DOI: 10.1002/cam4.72302 ISSN: 2045-7634

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

Nicolas Penel, Sylvie Bonvalot, Julien Elices‐Diez, Antoine Italiano, Benjamin Verret, Jean‐Emmanuel Kurtz, Pascale Dubray‐Longeras, Cécile Guillemet, Florence Duffaud, François Bertucci, Julien Thery, Marie Vanseymortier, Emmanuelle Bompas, Nelly Firmin, Sixtine De Percin, Jennifer Wallet, Sophie Piperno‐Neumann, Thibaud Valentin, Jean‐Yves Blay, Marie‐Cécile Le Deley
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ABSTRACT

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

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