Neuroblastoma Diagnosis and Treatment Fail to Sap Spirit of Young Patient

On the desk of Massachusetts General Hospital pediatric surgeon Danielle Cameron, MD, MPH, sits a small cactus. It was a gift from the family of one of her patients, six-year-old Theo Divoll. 

At age five, Theo was diagnosed with high-risk neuroblastoma. It is a form of cancer that grows in the nerve tissue of babies and young children. Treatment can be grueling and involve chemotherapy, surgery, stem cell transplant, radiation therapy, and immunotherapy. 

In spite of all he has been through, Theo has maintained a fierce determination and positive attitude.

"Theo's mother wrote a note saying she hopes that when I look at the cactus, I'll think of him and his treatment journey," Dr. Cameron said. "It can be prickly, but every now and then it blooms a flower." 

Broken leg leads to neuroblastoma diagnosis

Theo lives in Andover, Massachusetts, with his parents, Paige and Ira, and brother and sister. One day, Theo broke his leg while skiing. After getting a cast, he started experiencing fatigue, loss of appetite, a swollen abdomen, and weight loss. 

Paige took Theo to their pediatrician, who noticed Theo's blood pressure was alarmingly high. The doctor sent him directly to the emergency room at Mass General for Children (MGfC).

Tests showed that Theo had neuroblastoma, one of the most common solid tumors found in young children. A large tumor, which had started in his abdomen, had already spread to his bones and wrapped around his right kidney. 

"We treat children who have high-risk neuroblastoma with an aggressive regimen to kill all the tumor cells and minimize their risk of local recurrence or long-term relapse," Dr. Cameron said. "Their bodies can physically tolerate a lot more than an adult patient."

It is crucial for children with high-risk neuroblastoma to receive care from a multidisciplinary team of experts. Dr. Cameron has extensive experience in surgically treating neuroblastoma. Her colleague, pediatric medical oncologist Lauren Boal, MD, trained under one of the country's leading neuroblastoma experts. 

Before joining MGfC, Dr. Boal helped lead a clinical trial focused on pediatric solid tumors, including neuroblastoma. She now cares for children with neuroblastoma and other cancers. In Theo's case, she designed and managed the inpatient and outpatient care plans, which spanned chemotherapy, two stem cell transplants, and immunotherapy. 

"We knew he had a long and intense road ahead," Dr. Boal said. "I was worried about him, but I felt like we could get his blood pressure under control and then start the road to curing his body of neuroblastoma." 

Theo's initial workup included an admission to the MGfC Pediatric Intensive Care Unit (PICU), where his care team gave him medications to control his blood pressure. He also underwent imaging and tumor biopsy to confirm the diagnosis. 

Next, Theo was transferred to the MGfC inpatient pediatric floor on Ellison 17, where he began chemotherapy. The goal was to kill as much of the cancer as possible before having an operation to remove the tumor. 

"Kids are so resilient. His hair started coming out, but he just kind of shrugged and moved on," Paige said. "Then Ira said, 'Let's go for it—why don't we just shave it?' Ira has very short, cropped hair, and Theo was excited to look just like his dad." 

Prior to surgery, Theo completed several rounds of chemotherapy with good response. He also underwent stem cell harvesting, a process that involves collecting stem cells (special cells with the ability to turn into many different cell types). The stem cells were frozen and stored so they could be transplanted back into Theo's body later.

Surgery to treat neuroblastoma is notoriously challenging. As Dr. Cameron explained, "This is a tumor that tends to grow around blood vessels. Removing the tumor can be high-risk because injury to any of these blood vessels can cause harm to other organs." 

For this reason, it's not always possible to remove the entire tumor with surgery. In many cases, removing 90% of the tumor is considered a success because the remaining tissue can be treated with chemotherapy, stem cell transplant, radiation therapy, and immunotherapy. 

