Childhood Cancer

Neuroblastoma

Neuroblastoma is a type of childhood cancer that most commonly originates in the adrenal glands, but can also develop in the nerve tissues in the neck, chest, abdomen or pelvis. It is the most common extracranial solid tumor cancer in childhood and the most common cancer infancy. Neuroblastoma is divided into three risk categories: low, intermediate and high risk. 

Latest Neuroblastoma grants

Peter J. Murray, PhD, Principal Investigator
St. Jude Children's Research Hospital
Springboard Grants, Awarded 2013
Rohan Fernandes, PhD, Principal Investigator
Children's Research Institute
'A' Award Grants, Awarded 2016
John Maris, MD, Principal Investigator
The Children's Hospital of Philadelphia
Reach Grants, Awarded 2016

Latest Neuroblastoma blog posts

February 22, 2017

by Kelby Wright, a senior at Bozeman High School, Bozeman, Montana

December 4, 2000 was a terrible day for a particular family. Their toddler daughter was diagnosed with stage IV neuroblastoma. The cancer had already spread to various areas in her body. She was just 18-months-old. 

The tumor was noticeable in her left cheek. However, the parents of the child did not know that there was also a tumor in her right adrenal gland that would most likely result in having that organ removed.

She survived the round of cancer. But soon enough, about nine months after the treatment, her stage IV neuroblastoma returned in her jaw. Her parents, devastated once again, stayed with her through the harsh chemotherapy and radiation. Her mother was always feeling around in her jaw in order to check on the tumor. 

She was a happy little girl, minding her own business and playing with the hospital toys. She always had a smile on her face. The doctors had warned that she had a very low chance of surviving both the first and second time. Soon enough, the cancer in her jaw left for a second time. And then, as doctors predicted, it returned. 

That little girl was me, Kelby Wright.

When I was 3-years-old, I broke my arm after falling off a bike that was too big for me. My parents took me to the doctor, who discovered the third round of neuroblastoma. At the time, the side effects from chemotherapy were making me miserable. The neuroblastoma I was battling had an amplified gene, which gave me a slim chance of survival after my first relapse. Now I was on relapse number two. 

My parents decided to take me off chemotherapy, choosing radiation and pain management, so I could be happy for the rest of my life, instead of miserable. My parents and doctors did not expect me to live. 

But, as unexpected as it was, even without chemotherapy, I survived for a third time. 

You might think that was the end of it, but no, cancer returned for a fourth time. I was just 6-years-old and neuroblastoma was found in a lymph node under my arm. Once again, doctors gave me very little chance for survival.

But here I am, 12 years later and a senior in high school writing about neuroblastoma, fundraising for Alex’s Lemonade Stand Foundation for my senior project and full of hope for the future. 

Hope is not something to give up. 

My parents never gave up hope for me, even when they thought I might die. To give up hope is essentially to give up in general. With hope, one feels a sort of humble empowerment. You know you do not have control, but you can’t help feeling that things will eventually get better.

Hope makes life easier. Without it, we are helpless. We can’t function.

Kelby Wright is a senior at Bozeman High School in Bozeman, Montana. She is a four-time neuroblastoma survivor. Kelby graduates in June and aspires to teach art to children. This year, she put her graphic design skills to work and hosted a fundraising event selling greeting cards to support Alex’s Lemonade Stand Foundation, raising over $2,000. 

February 13, 2017

Neuroblastoma, the type of cancer our founder Alex Scott battled, is the most-common extra-cranial solid tumor in childhood. Alex’s Lemonade Stand Foundation (ALSF) has helped power research that is getting us closer to cures every day. Researchers like Dr. John Maris from the Children’s Hospital of Philadelphia (CHOP) and Dr. Katherine Matthay from the University of California San Francisco School of Medicine, who sit on the ALSF Scientific Advisory Board, are working to study what makes neuroblastoma tick — to find its Achilles heel and then go after it. 

“Your child has neuroblastoma.”

