Shoulder Desmoid Tumors: More Symptoms, No EFS Site Difference

Key Takeaways

• In a nationwide French cohort, shoulder desmoid-type fibromatosis was associated with more frequent baseline pain than head-and-neck or other-site disease.
• Functional impairment at presentation was significantly more frequent with shoulder tumors than with tumors at the comparison sites.
• Overall event-free survival did not differ significantly by anatomical site.

How do head-and-neck and shoulder desmoid-type fibromatosis present and progress compared with tumors at other locations? Head-and-neck and shoulder tumors were compared with tumors arising elsewhere in a French cohort.

Patients in the ALTITUDES study were followed in a prospective, nationwide observational cohort in France. Among newly diagnosed patients enrolled from January 2016 through February 2021, 610 had a known tumor location, including 26 with head-and-neck and 28 with shoulder disease; diagnoses underwent central pathological confirmation. Baseline symptoms were assessed only among patients enrolled within six months of diagnosis. Initial approaches included surveillance, surgery, and systemic therapy, without assigned treatment. Event-free survival (EFS) counted local relapse after complete or microscopically incomplete resection, investigator-assessed progression after surgery leaving gross residual disease, surveillance, or systemic treatment, and death from any cause. Patients starting second-line treatment for symptoms without documented relapse or progression were censored. Patients initially surveilled were also analyzed separately.

Among patients assessed soon after diagnosis, baseline pain was reported in 62% of shoulder cases, 53% of head-and-neck cases, and 33% of other-site cases, a statistically significant difference by location. The comparison captured pain around enrollment rather than pain emerging later during management.

Baseline functional impairment affected 29% of shoulder cases, 0% of head-and-neck cases, and 6% of cases elsewhere, also a statistically significant site difference. Initial active surveillance was more frequent for shoulder than head-and-neck disease, indicating that management patterns varied alongside baseline symptoms.

Across initial management approaches, three-year EFS was 61.9% for head-and-neck disease, 79.7% for shoulder disease, and 65.4% for disease elsewhere. The between-site difference was not statistically significant; these estimates reflect outcomes under differing initial approaches rather than an untreated disease course.

The head-and-neck and shoulder groups were small, and management was heterogeneous and nonrandomized. Head-and-neck subsite descriptions were sometimes imprecise, while the time between first symptoms and diagnosis was unavailable. Composite EFS cannot establish equivalent disease courses across locations or characterize untreated growth, particularly because symptom-driven second-line treatment without documented progression or relapse was censored. According to the authors, shoulder tumors presented with more pain and functional impairment, while EFS differences by site were not detected overall or among patients initially managed with surveillance.

Clinician Questions

How was baseline pain assessed in patients with head-and-neck or shoulder desmoid-type fibromatosis?

Patients with head-and-neck or shoulder desmoid-type fibromatosis in ALTITUDES self-assessed baseline pain using a yes/no question and a numeric rating scale.

Did the quality-of-life questionnaire fully capture functional impairment in desmoid-type fibromatosis?

The authors considered the generic EORTC QLQ-C30 quality-of-life questionnaire less sensitive to functional impairment in desmoid-type fibromatosis than investigators’ assessments in ALTITUDES.

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