Cure Alveolar Soft Part Sarcoma International (iCureASPS)

My daughter Mira was diagnosed with ASPS a few months ago. The tumor appeared when she was 18 months old. Right chest wall below clavicle. The doctors initially diagnosed as a hemangioma. We followed up yearly The tumor kept growing so we decided to resect a few months ago. When the tumor specialist palpated her, she noticed the tumor was firm. Doc wanted a biopsy. We got results back as ASPS and we scanned the whole body. By now, the tumor has been sitting inside her for 4.5 years. We found a single 3mm nodule on right lower lung. This was also resected at Texas Children's despite MD Anderson not wanting to get the lung nodule due to size. We resected the main tumor last week and Mira is already healed up. Unfortunately on Pathology reports, we found the superior and lateral margins at 1mm (with negative margins) We are going back in to resect a little more tissue. What we have learned from this whole ordeal.

1. Never take the word of one doc. They are human. Our surgeon at TCH had no issue with resecting the nodule despite size. He injected a dye the night before to find the tumor and it glowed. The MDA surgeon did not even offer this.

2. No matter which oncologist we speak with, no one knows enough about this tumor. In fact, I have learned more from this forum than doctors.

3. Be persistent, ask questions that make sense.

4. Stay in the present. Stop worrying about what the future holds. Live here today to the fullest.

Hi,
welcome to our forum community. We are very supportive here and are ready to share all the information we have about this extremely rare sarcoma, and we appreciate when patients or their families share what they can in return. I also agree that doctors are also people with their own opinions and experiences and requesting second, third or numerous opinion is advisable in order to get the better understanding of the situation and treatment options.
I have a question - do you have the pathology report on the resected lung nodule?

Thank you for Mira’s info.
I’m sure sorry of Mira’s diagnosis (dx’d ), however with the dx’d comes reality and action .
Information and awareness have come x fold , even since our son was dx’d in 2012 and this forum has been a god send to our family .
It sounds like you all have learned from the initial experience and have a great philosophy in enjoying each day.

4. Stay in the present. Stop worrying about what the future holds. Live here today to the fullest.

Let us know how Mira is doing .

Olga wrote: ↑Tue May 18, 2021 7:47 pm Hi,
welcome to our forum community. We are very supportive here and are ready to share all the information we have about this extremely rare sarcoma, and we appreciate when patients or their families share what they can in return. I also agree that doctors are also people with their own opinions and experiences and requesting second, third or numerous opinion is advisable in order to get the better understanding of the situation and treatment options.
I have a question - do you have the pathology report on the resected lung nodule?

Olga, is it better to post the path results here or in the path section?

It was basically a question if the resected lung met was confirmed to be ASPS by pathology report?

She had 2 nodules removed. Each was 3mm. One came back positive for ASAP.

Thank you for clarification. The finding confirms the metastatic state of her disease, unfortunately, although ASPS very often evolves to be metastatic later in life, as it seeds very early in course of the disease, but micromets stay dormant where they trapped in the organs, most often lungs, till they start to grow eventually.
Is the planned second look surgery going to be extensive? in ASPS even thin margins are usually sufficient as it is not that locally aggressive, so there is usually no justification to a surgery with the expected functionality loss or the big deformity.

Sunman wrote: ↑Mon May 24, 2021 8:59 am She had 2 nodules removed. Each was 3mm. One came back positive for ASAP.

What kind of follow up is she on presently?

It’s been two years for Mira. So far we are clean on chest and abdominal scans. We will do a full scan per schedule on this forum next appt. She has been doing great so far. Her initial full body scan was clean except for one met 3mm in lung. Everything was resected.

Ivan wrote: ↑Sat Aug 14, 2021 12:04 pm

Sunman wrote: ↑Mon May 24, 2021 8:59 am She had 2 nodules removed. Each was 3mm. One came back positive for ASAP.

What kind of follow up is she on presently?

We were doing scans for chest every 4 months. For one year with contrast. We are on a 6 month schedule currently. We did have head and abd scanned also

Sunman,

Excellent news !
Sending hugs your way !