(423) Reconsidering Surgery in Pediatric Head and Neck Desmoid Tumors

Introduction: Desmoid fibromatosis and desmoplastic fibroma are low grade sarcomas that are rare in children, but are often found in the head/neck. Most relevant series in the head/neck present heterogenous treatment approaches across multiple centers over large time periods. Due to a reported 50% recurrence rate irrespective of surgical margins and the morbidity of complex head and neck resections in children, treatment paradigms have varied widely with recent emphasis on non-operative management and targeted therapy. We report our relatively large surgical experience in pediatric head and neck desmoid tumors treated by a single team over a 6-year period with an emphasis on surgical decision making.

Methods: Retrospective analysis of desmoid tumor patients treated by a single pediatric head and neck tumor team from 2016 to 2022.

Results: 10 patients (6 female) averaging 5.62 years (1.1 – 17.7 years) were treated for desmoid tumors (5 mandible, 3 mandible/infratemporal fossa, 1 scalp, 1 maxilla). Four patients with tumor isolated to the mandible body/ramus or scalp underwent primary surgery. The other 6 patients received neoadjuvant chemotherapy, some after an interval of initial observation, and went on to planned surgery. With an average follow up of 2.59 years (1.03-6.41, SD 1.7), only 1 patient (10%) had recurrences, at 7 months and again at 2 years, in separate subsites.

Conclusions: When treated with oncologic surgical principles by a consistent and experienced team, lower recurrence rates than previously reported can be achieved for pediatric head and neck desmoid tumors. Surgery should still be considered in multidisciplinary treatment paradigms with adjuvant therapies focused on optimizing resectability and minimizing morbidity.