Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Dear ASPS friends,
I am bernard banarez from Philippines. My son bence, 9 year old, was diagnosed with hemangioma on his left palm. We observed the mass on his left palm last year September 2015. He admitted at Philippine General Hospital and scheduled last june 15, 2016 for operation and the mass were sent to the surgical pathology. June 20, 2016 my son discharged at the hospital and the doctor told us that after 2weeks we will come back for his follow up check-up. July 5, 2016, we went back at the hospital. I took the result of the biopsy and its written as follows: rhabdoid tumor and rhabdomyosarcoma. The doctors told to me that they need some stains for further confirmation. After another 2weeks the result was alveolar soft part sarcoma.
His first doctors recommended us to the cancer institute of the hospital. His attending physician recommend us laboratory procedures like bone marrow biopsy (negative result), whole body bone scan (negative result), chest and abdominal ct scan with contrast (all organ are intact and no swollen parts).
His doctor told me that it is her first time to encounter this type of cancer. Yesterday, sept. 23, 2016, was a check up of my son. His doctors told me that she researched this type of cancer and told me that this type of cancer will not undergo for chemo instead will undergo for radiation.
I love my son, and i don't know if this is the right cure for him. I think his doctor did not know what she is doing or i am just paranoid upon searching some pictures and cases of asps thru net.
Please help me with the case of my son, i am a tricycle driver and my wife is a fisheries technician which receiving a monthly salary of 9 thousand Philippine peso which is only enough for our daily expenses.
Thank you and more power.

Hello Bernard
My name is Debbie. I sure am sorry to hear of your sons diagnosis.
How big is the tumor on your sons hand ? Left correct ?
Does there appear to be tumors in his lung ?
Has surgery been discussed?

I know and remember how upsetting it was when our then 32 year old son was diagnosed back in 2012 but there is hope with knowledge of the best ways to go about understanding and treating alveolar soft part sarcoma (ASPS)

Write when you are able
Debbie

Bernard
Here's a resource for your doctor to talk to who are children's doctors dealing with ASPS
There are several parents with young kids within this forum when you are ready to visit about your sons care

Here's the link of the hospital

http://www.childrenshospital.org/condit ... rt-sarcoma

There are new medicines being tried for ASPS called immune therapy drugs helping lots of people

Surgery can sure be the beginning as doctors know how tomake sure the sarcoma is gotten. Ask lots of questions

Parents on forum to name a few are -
Crystal with Brinkley

http://www.cureasps.org/forum/viewtopic.php?f=4&t=739

And MJ

http://www.cureasps.org/forum/viewtopic ... 1108#p8390

Kat and Daimian. Daughter Annika

http://www.cureasps.org/forum/viewtopic.php?f=4&t=822
They know as well as I do how scared it feels but medicines are being discovered to help our kids

Just wanted you and your family you are not alone
Love
Debbie

Dear Bernard,
My name is Bonni Hess, mother of now 34 year old ASPS patient Brittany who was diagnosed 15 years ago at age 19. I am so sorry for your young son Bence's ASPS diagnosis and I know far too well how very frightened and helpless you must feel right now, but am grateful that you found your way to our Web site and that you have reached out for information and support. I am sorry too to be slow in responding to your post, but Brittany and I just returned from Miami where we attended a wonderful ASPS workshop and learning seminar which provided invaluable information for our ASPS Community and great Hope for promising new treatments.
The misdiagnosis of Bence's cancer is unfortunately very common with this extremely rare and little known disease. I am very grateful that the scans showed no current metastasis to his lungs, bones, or abdomen, and Hope that his disease will remain stable, but it is critically important to remain vigilant with regular scans to ensure that any possible mets will be found at the smallest most treatable size. Additionally, if he has not already had one, it is very important that Bence have a brain MRI ( NOT just a brain CT which is inadequate to detect small brain mets) since ASPS heartbreakingly can and does metastasize to the brain. Your doctor is correct that traditional chemo has unfortunately not been shown to be effective in treating ASPS, but radiation is usually not a good treatment option either because ASPS is notoriously radiation resistant. However, there are thankfully several promising new systemic treatments including Cediranib that has now very gratefully provided Brittany with 7 and a half years of disease stability, no new tumors, and dramatic shrinkage of her multiple widely disseminated mets. Because Bence currently thankfully has no scan visible metastasis, I and some others on this Board personally feel that the best approach is to just closely monitor the disease with regular scans and not to begin any type of systemic treatment unless the patient develops disease progression which can't be treated with surgical removal (resection), ablation, or radiosurgery because the currently available systemic treatments are sadly not a permanent cure and may preclude future systemic treatment. I strongly encourage you to remain as pro-active and knowledgeable as possible in your son's care, and to actively communicate and network with other ASPS patients/families on this Discussion Board which is an invaluable source of shared anecdotal treatment information and strengthening support and encouragement. I have requested that Olga, our wonderful Web site manager, move your entry to the Personal Updates topic so that it will be easier for people to access and follow Bence's journey. Take care dear Bernard, stay strong, let Hope lead you through each day, and keep in touch with the Board as you are able.
With special caring thoughts, healing wishes for dear Bence, and continued Hope,
Bonni Hess, mother of 34 year old Brittany diagnosed 15 years ago in July 2001 at age 19

