KJ from Utah - Dx Aug 2013 at 6 years old

[LParsons]

Hello Everyone,
I have been lurking around here since my sweet little 6 year old grand daughter was diagnosed with ASPS on August 29th.
This process has been a blur, but of course I have been desperately trying to learn about ASPS and what I find is really very frightening.
My daughter noticed a small bump on her little girls shoulder blade earlier this summer, so she took her to her pediatrician. The pediatrician was certain it was only a lipoma, but sent my daughter to get an ultra sound just to be certain. The ultra sound was not confirming it as a lipoma, so she referred her to a CT scan. This scan seemed to indicate blood flow, so they referred her to a dye contrast MRI to be done on August 2nd.
At this point they were referred for a biopsy with Primary Children's Medical Center here in Utah. The pediatrician stepped in and told my daughter she preferred working with a surgeon at another hospital so they took my grand daughter there. This surgeon felt utterly certain it was only a lipoma and they could either remove it or leave it, since lipomas pose no risk. My daughter and her husband wanted to remove it. Surgery was scheduled for August 9th. Coming out of that surgery to speak to her parents, the only thing the surgeon could tell them was that it was NOT a lipoma. He said her carefully removed it, and felt certain he "got it all". The tumor was then sent for testing.
On August 15th, we were told it was sarcoma. Not a type yet, only that it was sarcoma. By now my daughter asked the doctors to stop speculating to her, and in her words; "I would rather be stabbed one time in the heart with a knife rather than being poked over and over with an ice pick." Our little girl had just started 1st grade, and her Mom has two younger boys at home. She was determined not to panic and second guess the results, since her speculation would not change the diagnosis or the prognosis. All it would serve to do is frighten her.
A PET/CT scan was ordered for August 28th, and we were give the results on August 29th.
Alveolar Soft Part Sarcoma, and good news is a clear scan.
With all of my searching and looking and trying to guess what was going to happen, I had not run across this word.
They wanted to go back in for clear margins. Her surgery was scheduled for September 6th. The tumor board met in the morning of her surgery, and her case was presented prior to surgery. The consensus was 100% that this was the course of action to take. The surgeon came out happily to tell us we had clear margins. They were concerned they may have to cut into her ribs, and remove muscle to get clear margins but they were able to get the clear margins without these things happening.
I marvel at her strength and her courage. She refused to stay in the hospital the night of her surgery, and since they took her home her only pain relief has been Tylenol. She does not like the way pain medication makes her feel. She still has a drain in, since enough tissue was taken the surgeon was concerned about the fluid. Also going in twice to the same site increases infection risk.
Today, September 9th we are waiting for the final pathology. We are praying it is clear, too. Results will not be in until tomorrow.

I am hoping to hear this is all good news, and we can look forward to years of a healthy happy girl. From what I read this can reoccur at any time and we have only uncertainty. The only control we have is how we manage the uncertainty. We will have her scanned every three months now for the first year. If we are clear after a year, they slowly open the time on the scans but regular scans are an important part of her future.

Anyone who has input, I look forward to hearing from you. Our baby girl will be turning 7 years old on September 25th.

[Olga]

Hi, welcome here,
first of all we all are hoping for the good news in the pathology report from your granddaughter second surgery. Younger kids seem to be doing better in the long term run. What scans were done - post here and we look into them so make sure nothing was missed.
You were a good lurker at our board, it feels that you already have a good understanding of the seriousness of the situation, difficulties that might be ahead and the need to be very vigilant. I am gland that our board is helping.

[D.ap]

Hello
Our son , Josh 33 years old , was dx August 29th of 2012.
Thanks for coming forward . We all have been where you are and know how incredibly hard this time is beginning your journeys to try and make sense of it all
Please be assured that our hearts go out to you and we are here to strengthen each other as we are able
Stay strong.

Debbie

[LParsons]

Olga,
If you mean the scans that have been done, we started with a ultrasound, then a CT scan then the MRI with contrast and finally a PET/CT. Her oncologist the surgeon and the pathologist were all in on the final consult with the results and said the scans to follow will be MRI with contrast. If cause for concern arises out of that, they will use a PET/CT to confirm and isolate. They said the amount of radiation with the MRI is lower and they do not want to hit her every three months with that.
I feel they have been thorough. I hear there is no specific successful therapy other than excision, so my mind goes to alternative treatments. Is anyone here familiar with any successes with say The Gerson Theory? I wonder if things like this warrant attention.
Here I sit again at 1:30am worried sick and unable to sleep. This child is so special to me. I will eventually accept our new reality, I feel like I am mourning our old life. Her pediatrician told my daughter the process of mourning is normal. I do think it sounds right. In 1998 I lost my oldest daughter when she was 17-1/2 in an auto accident. I am feeling the same sense of loss. I do have a positive attitude, but this pain... Oh how familiar it is.

