Lived experience of people with adrenocortical carcinoma and associated adrenal insufficiency

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare cancer with an annual incidence of 0.7–2 cases per million population and 5‐year survival of 31.2%. Adrenal insufficiency (AI) is a common and life shortening complication of ACC, and little is understood about how it impacts on patients' experience. OBJECTIVE: To understand patients' lived experience of the condition, its treatment, care process, impact of AI on ACC wellbeing, self‐care needs and support. METHODS: Systematic review of MEDLINE, EMBASES, CINAHL, PsycINFO and Open Grey for studies published until February 2021. All research designs were included. The findings underwent a thematic analysis and narrative synthesis. Studies quality was assessed using mixed method assessment tools. RESULTS: A total of 2837 citations were identified; 15 titles with cohort, cross‐sectional, case series and case report study designs met the inclusion criteria involving 479 participants with adrenal insufficiency secondary to adrenocortical carcinoma (AI/ACC). Quantitative research identified impacts of disease and treatment on survivorship, the burden of living with AI/ACC, toxicity of therapies, supporting self‐care and AI management. These impact factors included adjuvant therapies involved and their toxicities, caregivers/family supports, healthcare and structure support in place, specialist skill and knowledge provided by healthcare professional on ACC management. No qualitative patient experiences evidence was identified. CONCLUSION: ACC appears to have high impact on patients' wellbeing including the challenges with self‐care and managing AI. Evidence is needed to understand patient experience from a qualitative perspective.