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. 2022 Jan 29;14(3):709.
doi: 10.3390/cancers14030709.

Development of a Disease-Specific Health-Related Quality of Life Questionnaire (DTF-QoL) for Patients with Desmoid-Type Fibromatosis

Anne-Rose W Schut  1   2 , Emma Lidington  3 , Milea J M Timbergen  1   2 , Eugenie Younger  3 , Winette T A van der Graaf  1   4 , Winan J van Houdt  5 , Johannes J Bonenkamp  6 , Robin L Jones  3   7 , Dirk J Grünhagen  2 , Stefan Sleijfer  1 , Cornelis Verhoef  2 , Spyridon Gennatas  3   8 , Olga Husson  2   4   7
Affiliations

Development of a Disease-Specific Health-Related Quality of Life Questionnaire (DTF-QoL) for Patients with Desmoid-Type Fibromatosis

Anne-Rose W Schut et al. Cancers (Basel). .

Abstract

Sporadic desmoid-type fibromatosis (DTF) is a rare, non-metastasising soft-tissue tumour. Patients can experience a variety of disease-specific issues related to the unpredictable clinical course and aggressiveness of DTF, which negatively impacts health-related quality of life (HRQoL). These DTF-specific issues are not captured by generic HRQoL tools. A 102-item provisional DTF-specific HRQoL tool, the DTF-QoL, was previously developed. The aim of this study was to pre-test the psychometric properties of the DTF-QoL by administering it together with the EORTC Quality of Life Questionnaire Core 30 (EORTC QLQ-C30) to 236 DTF patients from the United Kingdom and the Netherlands. Construct validity and reliability were determined based on factor analysis, multi-trait scaling analysis, Cronbach's alpha, and correlations with the EORTC QLQ-C30 scales. Ninety-six items were selected, conceptualised into three symptom scales, eleven disease-impact scales and six single items, together forming the final DTF-QoL. Scaling assumptions were fully or moderately met for ten out of fourteen scales. Cronbach's alpha ranged from 0.551-0.908. Most scales of the DTF-QoL were weakly or moderately correlated with the EORTC QLQ-C30. The DTF-QoL is a promising tool capturing the whole spectrum of DTF-specific issues. Implementation of the DTF-QoL in research and clinical practice will help to personalise HRQoL measurement and clinical care for DTF patients.

Keywords: desmoid-type fibromatosis; disease-specific measures; health-related quality of life; patient-reported outcomes; rare diseases.

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Conflict of interest statement

R.L.J. has received research grants from MSD and GSK and is a consultant for Adaptimmune, Athenex, Bayer, Boehringer Ingelheim, Blueprint, Clinigen, Eisai, Epizyme, Daichii, Decipheara, Immunedesign, Lilly, Merck, Pharmacar, Springworks, Tracon and UptoDate. W.T.A.v.d.G. has received research grants from Novartis and Lilly, and advisory board fees from Bayer, and is consultant to Springworks and GSK. All fees to the institute. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

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