In Theo's case, the tumor completely encased the blood vessels going to his right kidney. During a 12-hour operation that she performed with pediatric surgeon Daniel Ryan, MD, Dr. Cameron carefully carved the tumor off of the blood vessels. In addition to removing over 95% of the tumor, she managed to preserve both the blood vessels and the kidney. 

Paige and Ira appreciated the depth of Dr. Cameron's experience with neuroblastoma surgery as well as her caring, compassionate bedside manner. 

"She gave us the feeling that she would treat our child as her own," Paige said. "She took so much time to get to know our family and went the extra mile to make a really scary process for Theo a little less scary for all of us."

Surgery was the most critical milestone in Theo's neuroblastoma treatment—but hardly the endpoint.

About a month after surgery, Theo was readmitted to MGfC for a five-day course of high-dose chemotherapy. That was followed by a stem cell transplant, in which Dr. Boal and the oncology team injected the stem cells that had been collected several months earlier back into his body. There, they would travel through the bloodstream and into the bone marrow, where they would begin making new white blood cells, red blood cells, and platelets. 

At the time of the transplant, Theo's immune system barely functioned. To guard against infection for the next month, he was almost completely isolated in his hospital room while dealing with nausea and other side effects. It was an extremely difficult stretch for the young boy, physically and mentally. 

The second stem cell transplant, which started the day after Thanksgiving, took an even greater toll. "That was a grueling month; the things he endured were unbelievable," Paige said. "But because he was so heavily sedated to treat the pain, he thankfully doesn't remember most of it." 

Much to his family's delight, Theo was discharged and able to return home on Christmas Eve. He had a month to recover in preparation for the next step in his treatment: an advanced form of radiation therapy called proton beam therapy.

Proton therapy delivers a high-energy, focused proton beam to the tumor. Unlike traditional radiation therapy, it does not damage healthy tissue near the tumor if administered properly. 

"Proton therapy can be a better form of radiation in cases where you're trying to protect organs that are close to the tumor site, like the kidneys, liver, or bladder," Dr. Boal said. "This is particularly important with young kids, who have the rest of their lives in front of them." 

Pediatric radiation oncologist Torunn Yock, MD, oversaw Theo's proton therapy. The two-and-a-half-week treatment took place on an outpatient basis at Mass General, home of the only proton therapy site in New England. 

Soon after completing proton therapy, Theo started immunotherapy, a type of cancer treatment that uses the body's immune system to find and attack cancer cells. The first five rounds each required a week-long stay in the PICU. For the final round, he was able to take medications at home. The entire course of immunotherapy took about six months. 

About 16 months after Theo's cancer journey began, scans showed no evidence of cancer in his body. However, high-risk neuroblastoma returns in roughly half of children who are successfully treated. Theo thus will require ongoing monitoring at MGfC for several years. 

"I can't imagine what these families go through and how they manage to put one foot in front of the other," Dr. Boal said. "But Theo has shown such resilience, and Paige and Ira are truly impressive parents. Our hearts are with the family, and our fingers crossed." 

As Theo's parents have seen firsthand, caring for children with neuroblastoma demands multidisciplinary collaboration. In addition to Drs. Cameron, Boal, Ryan, and Yock, Theo's care team has included pediatric experts in psychiatry, nephrology, pulmonology, anesthesiology, nutrition, palliative care, and other specialties. 

"Everyone without fail has been compassionate, caring, friendly, and invested in our journey," said Paige, who somehow found the time to train for and run the 2024 Boston Marathon to raise funds for pediatric care at MGfC. "We never walk through the hospital without someone stopping to say hello to us." 

The care Theo has received hasn't been purely medical in nature. He has also received a wide range of support services and programs, from music, art, and massage therapy to child life and social services. Even the parking staff, Paige noted, have gone above and beyond. 

"I can't imagine that we'd have had the same kind of personal experience anywhere else," Paige concluded. "It's remarkable how many people across the hospital have stepped up for us, and I'm grateful for it."