRI

Hi all

As you may or may not be aware of, Joshua's main brain tumor treated by SRS, temodar and neurablated has been declared on May 7th as dead for the lack of the report in front of me.
Looking at the MRI with contrast gave me, my husband ,Josh and his wife a "guarded " but great sense of relief as we saw the interior of the tumor darkened from the procedure of over 30 days ago and the vein that fed the large tumor disappear.

The ill defined images on the right side SRS treated 4 and 5 mm lesions and the ill defined image next to the left side neuro-ablated 1.5cm tumor all were no longer lightening up and were almost diffused into the back ground.
The 2 right side unofficially are reduced by 2mms plus!

The ill defined could of been radiation fall out? We are CC our radiologist oncologist and asking for his input as this really is important to document for future ASPS pateints.

We've been discussing an additional drug to add to our Temodar up stage treatment if you will, by a confident ,to see if we can over the next month reduce Joshua's largest tumor in his left lung before we possibly enter the ced/sutent trials if accepted that is..

Oh by the way we've discovered a 25mm lesion on Joshua's liver with the extended chest CT-- Our next journey

Since our recent MRI's results, we can now reapply to the trial and so here goes the parallel journey.. No rest for the weary. but incredibly happy family for todays blessing.

You guys, folks who are waking up facing your day with this cancer rock to say the least. Staying strong. Never give up. We love you lots!

Good night
Debbie


Wanted to leave us all with the thought of possibly going from Temodar to a TKI, Nexavar if we proceed with one more dose of Temodar and the lungs and liver and the brain aren't players with the current Temodar clan on the 24th of May and June 18th?



http://www.ncbi.nlm.nih.gov/pubmed/22987955

Deb, sorafenib is a less active/different type of the TKI drugs. There is no information re. its efficacy in ASPS - from what we observed with our ASPS patients it is rather inactive. Isn't taking sorafenib going to preclude Josh from the cediranib clinical trial enrollment?

You are absolutely correct Olga.
Thanks for the reminder.

Hello all

Quick up date.
Josh is in the process of taking another course of Temodar, 5 days at 250mg.
He has recently experienced some headaches and as a preventative of the swelling reoccurring he is back on dexamethasone liquid mind you , of 2mg.
The only difference from the February and March and April dosages will be that we are on a very low dose of dexamethasone, 2 mg, verses 12mg , 8mg and 24mg respectively.
As you may recall there can be a conflict where as dexamethasone keeps the Temodar from destroying the DNA in cells so inadvertently protecting all cells including the cancer cells during the treatment.
Also I am in hopes that Joshua's appetite will prevail as the steroid sure helped with that the other 3 times.

Anyway we will have answers on or about June 18 after our MRI of the brain to how things are doing with the ablated and SRS treated tumors.
The liver tumor we are still consulting several people in hopes of convincing a reputable doctor into ablating it. The current thought with any surgery has been that the effort to eliminate the tumor can cause additional growths, satellite or in transit mets to pop up. The Temodar is an effort to possible keep that from happening as well as treating Joshua's brain for any possible additional tumors. Brain or otherwise.

Josh has had some numbness on his left foot as well as a bit of a slight shakiness. We are looking for those two problems to be resolve after the discontinuation of the steroids .Diet really plays a role in keeping the shakes down as I could tell when he hadn't eaten. The low blood sugar shakes.

Hope all is well with you and yours, always

Will update later
Much love
Debbie

Hello all
Joshua began the temodar treatment on May 15 and ended on May 19.
Today he ended up having to be taken by ambulance to the city hospital after having had a 5 minute seizure while with his wife and daughter. Our family agreed that if the seizure had to happen ,we are thankful that it happened with some one around and not when Josh was by himself.

The MRI at the ER showed right side swelling around the two tumors we believe we're the previously tumors with SRS in December. Josh had been off dexemethesone for 4 days prior to today and really hadn't experienced anything except sleepiness

At the ER we were given 10mg of dexa and a dose of antivan as well as a dose of Keppra an anti seizure medicine.
Blood was taken as well to send to chemo doc
We were sent home with scripts of dex 4 tablets every 6 hours for 2 days then down to 2 tablets every 6 till we see a doctor and discuss the scan. We are being put on Keppra till further notice Insurance would of kept Josh in the hospital however his blood platlets were at there almost absolute lowest and being at the sickness place in town would of not of been a good choice.


We aren't sure if swelling is because of the withdraw from the dexa or the tumors or the Temodar ,but we hope to get more answers in the morning

We will be calling doctors neurologists as well as neuro surgeon as well as radiation onc and medical oncologist to get a plan

Will update in morn
Love
Debbie

Dear all

We have an appointment with neurologist and neuro surgeon Wednesday and Tuesday. The neurologist is the same one that specialized in MS BUT also specializes in epilepsy.
The neuro surgeon can possibly see if the two can be resected ? Our feeling is using radiation if need be
Obviously the medical oncologist over seeing Josh's Temodar schedule will be on our list as well as the radiation onc.
Have a beautiful Sunday

Love
Debbie

Hello all
Our appointment with the neuro surgeon yesterday and the viewing of the MRI showed profound edema around the ablated left larger tumor and swelling around the right side tumors. The right side tumors to my eye couldn't be seen thru the scans contrast however the neuro surgeon saw them as stable
We discussed the likely hood of seizures in general and I believe that he mentioned a 20% chance of patients having a seizure
My question is it the steriods or the temodar or the tumors?
We've been off the steriods and as I understand it dexamethasone withdraws are hard to do.
We've taken 4 doses of temodar and could it be aiding the ablation and radiation with it's continued attack on those already compromised tumors thus causing necrotic tissue to inflame the brain ?
Regardless we will be on the dexa of 16mg a day till we get in to see medical oncologist

We are seeing today the neurologist to talk about the seizure care
We were told that not having seizures for 6 months could get us re -evaluated in taking our seizure med
Will write more after docs visit today
Love
Debbie