Facial Desmoid Tumor in a 2-Year-Old

Summary

A 2-year-old male presented to the emergency department with a rapidly growing right facial mass. Imaging revealed a 6 × 4 cm enhancing mass centered within the right parapharyngeal space with extensive local tissue compression and osseous remodeling. The patient underwent transoral biopsy that demonstrated both spindle and rhabdoid cells with mitotic figures. Immunostaining was positive for desmin, actin, and beta-catenin, suggesting a desmoid tumor. Given the extent of the tumor and its location, the patient was initiated on a weekly regimen of vinblastine and methotrexate due to concerns for serious cosmetic and functional morbidity with surgery. Over the next 4 weeks, there was an interval increase in size by ?25%, primarily at the biopsy site, and the patient became increasingly symptomatic with intermittent bleeding and episodes of upper airway obstruction. This prompted surgical intervention by a multidisciplinary team comprised of oral-maxillofacial surgery, otolaryngology—head and neck surgery, and microvascular reconstructive surgery. Reconstruction was discussed at length, and a decision was made to defer definitive bony reconstruction because of growth concerns and lack of an adequate anatomic replacement for the mandibular ramus at this age. At completion of surgery, the patient’s face was symmetric with complete oral competence. The postoperative course was uncomplicated with no evidence of recurrence at 1 year. His maximal incisal opening and speech are excellent. He continues to do well, is taking an oral diet, and is pending decannulation in the near future.