Head & Neck and Shoulder Desmoid-Type Fibromatosis: Presentation and Natural History of These Ultra-Rare Locations.

BACKGROUND

Active surveillance is recommended as the first-line management for desmoid-type fibromatosis (DF); however, data on DF of the head and neck (H&N) and shoulder regions are limited.

METHODS

We examined patients with DF located in the H&N and shoulder regions, comparing baseline characteristics and event-free survival (EFS). A subgroup analysis was performed among patients initially managed with active surveillance.

RESULTS

H&N accounted for 4% of all DF cases, and shoulder accounted for 5%. Pain (62% vs. 53% and 33%, respectively; p = 0.01) and functional impairment (29% vs. 0% and 6%, respectively; p = 0.002) were significantly more frequent in shoulder DF compared with H&N DF and DF at other sites. Regardless of initial management, the 3-year EFS rates for H&N and shoulder were similar to those for other locations (p = 0.74). Among patients initially managed with active surveillance, the 3-year EFS rates for H&N and shoulder were similar to that for DF at other locations (p = 0.63).

CONCLUSION

Pain and functional impairment differed significantly by location; however, the outcomes of both H&N and shoulder DF did not differ significantly from those of DF at other locations.

TRIAL REGISTRATION: This investigation was registered under the clinical trial registration number NCT02867033.