[LParsons]

Debbie, thank you for your kind words. I am sure I will be here a lot.

[Ivan]

Welcome to the forums!

This surgeon felt utterly certain it was only a lipoma

I am getting a sense almost all of us ASPS patients have dealt with a moron like this. This guy caused your grand daughter major pain and suffering and further unnecessary damage during that re-do surgery. I also lost some healthy muscle in my arm due to an incorrect initial diagnosis.

[Olga]

You are saying that the scans to follow will be MRI. Is it going to be used for the primary location surveillance? MRI can not be used to watch for the possible lung mets, it has to be the CT scan as the lungs are moving. Even though the radiation dose she gets is a concern, it is still necessary. Sometimes the alternating X-Ray and CT scans are done, or some children's hospitals have reduced dose CT scanners.

[D.ap]

Dear L
I hope this day brought good news on your grand daughters pathology report
Write when is good for you
Love
Debbie

[LParsons]

Ivan,
I have been very upset with this initial surgery. I am mostly angry the pediatrician inserted herself and diverted them from Primary Children's Medical Center oncology to some one SHE preferred to work with at another hospital.
I am sorry for the pain it caused her, and your situation as well.

Olga,
Thank you SO much for the scan information and I will relay this to my daughter immediately.
I deeply appreciate your information!!

No report on the final pathology today. The doctor did speak with my daughter to see how our little one is getting along and so she was not left wondering. He just said "no information"... Yet. Hopefully tomorrow. I will certainly report what we hear.

Today I took my very precocious grand daughter to my friends salon and spa for a manicure and hair wash and style. She is still recovering from her surgery in Friday, and she didn't have her usual bounce. She still has her drain in and she calls it her "cord". By the end of the fussing and primping she was lit up and chatting and filled with her usual energy. It was good for her little soul to be pampered.

[D.ap]

L
Your day sounded wonderful at the spa!
Our grand daughter , Joshua's daughter , and I love to paint our nails
She will be 5 this month.

By reading more and more articles and visiting on this forum more and more you and your family will begin to feel more hopeful with the future.
Having scheduled scans will help a lot in keeping you and the doctors ahead of the game as ASPS typically is slow growing but a very persistent cancer. Because there is not much info ,as it is so rare ,you and your family need to find someone who will work for you and listen to your ideas. There is very little info for them to research from but it is out there if they and you look and research it. This forum has a library and also a wealth of people with over 10 years experience ie Olga and Bonnie as well as others.
Hope your grand daughter continues to feel better. What a joy for us grandmas and parents when our little ones aren't hurting!
You all continue to keep up the HOPE and faith

Have a beautiful day
Love
Debbie

[Kat]

Dear L,

I was so very sorry to hear about your grand daughter's diagnosis, she sounds like a beautiful brave little girl. I feel our stories may have many similarities.

My daughter Annika was diagnosed in July this year just after her eighth birthday (her birthday was spent in hospital after her first surgery). She too has had a second surgery to achieve negative margins (her tumour was inside her right triceps on the back of her arm).

I smiled reading about your spa day as this was the first thing I did with my daughter after her readmission for infection (cellulitis) after the second surgery. She was so sick of hospitals, lying around and drips! We went out and had her first ever pedicure together - she loved it!

My focus is on educating myself about ASPS and making the best choices I can for Annika whilst maintaining a normal, healthy childhood for her as much as possible.

Please feel welcome to contact me at any time as we (and I'm sure you also) have found no other families who are dealing with this disease at such a young age and would love to be of whatever support we can to each other.

Warm wishes for you and your gorgeous grand daughter,
Kat

[LParsons]

Kat,
So sorry to read your story. I feel grateful that KJ has been so fortunate in her surgeries. No infection and no complications. I will be in touch for sure!

FINAL PATHOLOGY; CLEAR MARGINS!!!!
We are elated and enjoying this great news tonight.
Her drain will be removed Friday and back to school on Monday.

[D.ap]

Lorryn

We are doing the happy dance!
Thanks for sharing the great news of clear margins
Here's to continued good news

Love
Debbie

[Kat]

Dear Lorryn,

Wonderful news! So happy for you and your precious grand daughter.

Warm wishes,
Kat

[Kathy]

Tears of Joy! We will continue to pray for you all. Please keep us posted!