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,Stefan Sleijfer,Olga Husson,Winette T Van Der Graaf,Cornelis Verhoef,Dirk J Grünhagen

doi:10.1007/s11136-018-1931-3

Abstract

PurposeSporadic 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.MethodsA 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.ResultsThe 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.ConclusionA 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.

Highlights

MethodsDesmoid-type fibromatosis (DTF) is a soft tissue tumour that arises from musculoaponeurotic structures
No desmoid-type fibromatosis (DTF)-specific questionnaires were identified in this literature review (Table 1)
The MD Anderson Symptom Inventory (MDASI) measures the severity of 13 cancer-related symptoms experienced by the patient during the previous 24 h

Summary

Introduction

Desmoid-type fibromatosis (DTF) is a soft tissue tumour that arises from musculoaponeurotic structures. It is incapable of metastasising and is often described as a benign tumour in clinical practice. Due to its local aggressive behaviour and its known tendency of local recurrence after initial surgical resection, it is categorised as a borderline tumour [1]. Desmoid-type fibromatosis is rare, with a reported incidence of 5.4 new cases per million persons per year in the Dutch population [2]. Two types can be distinguished: sporadic DTF with extra-abdominal or abdominal wall tumour formation and familial adenomatous polyposis (FAP)-related DTF with intra-abdominal tumour formation [3, 4]

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