Thirty years ago, those four words came with dismal survival rates (only 10-30%). Thanks to intense research and scientific collaboration, long-term survival rates have increased to 50-60%  for children diagnosed over the age of 18 months.

However, survival rates do not tell the whole story. Stage of disease, age at diagnosis and several other factors can affect outcome. In addition, some current treatments can leave children with long-term side effects impacting their quality of life and the long-term health of survivors. 

There is still more work to be done. ALSF-funded researchers are working to better understand neuroblastoma and learn its true Achilles heel — the very thing that cause and power the disease, so doctors can stop it. 

Understanding Neuroblastoma
Neuroblastoma is the most common extra-cranial solid tumor childhood. The disease arises in the peripheral sympathetic nervous system — the part of the nervous system outside of the central nervous system (the brain and spine) which  releases adrenaline. It is most commonly diagnosed in young children and infants; although it can arise in older children and adults. 

No two neuroblastoma stories are the same — just ask some of our neuroblastoma heroes. It can range from very benign to very malignant, with about half of the cases of neuroblastoma falling in the  malignant range. The disease spreads through the sympathetic nervous system leaving solid tumors in the abdomen, adrenal gland, the sympathetic nerves next to the spine and anywhere it can.

Ten years ago, the standard of care included surgery, chemotherapy, stem cell rescue, radiation and Accutane at the end of therapy to prevent relapse. Five years ago, the standard of care changed only by increasing the intensity of these treatments. Fast forward to today, and treatment is becoming more targeted with the inclusion of immunotherapy for high-risk neuroblastoma once the disease is in remission.

Immunotherapy brings the most promise for cures. Dr. John Maris, an oncologist from the Children’s Hospital of Philadelphia (CHOP) and a member of the ALSF Scientific Advisory Board says the current immunotherapy protocols prove it can work, but there is more to be done to improve effectiveness and decrease side effects, which can range from flu-like symptoms to severe pain throughout the body. 

Accelerating Towards Breakthrough Treatments

Research, says Dr. Maris is now focusing itself on finding the Achilles heel of neuroblastoma.

“We are working to figure out the genetics of why neuroblastoma happens. And then we ask the question: once it arises why do some become more malignant?” said Dr. Maris.

The current research builds on past discoveries and breakthroughs, such as the discovery of the MYCN amplification, which occurs in lower risk neuroblastoma. Dr. Katherine Matthay, from the University of California San Francisco School of Medicine and member of the ALSF Scientific Advisory Board, explained that this discovery has enabled children battling lower risk neuroblastoma to avoid intense treatments with severe side effects, while still realizing a 95-percent long term survival rate. This same discovery has also allowed doctors to intensify treatments for high-risk neuroblastoma. 

The discovery of ALK-gene driven neuroblastoma has also led to cures for some patients who are given ALK inhibitors

Dr. Matthay says efforts are ongoing in the U.S. and Europe to further improve immunotherapy with CAR-T cell, vaccine or natural cell therapy.  

Dr. Maris says that more hope lies in continuing to sequence the neuroblastoma genome, studying and finding all the genetic mutations and using this information to create personalized treatment approaches.

“We have several important new clues to personalized treatment approaches being tested in the clinic through some of the projects that ALSF funds,” said Dr. Maris

Since 2005, ALSF has funded over 690 multi-year research grants in the U.S. and Canada. Learn more about ALSF funded research, including the breakthrough ALK-gene therapy that saved Edie Geiger’s life here

February 2, 2017

by Lisa Metzger, Hero Mom

Lisa Metzger, Hero Mom to Caroline, shared her story with us. When Caroline was just 6-months-old, she was diagnosed with neuroblastoma, a type of childhood cancer that often occurs in infancy. Caroline is now a wild and fun loving 3-year-old and a big sister to her little brother "Super Sib" Stephen.  Keep reading to learn more about Lisa, her daughter and how your support of ALSF is truly helping get us closer to cures for childhood cancer. 

