July is sarcoma awareness month. In honor of that I am going to share some facts and share Ariella’s story for those who have only gotten to know her in the past several months.
What is Sarcoma?
Cancerous (malignant) tumors of the connective tissues are called “sarcomas”. The term sarcoma comes from a Greek word meaning fleshy growth. Sarcoma arises in the connective tissue of the body. Normal connective tissue include, fat, blood vessels, nerves, bones, muscles, deep skin tissues, and cartilage. Sarcomas are divided into two main groups, bone sarcomas and soft tissue sarcomas. They are further sub-classified based on the type of presumed cell of origin found in the tumor. They all share certain microscopic characteristics and have similar symptoms. Sarcomas can develop in children and adults. For children under 20 approximately 15 percent of cancer diagnosis are sarcomas. Although rare, there are approximately 15,000 new cases of sarcoma diagnosed each year in the United States.
In general sarcomas are divided into the large groups:
soft tissue sarcomas, and
bone sarcomas.
Ariella had Ewing’s Sarcoma
(from Stanford Health Care website)
What causes Ewing sarcoma?
The majority of Ewing’s sarcomas result from a chromosome rearrangement between chromosomes #11 and #22. This rearrangement changes the position and function of genes, causing a fusion of genes referred to as a fusion transcript. Over 90% of individuals have an abnormal fusion transcript, involving two genes known as EWS and FLI1. This important discovery has led to improvements in diagnosing Ewing’s sarcoma.
Similar to osteogenic sarcoma, trauma or injury is sometimes involved with the site at the time of diagnosis. However, this trauma is thought to bring the condition to attention rather than to have any causal relationship.
Some doctors classify Ewing’s sarcoma as a primitive neuroectodermal tumor (PNET). This means the tumor may have started in fetal, or embryonic, tissue that has developed into nerve tissue.
Ariella began complaining of pain in her right leg in September 2016. However this was following an injury, she had accidentally gotten hit by a chair in school. Ariella was a dancer and she pretty much only complained of the leg pain at that time after she had been dancing. We did have it x-rayed which didn’t show anything and we were advised to have her rest the leg for an extended period of time so it could heal. Since the pain did get better with rest we just figured it was injured further with dancing and didn’t think anything of it. At the same time, Ariella had lost an alarming amount of weight which we were working with her pediatrician to figure out. As part of diagnosing the cause of the weight loss blood work was done. Blood work came back normal. Sarcomas cannot be detected in the blood. We did not at all connect the leg pain to her weight loss. Her pediatrician was unaware of her leg pain since it came and went, not sure if he would have made the connection or not. Rest seemed to help so she continued dancing but by the end of January the pain was constant, not just when she used her leg. It was also red and swollen. She saw an orthopedist who was concerned with what he saw on the x-ray and sent us next door to his office for an MRI. Not 20 minutes after the MRI I received a phone call that she had a tumor, it looks malignant, and a referral to the orthopedic oncologist. We saw the oncologist the next day, January 27, 2017 and had more tests and scans. She went in for a biopsy Monday January 30, 2017.
I actually don’t remember the exact day we had the official confirmation of cancer but it took several weeks after that to determine that it was Ewing’s Sarcoma. Here we are, anxious and wanting to start treatment immediately but had to wait for the type of cancer because all cancers are treated differently. February 27, 2017 we got confirmation. Luckily the cancer was localized to her right tibia. We then had to meet with the pediatric oncologist who would detail the treatment plan.
Ariella began her treatment in March, 2017. It started with placing a Hickman Catheter, which ended up causing a pneumothorax requiring a chest tube. This delayed treatment further as it took a good 5 days at least for the pneumo to heal and the chest tube to be removed. Then finally, treatment started.
Ariella endured 17 rounds of chemo which required her to be inpatient every other week in the hospital for at least 2 or 5 nights. She pretty much had every side effect you could have; hair loss, nausea, fatigue, neuropathy, etc. She had fevers several times requiring ER visits and an infection that required her Hickman to be removed and replaced, causing another pneumo requiring another chest tube. However when she was not in the hospital you would never know she was sick. She continued going to the dance studio (even though she couldn’t dance), spending time with friends, and having sleepovers.
After the 6th round of chemo the chemo was paused so Ariella could have her limb salvage surgery to remove the tumor. Rather than using an implant the doctors decided to place an external fixator to allow Ariella to regrow her own bone.
Ariella rang the end of treatment bell December 28, 2017. She had her Hickman removed January 2, 2018 and returned to school that same week. The kid was amazing. She jumped back into her life like nothing happened. We were relieved when her first set of routine scans came back clear and devastated when the second set of scans June 1, 2018 did not. This time the cancer came back in her lungs, acetabulum, and sacrum. Ariella did have her fixator removed the following week.
This time Ariella had a port placed instead of a Hickman, which allowed her to have a decent summer. Treatment was outpatient and we were still able to go on our planned trips to Disney World, LA, and Ocean City. Ariella even went to school when on this chemo. She went through 2 different chemo regimens which were not working and finally went on an oral pill. She also had radiation to all the various spots. The pill and radiation were what got her stable, showing no evidence of active disease. We got this great news on January 25, 2019 and we began preparing for bone marrow transplant. The hope was that her new bone marrow would kill any residual cancer cells.
Ariella was admitted to the hospital on February 18, 2019 for pre-transplant radiation and chemo. The transplant was on February 26, 2019 with her father as her donor. Though she didn’t feel well after her transplant things were going well, until they weren’t. On March 7, 2019 she was transferred to the PICU. She had strep pneumonia and also the flu. She was septic with a low blood pressure (the reason she was transferred) and requiring oxygen. She began to show improvements, weaning down on the oxygen but then had nosebleeds and began to require more oxygen support requiring intubation on March 15, 2019. I’m not going to go into all the ICU details and complications, but just say that we were so hopeful because she would show improvements. But then she would have a setback. This was the pattern for her entire ICU stay. Until finally she couldn’t recover from her setbacks. Ultimately she died on May 9, 2019 from respiratory failure and kidney failure.
If you want to read Ariella’s story from the very beginning you can at https://www.caringbridge.org/visit/ariellastein This will take you from diagnosis through ringing the bell at end of treatment. Ariella wanted to be able to share her own pictures and updates so we started www.facebook.com/ariellapaigestein near the end of her first round of treatment in November 2017. You can see the rest of her story there.