Abstract
Purpose Desmoid-type fibromatosis (DTF) is a rare, nonmetastasising soft tissue tumour. Symptoms, unpredictable growth, lack of definitive treatments, and the chronic character of the disease can significantly impact health-related quality of life (HRQoL). We aimed at identifying the most important HRQoL issues according to DTF patients in two countries, in order to devise a specific HRQoL questionnaire for this patient group. Methods DTF patients and healthcare providers (HCPs) from the Netherlands and the United Kingdom individually ranked 124 issues regarding diagnosis, treatment, follow-up, recurrence, living with DTF, healthcare, and supportive care experiences, according to their relevance. Descriptive statistics were used to calculate priority scores. Results The most highly ranked issues by patients (n = 29) were issues concerning “tumour growth,” “feeling that there is something in the body that does not belong there,” and “fear of tumour growth into adjacent tissues or organs” with mean (M) scores of 3.0, 2.9, and 2.8, respectively (Likert scale 1–4). British patients scored higher on most issues compared to Dutch patients (M 2.2 vs. M 1.5). HCPs (n = 31) gave higher scores on most issues compared to patients (M 2.3 vs. M 1.8). Conclusion This study identified the most relevant issues for DTF patients, which should be included in a DTF-specific HRQoL questionnaire. Additionally, we identified differences in priority scores between British and Dutch participating patients. Field testing in a large, international cohort is needed to confirm these findings and to devise a comprehensive and specific HRQoL questionnaire for DTF patients.
Highlights
Sporadic desmoid-type fibromatosis (DTF) is a rare, borderline tumour of the soft tissues [1,2,3]
Sporadic DTF arises in musculoaponeurotic structures with the most common sites being the abdominal wall and the extremities [4]
Issues that had previously been identified to be of concern to DTF patients were listed per country (UK and NL)
Summary
Sporadic desmoid-type fibromatosis (DTF) is a rare, borderline tumour of the soft tissues [1,2,3]. “active” forms of treatment can be debilitating, causing greater morbidity than the tumour itself For these reasons, active surveillance is recommended as a first-line management for most patients with DTF [6, 9]. Generic HRQoL measures (e.g., the cancer-specific core questionnaire from European Organisation for Research and Treatment of Cancer; the EORTC Quality of Life Core Questionnaire (EORTC QLQ-C30)) may not consider disease-specific issues in DTF patients. In order to gain greater insight into the issues that patients with DTF experience in their daily lives, and to evaluate their experiences of healthcare including the supportive care system, we previously organised focus groups and semistructured interviews, in the United Kingdom (UK) and in the Netherlands (NL) [12, 13]. We used the previously identified issues to (1) identify the most relevant issues to patients with DTF in two healthcare settings (UK and NL) and to (2) identify differences in scores between both countries
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