Pediatric desmoid fibromatosis of the parapharyngeal space: A case report and review of literature

Abstract

Desmoid fibromatosis, or aggressive fibromatosis, is a benign but locally infiltrative fibroblastic neoplasm arising from fascial or musculoaponeurotic tissues. Although lacking metastatic potential, head and neck fibromatosis can have significant functional or cosmetic morbidities. 7%-15% of all desmoid tumors are seen in the head and neck region, 57% of which occur in the pediatric population. The incidence of pediatric desmoid tumor peaks around age 8. Treatment of choice is complete surgical resection; however, local recurrence is common. We present a case of a 14-month-old male with an 8-cm desmoid tumor in the right parapharyngeal space and provide an overview of diagnosis and management of pediatric head and neck fibromatosis. This is the largest desmoid tumor of the parapharyngeal space in the youngest patient described in the literature.