Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hello
My name is Ellen and my 8 year old daughter, Amelia, developed a tumor over her left shoulder in January of this year...she had a diagnostic mri, a chest ct scan (that noted a 2mm lesion right upper lobe) and then an ultrasound to determine the vascularity of the mass...The lesion was just under 5cm and was excised (March) .... On gross pathology it looked to be a hemangioendotheliaoma, however, the final pathology, of course, was ASPS......The microscopic slides showed a small nest of vascular invasion remaining in the infraspinatus muscle so she returned to have the entire muscle removed and a thoracotomy for excision of the metastatic lung nodule (April)....Her post op recovery was amazing and she is back to swimming trying to have a normal summer....

She is scheduled for scans (ct / mri /pet) this month and then we hope to get to chest ct only at regularly scheduled intervals.

I cannot express how important this website has been in helping us to formulate a plan of care for our daughter....Thank you all for sharing your treatments.....We have had an excellent surgeon and oncology team in Tampa, FL and have contacted St Jude's Children's Hospital in Memphis, TN and Dana Farber Cancer Institute in Boston, MA for opinions.

We have been offered radiation and chemotherapy, radiation alone or no treatment and monitoring.....We have elected to monitor.

It sounds childish and uncharacteristically superstitious of me but I have resisted posting our case study because it seemed like that would finally cement the fact that this is true and that we have to figure out a way to live with ASPS. As you all know, this has been a challenging time but your posts have helped me to understand that we need to focus on healthy choices, appreciating each day and working to raise funds for research. We will continue to explore options as they become available.....If ANYONE has any advice they'd like to offer or insight to share, please do so.

I am most encouraged that Dr Visticia and colleagues have been successful in replicating the tumor in mice!!! This is an extraordinary break since this tumor is so difficult to study...We need to raise $$$

I would especially like to thank Yousef Landesman for founding this organization.

Dear Ellen,
My Best Wishes to Amelia. Thank you very much for writing. No one of us chose this ASPS journey, but now when we are there we will walk this way together and find the cure for our children. I know that many other ASPS patients, their family and friends are reading us and I am very glad to know that the information on the iCureASPS website helps patients to formulate treatment based on experience and knowledge. This is a rare disease and it is important that we share information.

I hope that additional ASPS patients and relatives will join and write.
There is slow progress in the research and the understanding of ASPS but it is in our hands to enhance this progress.

Our power is coming from the ASPS community members who communicate with us through open discussions and shared information and from funds that we direct towards specific projects that aim to find cure for ASPS.

Hi Ellen, I can relate to what you say about how difficult is to post/tell/speak about what is happening to us - patients and their families - after this Dx strikes. Everything is different now and I still dream about waking up and getting back our wonderful life as it was. But we are responsible for doing our best in the management of this cruel disease and it includes collecting and sharing the info. There is a lot of information was lost because the people kept their cases and were just reading what we post. We do not know who and when can use this info and what we will be looking later here for. For example - we found a small lump above of my son's eye and I was researching all over the studies to find out if there were any cases with the mets to the face bones - couldn't find any so decided it is not likely to be of importance. Our oncologist was in a different mood and went right away to take a biopsy - it proved to be nothing - but later I found that we had a case with the met to a jaw bone and it was never made public - so the danger was very real and I was not aware of it. The same is with Amelia. After you shared the info that a small undetermined lung nodule was proved to be a lung met it can rise an awareness in the other people and stress the point that ASPS lung met can stay stable for a while, no changes, be not multiple - but still be a real met not the scar tissue. Some oncologists use the wait and see approach and assume that the nodule is not a met after a year or two stability. I don't really understand what was the point of doing the full thoracotomy for an exploration or was it a VATS procedure? We have a case here -Sean Anderson - he had a VATS procedure to verify the same situation and his lung nodule was also found an ASPS but it was may be two years ago and he is doing just fine now, Elaine can give you more info about the case I am sure. About the chemotherapy and ASPS - this subject is still open as it is more or less proved that chemotherapy is ineffective in the advanced stage but there is a very limited information about its work for the smaller or even dormant mets as in most of the studies patients with no visible distant disease didn't have any chemo after the resection of the primary but the study done by the Andrea Ferrari in the paediatric setting has lower metastatic rate comp. to the other studies - they give a chemotherapy to the most of the children with ASPS there. Also there are a few single cases when chemotherapy given after/before of the resection of the lung mets was apparently effective as smaller lung mets were found completely necrotic and the bigger ones were partially necrotic so when the study tells that there is no conclusive point reg. the role of the chemotherapy in ASPS it means that it is still not known when and who may benefit for the chemo.

Olga,
Thank you for your response and insight. I have found your information on the site to be quite valuable. Amelia's surgeon had planned a VATS but the size of her chest with relation to the lesion and the available instruments made thoracoscopic excision of the met impractical. They attempted and progressed to an open thoracotomy was a small midaxillary incision with a chest tube insertion site. The chest tube was out in 24 hrs and she was home in 48 with almost no narcotic required. It was a truely amazing recovery. I think her age (8) and her constiturion coupled with the surgeon's skill and his use of intercostal nerve block and preemptive analgesia given by the CRNA (nonsteroidal antiinflamatory and 'upfront' narcotic) helped to prevent the pain 'windup'. I think everyone with any choice would elect the VATS over a frank open thoracotomy. In either case I know first hand how important it is to address the preemptive strike on the pain receptors with combination therapy to reduce the noxious mediators of pain.
Local anesthetics pre and post incisioon should play a pivitol role in prevention of discomfort post chest surgery....If any one would like to know about options that may be available to them for I would be happy to explore those options . Have a great summer -- God bless.......ELLEN