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Peer-reviewedCC BY-NC-NDCancer medicine

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

  • Nicolas Penel1,2
  • Sylvie Bonvalot3,4
  • Julien Elices-Diez5
  • Antoine Italiano6
  • Benjamin Verret7
  • Jean-Emmanuel Kurtz8
  • Pascale Dubray-Longeras9
  • Cécile Guillemet10
  • Florence Duffaud11
  • François Bertucci12
  • Julien Thery5
  • Marie Vanseymortier5
8 more authors
  • Emmanuelle Bompas13
  • Nelly Firmin14,15
  • Sixtine De Percin16
  • Jennifer Wallet5
  • Sophie Piperno-Neumann17,18
  • Thibaud Valentin19
  • Jean-Yves Blay20
  • Marie-Cécile Le Deley5
20 affiliations
  1. 1Department of Medical Oncology, Centre Oscar Lambret, Lille, France.
  2. 2CHU Lille, ULR 2694-Metrics: Evaluation des Technologies de Santé et des Pratiques Médicales, Univ. Lille, Lille, France.
  3. 3Department of Surgical Oncology, Institut Curie, Paris, France.
  4. 4Université Paris Sciences et Lettres, Paris, France.
  5. 5Clinical Research Department, Centre Oscar Lambret, Lille, France.
  6. 6Department of Medical Oncology, Institut Bergonié, Bordeaux, France.
  7. 7Department of Medical Oncology, Gustave Roussy, Villejuif, France.
  8. 8Department of Medical Oncology, Strasbourg University Hospital, Strasbourg, France.
  9. 9Department of Medical Oncology, Centre Jean Perrin, Clermont-Ferrand, France.
  10. 10Medical Oncology Department, Centre Henri Becquerel, Rouen, France.
  11. 11Medical Oncology Department, Public Assistance-Hospitals of Marseille, La Timone Hospital, Marseille, France.
  12. 12Medical Oncology Department, Institut Paoli-Calmettes, Aix-Marseille Univ., Marseille, France.
  13. 13Medical Oncology Department, Centre René Gauduchau, Saint Herblain, France.
  14. 14Department of Medical Oncology, Institut de Cancérologie de Montpellier, Montpellier, France.
  15. 15INSERM U1194, IRCM, Univ. Montpellier, Montpellier, France.
  16. 16Department of Medical Oncology, AP-HP, Cochin Hospital, Paris, France.
  17. 17Department of Medical Oncology, Institut Curie, Paris, France.
  18. 18INSERM U830, Institut Curie Research Center, Paris, France.
  19. 19Department of Medical Oncology, Institut Claudius Regaud and Institut Universitaire de Cancerologie, Oncopole, Toulouse, France.
  20. 20Department of Medical Oncology, Centre Léon Bérard, Université Claude Bernard, Lyon, France.
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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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Cite

BibTeXRIS
BibTeX
@article{penel2026head,
  title = {{Head & Neck and Shoulder Desmoid-Type Fibromatosis: Presentation and Natural History of These Ultra-Rare Locations.}},
  author = {Nicolas Penel and Sylvie Bonvalot and Julien Elices-Diez and Antoine Italiano and Benjamin Verret and Jean-Emmanuel Kurtz and Pascale Dubray-Longeras and Cécile Guillemet and Florence Duffaud and François Bertucci and Julien Thery and Marie Vanseymortier and Emmanuelle Bompas and Nelly Firmin and Sixtine De Percin and Jennifer Wallet and Sophie Piperno-Neumann and Thibaud Valentin and Jean-Yves Blay and Marie-Cécile Le Deley},
  journal = {Cancer medicine},
  year = {2026},
  month = oct,
  doi = {10.1002/cam4.72302},
  url = {https://europepmc.org/article/MED/42905937},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - Head & Neck and Shoulder Desmoid-Type Fibromatosis: Presentation and Natural History of These Ultra-Rare Locations.
AU  - Nicolas Penel
AU  - Sylvie Bonvalot
AU  - Julien Elices-Diez
AU  - Antoine Italiano
AU  - Benjamin Verret
AU  - Jean-Emmanuel Kurtz
AU  - Pascale Dubray-Longeras
AU  - Cécile Guillemet
AU  - Florence Duffaud
AU  - François Bertucci
AU  - Julien Thery
AU  - Marie Vanseymortier
AU  - Emmanuelle Bompas
AU  - Nelly Firmin
AU  - Sixtine De Percin
AU  - Jennifer Wallet
AU  - Sophie Piperno-Neumann
AU  - Thibaud Valentin
AU  - Jean-Yves Blay
AU  - Marie-Cécile Le Deley
PY  - 2026
DA  - 2026/10/01
JO  - Cancer medicine
DO  - 10.1002/cam4.72302
UR  - https://europepmc.org/article/MED/42905937
AB  - 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.
SN  - 2045-7634
ER  -