It wasn’t until October 2013, that Alex Lemonade Stand entered “my reality.”  It seemed to be a typical Monday afternoon.  I was getting ready to lie down to sleep before my overnight shift at 7pm.  I was working as a nurse at a local hospital at the time.  My six month old daughter, Caroline, was napping beside me in her pack and play when she began to cough.  I didn’t think much of it because she had been congested for a few days, but it started getting worse.  I attempted to clear her nose with a bulb syringe, but it didn’t seem to work.  Then, suddenly, she stopped breathing completely.

After three attempts to call 911, I was finally able to feel my fingers enough to dial. An ambulance was on the way.  I began CPR and was able to revive her, but it was only a matter of time before she would stop breathing again.  I was in a shock.  Why would Caroline stop breathing?  It couldn’t really be happening.  EMTs arrived and transported Caroline to the closest children’s hospital. Once a breathing tube was secured, I was able to take what seemed like my own first breath since I made the phone call to paramedics. 

In the emergency department, Caroline had blood work, x-rays, a CT scan and was quickly transferred to the Pediatric Intensive Care Unit (PICU). Doctors ordered an MRI and within a few hours, on what started out as a typical day, my husband and I were delivered the worst news of our lives. Our daughter had cancer— a mass in her chest and neck that compressed her airway.

We decided to transfer Caroline’s care to the Children’s Hospital of Philadelphia (CHOP). We waited 16 hours for the transfer and at 4:30 am, we began our journey.  

It wasn’t until our daughter was admitted to the PICU at CHOP, that she was formally diagnosed with neuroblastoma. Within four hours of transfer, Caroline was in the operating room for a port placement, bone marrow biopsy and tumor biopsy. She received her first dose of chemotherapy later that evening; just 48 hours after we first heard the word “cancer.”  

Caroline spent a little over four weeks in the PICU. She received her first two rounds of chemotherapy to shrink the tumor just enough for the team to remove her breathing tube. She received a total of eight rounds of chemotherapy, the last one finishing around the week of her first birthday. I cannot remember every detail during that time. There were ups and downs and ins and outs. I will never forget how I felt when I heard the news she had cancer. And I will never forget how I felt when I heard the words, “but we have a treatment for it.” The darkest hours of my life had a spark.

We were beyond pumped and ready to fight. Caroline had a team of physicians who continued to reassure our hopes for Caroline’s recovery.

While my husband and I spent every moment focused on Caroline’s recovery and treatment plan, both of our families were researching Alex’s Lemonade Stand Foundation (ALSF). 

Our extended family felt helpless in a situation that was completely out of their hands. ALSF offered them information on her type of cancer as well as a way to come together and rally their bottled up emotion and energy towards the benefit of childhood cancer research. They created a team in honor of Caroline for the 2013 Lemon Run, and managed to raise $8,000 in just a few short weeks.  

It took me a while—until Caroline was done treatment—to be able to fully understand the magnitude of ALSF and its impact on Caroline’s experience.  

When I was delivered the news of her diagnosis, the first thing I thought was that she was going to die. But then the doctors sat down with my husband and I and informed us they had a roadmap of chemotherapy to treat her type of cancer.  They were passionate. We were fortunate for her team. After further genetic testing of the tumor, they assured me that she would beat it.  She would beat it and they were confident of it.  

Their confidence was contagious, but I wondered what made them so confident? Their answer was: “research.” I believe 13 years after her first lemonade stand, Alex Scott was somewhere close watching this interaction and smiling, knowing her legacy was growing strong. 

Alex’s vision was to live in world without pediatric cancer. Her mission is thriving today thanks to her parents and the amazing staff of ALSF. The Foundation is an educational and financial resource for families as well as a social support for siblings affected by cancer. They not only deal with the logistics of finding cures and treatments for childhood cancer, but they have also developed a positive culture between hero families (those on the front lines) that is deeply compassionate and encouraging.

Today’s pediatric cancer treatment is always improving because of Alex’s legacy: my own daughter, Caroline, is living proof.