Milea Timbergen

220 Abstract Background Sporadic desmoid-type fibromatosis (DTF) is a rare, chronic, non-metastasizing, disease of the soft tissues. It is characterised by local invasive and unpredictable growth behaviour and a high propensity of local recurrence after surgery thereby often having a great impact on health related quality of life (HRQoL). This study aims to review currently used HRQoL- measures and to asses HRQoL-issues among DTF patients. Materials and methods A mixed methods methodology was used consisting of (1) a systematic literature review, according to the PRISMA guidelines (2009), using search terms related to sporadic DTF and HRQoL in commonly used databases (e.g., Embase, Medline Ovid, Web of science, Cochrane Central, Psyc Info, and Google scholar), to provide an overview of measures previously used to evaluate HRQoL among DTF patients; (2) focus groups to gain insight into HRQoL issues experienced by DTF patients. Results The search strategy identified thirteen articles reporting HRQoL-measures using a wide variety of cancer-specific HRQL tools, functional scores, symptom scales (e.g., NRS), and single-item outcomes (e.g., pain and functional impairment). No DTF specific HRQoL-tool was found. Qualitative analysis of three focus groups (6 males, 9 females) showed that participants emphasised the negative impact of DTF and/or its treatment on several HRQoL- domains. Six themes were identified: 1) diagnosis, 2) treatment, 3) follow-up and recurrence, 4) physical domain, 5) psychological and emotional domain and 6) social domain. Conclusions A DTF-specific HRQoL-tool and consensus regarding the preferred measurement tool among DTF patients is lacking. Our study indicates that HRQoL of DTF patients was negatively affected in several domains. A DTF-specific HRQoL-measure could improve our understanding of short- and long-term effects and, ideally, can be used in both clinic and for research purposes. 8