Abstract
Simple SummaryDesmoid-type fibromatosis (DTF) is an uncommon soft-tissue tumour that is incapable of metastasising. Patients can experience a variety of physical and psychological symptoms, making assessment of health-related quality of life (HRQoL) highly relevant. Measuring the impact of DTF on HRQoL can be challenging due to the rare character and variable clinical presentation of the disease. Therefore, a HRQoL instrument assessing DTF-specific issues is needed. Previously, a provisional DTF-specific HRQoL tool was developed (the DTF-QoL). The aim of the current study was to pre-test the DTF-QoL. Pre-testing of the questionnaire led to the selection of 96 questions, conceptualised into three symptom subscales, eleven disease-impact subscales and six single items, together forming the final DTF-QoL. This questionnaire can be used in both clinical and research settings to evaluate HRQoL of DTF patients, which could help to provide personalised care and improve overall patient experience.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.
Highlights
Desmoid-type fibromatosis (DTF) is a rare soft-tissue tumour [1]
DTF tumours do occur in the context of familial adenomatous polyposis (FAP), but the majority of DTF tumours are sporadic and characterised by mutations in the β-catenin (CTNNB1) gene [4]
To develop a disease-specific tool for DTF patients that can be used to supplement the EORTC-QLQ-C30, we have developed a DTF-specific health-related quality of life (HRQoL) questionnaire according to the guidelines of the EORTC Quality of Life Group [19]
Summary
Desmoid-type fibromatosis (DTF) is a rare soft-tissue tumour [1]. DTF does not metastasise, its potential locally aggressive tumour growth can cause significant morbidity and it is categorised as an intermediate tumour [1]. DTF tumours do occur in the context of familial adenomatous polyposis (FAP), but the majority of DTF tumours are sporadic and characterised by mutations in the β-catenin (CTNNB1) gene [4]. Sporadic DTF can arise in any part of the body, most commonly in the extremities and the abdominal wall [5]. The biological behaviour of DTF is unpredictable and variable, including phases of progressive growth, growth stabilisation, and spontaneous regression [6,7]
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