Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: A literature review and focus group study

Milea J. M. Timbergen*, Lonneke V. van de Poll-Franse, Dirk J. Gruenhagen, Winette T. van der Graaf, Stefan Sleijfer, Cornelis Verhoef, Olga Husson

*Corresponding author for this work

Research output: Contribution to journalArticleScientificpeer-review

20 Citations (Scopus)
96 Downloads (Pure)

Abstract

Purpose

Sporadic desmoid-type fibromatosis (DTF) is a rare, chronic, non-metastasising, 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 (HRQL). This study aims to review currently used HRQL measures and to asses HRQL issues among DTF patients.

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 HRQL 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 HRQL among DTF patients; (2) focus groups to gain insight into HRQL issues experienced by DTF patients.

Results

The search strategy identified thirteen articles reporting HRQL 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 HRQL 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 HRQL 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.

Conclusion

A DTF-specific HRQL tool and consensus regarding the preferred measurement tool among DTF patients is lacking. Our study indicates that HRQL of DTF patients was negatively affected in several domains. A DTF-specific HRQL measure could improve our understanding of short- and long-term effects and, ideally, can be used in both clinic and for research purposes.

Original languageEnglish
Pages (from-to)3097-3111
JournalQuality of Life Research
Volume27
Issue number12
DOIs
Publication statusPublished - 2018

Keywords

  • Desmoid-type fibromatosis
  • Health-related quality of life
  • Focus group
  • Literature review
  • AGGRESSIVE FIBROMATOSIS
  • BETA-CATENIN
  • EUROPEAN-ORGANIZATION
  • SOFT-TISSUE
  • TUMORS
  • MANAGEMENT
  • BONE
  • SHOULDER
  • MUTATION
  • THERAPY

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