Hi Benard -
Yes, you are not alone. Unfortunately, there is lots to think about for treatment.
The tumor was removed - you need to ask if they were able to get positive margins. This is the area around the cancer all the way to the non cancerous cells. It indicates if they got it all out. Then is there are no other signs of metastasis - radiation can be considered over chemo. Importantly, after initial treatment, even if they think they got it all, you are going to have to be keeping up with scans to continue to monitor for other tumors in other places even, especially his lungs and brain. It's alot to take in, but people on this site will help you.

Keep us posted - stay strong for your son!

MJ

Dear All,

Thank you for all the messages.

The size of the mass remove in the left pal of my son is 3.0 x 2.2 x 2.0cm.

Last Sept. 26, 2016, the hema onc refer us to the radiation onc. The new doctor were interviewed me regarding on bence. After interviewing, he told me that it is also his first time to encounter this type of cancer so he told me to get the following:
1. SLIDE REVIEW
2.OR TECHNIQUE
3. CT SCAN PLATE
4. NEED TO BE X-RAY (PELVIS AP)

Now, i already got the OR tech, CT scan plate and pelvis xray result.

I am not a doctor but i am worried in the result of the pelvis ap xray.
Result showed (Sept. 26, 2016):
-Multiple fairly-defined lytic lesions of varying sizes are seen along the left femoral neck and inter trochanteric ridge.
-The rest of the cortical outlines are intact.
-There are no sclerotic changes noted.
-The joint spaces are maintained.
-The soft tissues are unremarkable with intact fascial planes.
Impressions:
Lytic lesions in the left femur, as described, worrisome for metastatic disease in the light of a known primary malignancy.

My son was undergone whole body bone scan last July 19, 2016
Findings shows, no bone lesion.
Interpretation, no evident bone

Which labtest is better the xray or the whole body bone scan?

We are poor family. My wife is a contractual employee of Bureau Fisheries and Aquatic Resources Region 2, Philippines who receives a wage amounting to Php 9,000.00 per month. I was once an OFW for 2 years and manage to invest in a second hand tricycle which i drive and the only source of my income. Our income is only enough to meet our daily expense, we make ends meet and hope that our children have a good education.

Hence, too all the members of the forum, i would like to appeal on your generousity and we ask for financial assistance that you could extent for the case of my son. We want him to live a normal life and help him to achieved his dreams.

Thank you and God Bless.

Sincerely yours,

Bernard Banarez

Hi Bernard,

My name is Martin and I'm ASPS patient my self.

I see that you guys are on the right path.

What actually the lytic lesions mean.

What does the Chest CT say. This disease if spread most likely goes to the lungs first although there are other cases also.

Are the chest clear of any mets? is the place of surgery healing well?

Stay strong as this is a slow disease and is a long battle

Dear Bernard, Thank you for your thoughtful update on the results of dear Bence's most recent scans. I am perplexed as to why a less definitive pelvic x-ray was done instead of a pelvic CT scan or MRI, but regardless, I share your concerns about the results of the x-ray which apparently showed multiple lyric lesions in the left femur. This is something which you need to discuss as soon as possible with the oncologist, and request that a more definitive CT scan or MRI, WITH CONTRAST, be done. I, like Martin, also question whether there may be lung mets since the lungs are usually, but not always, the first place that ASPS metastasizes. Regarding your plea for financial assistance from other members on this Board, while my heart breaks for your difficult financial situation and I deeply admire your and your wife's hardworking efforts to financially provide for your family, unfortunately all of us on this Board have our own personal immense financial burdens and challenges created by the enormous and often overwhelming medical expenses of this disease, and this is not the appropriate forum to request financial help from other ASPS patients and families. None of us may be in the position to help you financially, but what we can do is share invaluable researched and anecdotal treatment information as well as strengthening support and encouragement to help you on this difficult and challenging ASPS journey which we all share. I am not personally familiar with what type of medical coverage is available for Philippine citizens in the Philippines, but I encourage you to discuss your financial needs with the clinic/hospital social worker if there is one available. Also, it is my understanding that Saint Jude's Children's Hospital in the USA is committed to provide free medical care and treatment to pediatric cancer patients so this may be a resource for you to explore and contact. Hopefully others on this Board may have some other medical financial assistance ideas or resources that they will share with you. In the meantime, I Hope that you will be able to get the necessary scans for Bence to obtain an accurate diagnosis of his current mets which can then be better discussed with his oncologist for treatment plans to be made. Take care dear Bernard, stay strong, and keep in touch as your time and the situation allow. Sharing your heartache and concern with deepest caring, healing wishes for dear Bence, and continued Hope, Bonni

Dear MJ and Bonnie,

My son was not yet undergo for lung ct scan, i will talk t s doctor regarding to this including the brain MRI. Regarding on the excision of my son, the wound is healed already. He can move his fingers with no pain at all.

