The Health-Related Quality of Life of German Desmoid Patients: Results from the PROSa-DES and PROSa Study

TL;DR

German desmoid tumor patients experience significant quality of life issues, especially in emotional and social areas, highlighting the need for long-term care.

Contribution

This study provides the first comprehensive assessment of health-related quality of life in German desmoid tumor patients using both general and disease-specific questionnaires.

Findings

Desmoid patients report significant impairments in emotional, social, and role functioning compared to the general population.

Female gender, unemployment, and intensive treatments are linked to worse quality of life outcomes.

German desmoid patients have worse quality of life than Dutch/UK cohorts but similar to Indian cohorts.

Abstract

Desmoid-type fibromatosis is a rare soft tissue tumor that does not metastasize but can grow aggressively and cause substantial long-term symptoms. Many patients experience pain, fatigue, and psychological distress that affect daily life, work participation, and social relationships. However, data on the quality of life of desmoid patients in Germany are limited. This study evaluates health-related quality of life in a national cohort of German desmoid patients using both a general cancer quality-of-life questionnaire and a disease-specific instrument. The results show persistent impairments, particularly in emotional, social, and role functioning. Women, unemployed patients, and those who received intensive or multimodal treatments reported the greatest burden. These findings suggest that desmoid-type fibromatosis should be regarded as a chronic condition requiring long-term multidisciplinary care that integrates medical treatment with psychosocial and supportive interventions.

Desmoid-type fibromatosis (DT) is a rare, locally aggressive soft tissue tumor with a frequently chronic course and substantial impact on health-related quality of life (HRQoL). While international studies have demonstrated considerable symptom burden and psychosocial impairment, data from Germany are lacking. This study aimed to assess HRQoL in German patients and to identify factors associated with HRQoL. In this cross-sectional analysis, adult patients with histologically confirmed DT completed the EORTC QLQ-C30 and the disease-specific Desmoid-Type Fibromatosis Quality of Life Questionnaire (DTF-QoL). HRQoL scores were compared with reference data from the German general population, German sarcoma patients, and international DT cohorts. Sociodemographic, disease-, tumor-, and treatment-related factors associated with HRQoL were examined using multivariate linear regression analyses. A total of 155 patients were included (69.7% female; mean age 45.0 years). Compared with the German general population, DT patients reported clinically relevant impairments in role, social, and emotional functioning and higher symptom burden, particularly pain, fatigue, and insomnia. Compared with sarcoma patients, DT patients showed better physical, role, and social functioning, while emotional functioning and symptom burden were largely comparable. German DT patients reported consistently worse HRQoL than Dutch/UK cohorts and moderately worse outcomes than Indian cohorts. Female gender, unemployment or disability pension, intensive multimodal treatment, multiple lines of systemic therapy, and tumor location in the lower extremities were independently associated with poorer HRQoL. DT is associated with sustained and clinically meaningful HRQoL impairment. HRQoL is driven primarily by psychosocial and treatment-related factors rather than disease duration, supporting the concept of DT as a chronic condition requiring long-term, multidisciplinary supportive care.

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