Surgical Resection of Rare Pediatric Desmoid Jaw Tumor Optimizes Outcome and Helps Patient Avoid Chemotherapy

This Clinician's View is written by Joseph Lopez, MD, MBA, Chief of Pediatric Head & Neck Surgery at AdventHealth for Children.

When he was only 18 months old, Caden Flores developed an aggressive fibromatosis (desmoid tumor) on his lower jaw. A rare diagnosis, especially at this age, the condition was already causing him pain and negatively impacting function. Living in Rutgers, New Jersey, Caden’s parents consulted with two different children’s hospitals in the Northeast on the best treatment plan. They were advised that based on the size and location of Caden’s tumor, a two-year course of chemotherapy was their only option. Unfortunately, that medical therapy for this type of tumor, while commonly used, has limited efficacy. Often, it doesn’t shrink the tumor and only keeps it from growing larger.

Concerned about the low efficacy and negative impact chemotherapy could have on Caden’s quality of life, his parents decided to consult another pediatric surgeon in their hometown who referred them to me for another opinion. As it turned out, that surgeon and I had been fellows together at Memorial Sloan Kettering in New York – he in pediatric surgery, and I in head and neck oncology. He knew of my focused interest and experience in improving care for pediatric patients with head and neck tumors. After reviewing the case, I felt confident that our highly specialized pediatric head and neck surgical team at AdventHealth for Children could completely remove the tumor, allowing Caden to quickly heal and recover without the long-term burden and risks of chemotherapy.

After a couple of weeks of intensive planning and preparation, including development of 3D models and guides, we performed the procedure in April 2025 and achieved negative margins. Our amazing multidisciplinary clinical team then provided Caden with comprehensive rehabilitative care for about a month in our pediatric intensive care unit (PICU) to help him heal and recover. I’m thrilled to report that we discharged Caden in early May, and he has returned home to Rutgers where his mom reports he is an active, happy two-year old -- smiling, laughing, playing and discovering new things every day.

Pediatric Desmoid Tumors – A Difficult Diagnosis to Treat
Although benign, desmoid tumors, also known as desmoid-type fibromatosis, are locally aggressive and can be invasive. Unlike cancer, they don’t metastasize, but they can grow into surrounding tissues and organs, causing major morbidity, including pain and functional limitations. Additionally, these rapidly growing tumors have a high rate of recurrence, especially if resection does not yield negative margins. While relatively rare in the pediatric population, head and neck desmoid tumors can occur and require extra special attention because of their proximity to vital structures and potential to interfere with normal anatomical growth and development.

The Unique Challenges of Caden’s Case
Caden’s desmoid tumor, located on the inner aspect of his lower jaw and measuring 5 cm, made his case especially unusual and difficult for many reasons:

• The anatomic location compromised several key functions, including his ability to chew and eat normally on that side.
• The tumor was already impacting his smile because it sat very close to the main trunk of the facial nerve branch.
• It was infiltrative in nature, invading adjacent structures, which created challenges in determining a good margin for surgical resection.

Furthermore, only a small number of head and neck surgeons have experience removing jaw tumors in children under age 2. This most likely led to the initial recommendation of chemotherapy by the other providers who reviewed Caden’s case. However, that approach, in addition to its previously mentioned low efficacy, came with a slew of challenges and risks, both short-term and long-term:

• Typical side effects, including fatigue and nausea, reduce quality of life.
• There is a tremendous treatment burden on the patient and family with multiple required infusions each month.
• Chemotherapy carries long-term risk of infertility.
• It would compromise Caden’s immune system, predisposing him to infections that his body could otherwise fight off.
• Feeding issues, which were already occurring, could become worse if the tumor didn’t shrink or did so very slowly over the treatment period. This could necessitate placement of a gastrostomy tube (G tube) to ensure Caden was nutritionally optimized.
• A 24-month course of chemotherapy would mean Caden wouldn’t finish treatment until he was nearly 4 years old.

After careful review of all aspects of Caden’s case, I made the decision that with our team’s experience and expertise, surgical resection was possible and the best plan to maximize his quality of life.

Comprehensive Surgical Planning and Execution Yielded Negative Margins
We knew that our surgical intervention and post-operative management of Caden’s case needed to be strategically planned and executed, accounting for every possible contingency before we ever stepped foot in the operating room (OR). Use of computer-aided design and computer-aided modeling (CAD/CAM) helped us customize our surgical approach to Caden’s case, enhancing safety and precision. Additionally, we worked closely with our PICU Medical Director Glenn Stryjewski, MD, to ensure we had a comprehensive plan in place to keep Caden safe while his post-surgical swelling subsided and to maximize his healing and recovery. In the OR, our use of innovative intraoperative navigation technology further helped to attain pathologically negative surgical margins.

A Collaborative, Multidisciplinary Recovery Plan to Optimize Outcomes
After his surgery, Caden spent about a month in our PICU recovering, and it was a complete team effort from beginning to end:

• Because of swelling, he needed a temporary airway device initially, and pediatric ear, nose and throat (ENT) specialist Mitchell Austin, MD, managed that process, ensuring that once Caden left the hospital, he no longer needed it.
• Pediatric surgeon Chrisopher Anderson, MD, placed a temporary feeding tube percutaneously in Caden’s abdomen to divert food away from the mouth, allowing the area to heal.
• Under the leadership of Dr. Stryjewski, our entire PICU medical team was involved in Caden’s daily post-operative care and were instrumental to his successful recovery.
• Specially trained pediatric respiratory therapists helped manage his airway after the temporary device was removed.
• Our pediatric nutritionists ensured Caden was gaining weight and nutritionally optimized to promote proper healing.
• Numerous physical and occupational therapists, specialized in caring for children, worked with Caden, building his strength and getting him up and walking around again.

Caden’s Prognosis and Follow-up Care
Caden is back home now, playing and enjoying life with his family. Because his margins were all negative, he has a low chance of recurrence, and his prognosis is quite good. He will need imaging every 3 months to confirm there is no tumor. As Caden grows older and eats harder foods, he can be fitted for an obturator, a custom-fitted prosthetic device, to help restore his ability to chew better. Once he is a teenager and skeletally mature, he will require secondary dental rehabilitation to help him gain dentition on his lower jaw. Depending on how Caden’s facial bones grow, he may also need some form of corrective jaw surgery to improve his bite and ensure optimal function opening and closing his mouth.

Why Sub-Specialized Care for Pediatric Head and Neck Tumors is Important
Cases like Caden’s are why I do what I do and what spurs my ongoing passion and advocacy for advancing sub-specialized pediatric head and neck tumor care. Children are not little adults and should not be treated as such. They benefit significantly from sub-specialized care that considers their smaller anatomy and unique pathophysiology as well as their future growth and development. As one of just a few pediatric head and neck surgical oncology programs in the country, our team at AdventHealth for Children remains committed to collaborating, researching, educating and ultimately, paving a better path forward for our youngest patients.

Learn more about the AdventHealth for Children's Pediatric Head & Neck program here.