These are the result of chest ct scan w/ contrast (sept. 18, 2016):
- multiple axial tomograhic sections of the chest, with intravenous contrast, were obtained. No adverse reaction was observed
- there are no parenchymal infiltrates, masses nor abdominalcalcification seen.
- no evident enlarged lymph nodes
- no pleural effusion nor atelectasis noted
- the heart and blood vessels ate unremarkable
- tracheo-bronchial tree is patent, with no intraluminal lesions evident
- the rest of the soft tissues and osseous structures are intact
Impression:
Normal ct scan of the chest.

These are the result of whole abdomen ct scan w/ contrast (sept. 18, 2016):
-the liver is w/in normal size and configuration, w/ smooth contours and homogenous tissue attenuation. There is no calcifications nor mass lesion seen. The intrahepatic ducts are not dilated.
-gall bladder is unremarkable
-pancreas and spleen are w/in normal in size and configuration. There is no focal mass lession seen. Pancreatic and common bile ducts are not dilated
-the kidneys are within normal size and configuration. Renal parenchymal opacifications at both sides are symetrical and simultaneously. The cortical thickness of the kidney is normal. There are no evidence of lithiasis nor mass lesion. The collecting systems are not dilated.
-adrenal glands are unremarkable
-abdominal aorta is normal. There is no enlarged para-oartic/paracaval lypmh nodes.
-the stomach and included bowel loops are non dilated, w/ no evidence of wall thickening
-the messentric fat is normal. No ascites noted.
-the urinary bladder is well filled, w/ no thickening of the walls nor intraluminal densities/filling defects seen.
-lytic changes are seen in the bilateral femoral necks, more on the left
-the rest of the tissues and osseous structures are unremarkable.
Impression:
- Lytic changes, bilateral femoral necks; more on the left
-the rest of the tissues and osseous structures ate unremarkable.

Those are the result of my son's ct scan. I am sorry for asking financial assistance here at the forum but as a father i will do all my best eventhough it is shameful to seek help for my son's situation.

To Bonni, thank you for the info about st. Jude med but i am afraid i could not be able to contact that hospital due to my internet situation here in the philippines but, i will try my best to contact it.

We have a scheduled check up on monday (oct. 3, 2016). I will update you regarding on what conversations we tuckle by his doctor.

Again sorry and thank you for quick response to my message.

GOD BLESS US ALL!

Bernard Banarez

Hi Bernard,
to clarify some things here:
- lung ct scan=chest CT scan, so your son already had it (sept. 18, 2016).
- lytic changes in the femoral necks are most probably not the metastases as they are rarely bilateral in similarly looking in the bones, and bones are always not the first metastatic site;
- I am not even sure if he needs to have any treatment at this point. Find out if the resection of the primary tumor was done with the margins - i.e. if there was a healthy tissue taken around the tumor along with it, so the resection is clean. In that case he might even not even need a radiation (my son did not have it and never had any local recurrence);
- we do not provide a financial assistance here although it is not prohibited by the rules to ask. But I am not even sure what do you need to pay for now and what is the situation there. The future situation of your son will depend on whether his disease remains localized (as it is not based on the scans results) or it will spread and form distant metastases. He needs to be scanned at least once in 6 months from now on - CT scan of the lungs. Till then, your son will not need any treatment - the same treatment plan is done to the patients here.
I am a bit not sure re. correct pathology diagnosis as we did not see a case of the ASPS in the palm, only in the arm.

Dear Bernard, Olga in her great wisdom has done an excellent job of clarifying many important points regarding your young son's situation. Based on your most recent post it seems that on September 18th Bence already had a lung CT which is the same as a chest CT. The scan results that you shared seem to indicate that Bence thankfully does not currently have any lung, abdominal, pelvic, or bone mets so I agree with Olga's recommendation for Bence to just have monitoring scans at least once every 6 months with no other treatment at this time. However, I would still encourage you to request a brain MRI to obtain and establish a base line for future scan comparisons, and I think that it is important that you obtain clarification from the oncologist regarding the possible implications of the noted lytic changes in the femoral necks. As Olga has alluded to, the diagnosis of Bence's palm met as being ASPS is very unusual for a primary ASPS tumor, so it does need to be definitively confirmed through pathology that it is actually an ASPS tumor because mistakes can and have been made in correctly diagnosing this difficult to diagnose extremely rare type of cancer. It is certainly not "shameful" for you to seek help for your son's situation and no apologies are needed as all of us ASPS Community parents know and understand very well the desperate wish and desire to do whatever we need to do to try to save our children's precious Lives. I had just wanted to clarify to you as Olga did that this Forum does not provide financial medical assistance for patients and their families, and it is not an appropriate place to request financial help from other ASPS Community patients and families because we are all facing our own immense personal financial challenges and burdens in fighting this very expensive to manage and treat disease. Hopefully a medical social worker in the Philippines can help direct you to some local medical financial resources that may be available for you. My best wishes are with you for a successful and informative appointment with Bence's oncologist on October 3rd, and I will be anxiously awaiting your update on the results of the appointment. Take care Bernard. Reaching out across the miles to share special caring thoughts, healing wishes, and continued Hope, Bonni

