Reese Aquilio – Rare Carcinoid Tumor

The pain was overwhelming. It had come and gone in Reese Aquilio’s chest and back for several months when she played soccer, but during this big match it was much more intense. Later that night, the pain became unbearable. “She couldn’t breathe and her chest was really tight. She was up all night in pain,” her mother Lesley Aquilio says through tears, recalling that night. “I knew something was wrong.”

At 13, there was little reason to suspect a dangerous carcinoid cancer more commonly found in adults was slowly growing in Reese’s lungs, choking the life out of her. Once it was discovered, pediatric specialists at UPMC Children’s Hospital of Pittsburgh employed first-ever technology and a comprehensive team approach to remove the tumor and help Reese return to the sport she loves.

As an avid athlete and year-round soccer player, the Norwin Middle School student was accustomed to pushing her body to the limit. So, when an initial x-ray in November 2016 showed a diagnosis of pneumonia, she took it in stride — using antibiotics prescribed by her primary care physician and resting until she felt better. When she returned to her soccer workouts, the pain came back, too.

Following that night of unbearable pain in March 2017, Reese’s grandmother took her back to the primary care physician, who suggested a second x-ray. “As soon as I got home from work I took her for the x-ray. I just felt like something was not right. Call it mother’s intuition,” says Mrs. Aquilio.

“The next day her pediatrician called and said, ‘This is not pneumonia. I don’t know what it is, but I’m calling Children’s Hospital to get her in for an appointment as soon as possible,’” she says. At Children’s she underwent a computed tomography scan of the chest on April 18, and there it was: a mass in the lung, on the right main bronchus, a major airway.

Age-appropriate care

Carcinoid tumors, made up of neuroendocrine cells, rarely occur in the lungs of children, but are more often found in adults with lung cancer. “If caught early enough it is very survivable, but late-stage disease is incurable,” says Louis Rapkin, MD, clinical director of Oncology at Children’s. “If the tumor blocks the bronchial tube, the mass can cause pneumonia and other damage to the lung. If it spreads, there is little chemotherapy or other treatments can do to cure it.”

The Adolescent and Young Adult Oncology Program in Children’s Division of Hematology/Oncology offers specialized oncology and support services to meet the needs of children in their teens through young adults up to age 26. The program features a comprehensive approach to care that enables patients to be treated by pediatric oncologists in collaboration with adult-focused oncologists, when appropriate. Rather than simply requesting a consultation via telephone or computer, additional experts can be brought to Children’s Hospital in Lawrenceville.

“We are better geared to treat adolescents and young adults because we also take into account their developmental stage to make the experience more manageable for the patient. Even young adults are still developing in terms of their ability to cope with trauma. We account for their physical and psychological needs,” says Dr. Rapkin.

Reese’s team of physicians included Dr. Rapkin and Stefan Scholz, MD, PhD, a general and thoracic pediatric surgeon and Director of Minimally Invasive Surgery at Children’s. To give her the best chance possible, they collaborated with a thoracic surgeon at UPMC Hillman Cancer Center who has experience treating adult patients with carcinoid lung cancer.

“It was a very tricky mass requiring high-end thoracic surgery,” says Dr. Scholz. “The ability to bring in someone who has a lot of experience with carcinoid lung cancer was a great asset.”

Pioneering radiology

On April 27, Reese’s rare diagnosis was confirmed by something even rarer: positron emission tomography (PET) imaging using a Gallium 68 dotatate injection. The radioactive diagnostic agent is a less-invasive alternative than biopsy to help pinpoint carcinoid tumors. Although it has been used in other parts of the world for many years, it wasn’t approved by the U.S. Food and Drug Administration until June 2016. Reese became the first patient at Children’s Hospital — and the first pediatric patient in the nation — to undergo a dotatate PET scan.

“We were nervous about Reese being the first child to have the dotatate PET scan, certainly as anyone would be scared, but we knew we needed to have the right diagnosis and we wanted to avoid an invasive biopsy. We needed the team of doctors to have the most up-to-date and accurate information possible. So we were willing to do whatever it takes to make sure everything went well for Reese,” says Mrs. Aquilio.

“After the test, the technician handed Reese a stuffed animal and said to her, ‘Not only are you helping yourself, you are helping many other children.’ I think that made her feel powerful to know she was helping others by being the first child to undergo the scan,” Mrs. Aquilio adds.

Bilobectomy

While doctors carefully planned Reese’s surgery, she fought through the pain in order to try to find a sense of normalcy. She continued to play soccer, participated in a chorus concert, and attended her school picnic at Kennywood amusement park. On June 2, everything was ready.

Doctors had hoped to remove only one of the three lobes of the right lung. “The mass was located at the root of the right lung at and within the division of the main bronchus to the middle and the lower lobes,” says Dr. Scholz. During the four-hour surgery, doctors made the decision to remove the two lobes — a bilobectomy — in order to assure complete removal of the tumor with a “negative margin” of healthy surrounding tissue, the key factor for a good long-term prognosis.

Reese did very well following the procedure and was discharged from the hospital six days after surgery, determined to get back on track with soccer. Within a month, she started speed and strength conditioning. After two months, she began one-on-one footwork training with her coach. She joined her soccer team for its first practice of the season in August. “She’s still working to get back to the skill level she had prior to the diagnosis. She is determined not to let this hold her back,” says her mother.

“If you take out the lower and middle lobes of the lung, initially you feel it,” says Dr. Scholz. “But in the eight weeks after surgery, Reese trained herself to approach the level of activity that she had before.”

In Reese’s case, chemotherapy and radiation treatments were not necessary. Doctors will monitor her progress over the next five years with visits every four to six months that include physical examinations, chest x-rays, and pulmonary function tests.

Lesley Aquilio and her husband, Tony Aquilio, who live in North Huntingdon with Reese and her two younger sisters, Alyssa and Natalie, credit the medical professionals at Children’s Hospital for the high level of care and attention the whole family received during Reese’s treatment. “We felt like we were living a true nightmare, and everyone at Children’s made this process much easier,” says Mrs. Aquilio. “Our only option was to put our trust and faith in the professionals working with Reese. Everyone we encountered in the hospital and at all of the appointments has been nothing short of spectacular.”