OP217: Management of pediatric desmoid tumors of the head and neck

Abstract

Purpose Pediatric desmoid tumors (aggressive fibromatosis) of the head and neck are extremely rare clinical entities. The literature available to guide the management of this uncommon neoplasm in children is scarce. The goal of this retrospective analysis of three cases of pediatric aggressive fibromatosis is to begin to outline a successful treatment algorithm for the management of pediatric aggressive fibromatosis and contribute to the literature regarding the occurrence and treatment of this pathologic entity. Materials and Methods A retrospective chart review of three pediatric patients was conducted with meticulous analysis of initial presentations, aggressiveness of desmoid tumor, age, work-up, treatment rendered, any treatment complications, and treatment response with respective post-operative surveillance. The decision algorithms regarding non-surgical treatment modalities, extent of surgical resection, and choice of reconstructive strategies were reviewed and analyzed in detail. Results Two of the three patients received neo-adjuvant chemotherapy and adjunctive therapies which failed to provide any treatment response. Their tumors continued to rapidly proliferate through neoadjuvant therapy. Each of the three patients received a wide radical excision with concomitant reconstruction which provided acceptable source control. The most efficacious treatment outcome was present with aggressive surgical intervention regardless of neoadjuvant chemotherapy or adjunctive therapies. Radiotherapy was not used in this pediatric population secondary to the dose-dependent side effect profile including mucositis, xerostomia, osteoradionecrosis, growth retardation of the craniofacial skeleton, and primary sarcoma and papillary thyroid cancer formation later in life. Conclusion Aggressive wide radical excision with appropriate osteomyocutaneous reconstruction of pediatric desmoid tumors of the head and neck should remain the primary treatment modality with likely minimal contribution of neoadjuvant chemotherapy and adjunctive therapies due to the specific aggressiveness of these tumors in early childhood. Additional adjunctive therapies can have potentially devastating side effects especially in young patients while their effectiveness cannot be demonstrated.