Dear All,

Thank you for your clarifications and responses.

Previous Test:
-MTI OF WRIST AND LEFT HAND (may 10, 2016)
Impression: well-defined, hypervascular soft tissue mass,left hypothenar region (known case of hemangioma)

Date of operation june 15, 2016
1st histopathologic diagnosis(june 16, 2016)
-round cell neoplasm, considerations:
a. Rhabdoid tumor
b. Rhabdomyosarcoma
Recommend immunohistochemistry studies w/ vimentin,cytokeratin and desmin for confirmation

2nd histopath (july 7, 2016)
-Immunochemistry findings are as follows:
VIMENTIN: Strongly positive in cells of interest
DESMIN: Strong and focally positive in cells of intetest
CYTOKERATIN: Negative in cells of interest
Cutaneuos Granular cell tumor, rule out alveolar soft part sarcoma

3rd histopath (august 12, 2016)
Alveolar soft part sarcoma
Note: The immunohistochemistry studies shows the tumor is negative for calretinin and myogenin and positive for PAS. This and the morphologic features favor the above diagnosis.

Review of slides (sept. 30, 2016)labeled "16 IHC 1137 AND CHA 4315
Hypothenar mass, left excision:
-ALVEOLAR SOFT PART SARCOMA.
POSITIVE FOR TUMOR, SURGICAL MARGINS OF RESECTION.

The rad onc told us this morning to go back to the hema onc. He told me that he presented the case of my son to the consultant doctors and it seems that the mets of ASPS are in the left femur. Next thursday is our check up again to the hema onc.

Till here.

Bernard

Dear Bernard, Thank you for the shared pathology diagnostic reports which seem to confirm the diagnosis of ASPS. I am confused about what you meant about the radiation oncologist telling you that "it seems that the mets of ASPS are in the left femur". Is he indicating that Bence has an ASPS tumor in his left femur, or that ASPS is often located in the left femur which is true, but ASPS primary tumors can be located in any of the extremities or other soft tissue areas of the body and are certainly not restricted to the left femur. Please clarify what the radiation oncologist is alluding to when you are able. With special caring thoughts, healing wishes, and continued Hope, Bonni

To add to Bonni suggestion - if the suspicion is based on the findings of the whole abdomen ct scan w/ contrast (sept. 18, 2016) that you posted before on Sep 30, 2016, ask for a more definite MRI scan. I do not know how it works there and if you have to pay for the MRI scan (it could be expensive), but a CT scan is not that definite even when it is done with the contrast as there are other medical conditions when the contrast could be attracted to the spots - such as local inflammation. From the scan result they say that there are "lytic changes are seen in the bilateral femoral necks, more on the left" so why are they saying there are suspicious metastases on the left only as they are noted to be bilateral - both sides? Ask them to investigate if there are other medical conditions that could be typical for the growing kid that can produce these changes that might be just growth or diet related. Once again, it is pretty rare for ASPS to have bones as a first site of metastases and also to have them bilateral from the beginning. We have very extensive experience with different variants of the ASPS metastases and did not see any of that.
Also about the diagnosis. There is a very specific genetic deffect that creates ASPS - a translocation in ASPS that can make its diagnosis very definite and I do not see it was done, may be it is not done there or takes a long time - I do not know. The translocation creates a novel ASPL-TFE3 fusion protein, detection of TFE3 nuclear expression is useful in establishing the diagnosis of ASPS.
This is an article to read if you want:
http://sarcomahelp.org/asps.html

Hello again Bernard,
I agree with Olga on all points and urge you to share the information and questions that she has provided with your oncologist. I too was perplexed as to why the diagnostic pathology report did not make any reference to the ASPS specific genetic defect translocation which creates a novel ASPL-TFE3 fusion protein that is definitive for ASPS diagnosis. This is something which needs to be addressed and clarified to ensure that Bence's ASPS diagnosis is accurate.
With more special caring thoughts, healing wishes, and continued Hope,
Bonni