Health-Related Quality of Life in Adrenocortical Carcinoma: Development of the Disease-Specific Questionnaire ACC-QOL and Results from the PROFILES Registry.

Rachel S Van Leeuwaarde,Els J M Nieveen Van Dijkum,Koen M A Dreijerink,Marjolein N T Kremers,Michiel N Kerstens,Ellen Kapiteijn,Floortje Mols,Richard A Feelders,Myrte Zijlstra,Thomas M A Kerkhofs,Henry J L M Timmers,Elisabeth M W Eekhoff,Eleonora P M Corssmit,Rebecca V Steenaard,Harm R Haak

doi:10.3390/cancers14061366

Abstract

We aimed to develop a disease-specific adrenocortical carcinoma (ACC) health-related quality of life (HRQoL) questionnaire (ACC-QOL) and assess HRQoL in a population-based cohort of patients with ACC. Development was in line with European Organization for Research and Treatment of Cancer (EORTC) guidelines, though not an EORTC product. In phase I and II, we identified 90 potential HRQoL issues using literature and focus groups, which were reduced to 39 by healthcare professionals. Pilot testing resulted in 28 questions, to be used alongside the EORTC QLQ-C30. In Phase III, 100 patients with ACC were asked to complete the questionnaires twice in the PROFILES registry (3-month interval, respondents: first 67, second 51). Confirmatory factor analysis demonstrated the structural validity of 26 questions with their scale structure (mitotane side-effects, hypercortisolism/hydrocortisone effects, emotional effects). Internal consistency and reliability were good (Cronbach's alpha 0.897, Interclass correlation coefficient 0.860). Responsiveness analysis showed good discriminative ability (AUC 0.788). Patients diagnosed more than 5 years ago reported a good HRQoL compared with the Dutch reference population, but experienced residual fatigue and emotional problems. Patients who underwent recent treatment reported a lower HRQoL and problems in several domains. In conclusion, we developed an ACC-specific HRQoL questionnaire with good psychometric properties.

Highlights

Adrenocortical carcinoma (ACC) affects approximately one in a million persons each year [1]
This study aimed to develop and provide initial validation of a disease-specific health-related quality of life (HRQoL) questionnaire to be used alongside the EORTC QLQ-C30 in patients with adrenocortical carcinoma (ACC) (ACC-QOL)
We found no differences between respondents, non-respondents, and excluded patients, except for a high proportion of mitotane users among the respondents (Table 1)

Summary

Introduction

Adrenocortical carcinoma (ACC) affects approximately one in a million persons each year [1]. Treatment options for ACC include surgery, mitotane therapy, chemotherapy and radiotherapy [2,3]. Each of these treatment options and the disease itself can have a negative impact on patients’ health-related quality of life (HRQoL). The EORTC QLQ-C30 is a well-known questionnaire designed for use among patients with cancer [4]. Its items include general effects of cancer on HRQoL such as general physical and emotional functioning, and symptoms such as fatigue. The EORTC developed disease-specific questionnaires that can be used in addition to the EORTC QLQ-C30 questionnaire, for example, in breast cancer [5]. No disease-specific questionnaire has yet been developed for ACC and apart from questionnaires addressing hormone overproduction, there is no HRQoL questionnaire including items specific to ACC-related problems [6]

Objectives

Methods

Results

Conclusion