Health-related quality of life (HRQoL) challenges in patients with epithelioid hemangioendothelioma (EHE): analysis of an ultra-rare cancer

Background: Patients diagnosed with epithelioid hemangioendothelioma (EHE) face diverse, sometimes challenging issues stemming from the ultra-rare nature of their disease.EHE exhibits clinical heterogeneity; while some individuals experience a slow, asymptomatic course, others endure a rapidly progressive form.The impact of these clinical variations-compounded by the scarcity of information surrounding EHE-on patients' health-related quality of life (HRQoL) remains largely unexplored.This study examined EHE patients' HRQoL.Material and methods: Thirty-one patients with EHE from 11 countries completed one HRQoL questionnaire (European Organisation of Research and Treatment of Cancer [EORTC QLQ-C30]) and were interviewed.Descriptive analysis of the questionnaire data was carried out.Interview thematic analysis was conducted using the biopsychosocial model to identify themes. Results:The EORTC QLQ-C30 showed no floor or ceiling effects.The highest mean scores were for pain [3.29 (standard deviation 0.973)] or pain interfering with daily activities [3.29 (standard deviation 1.039)].Interviews identified 25 themes across three domains: physical (e.g.fatigue, pain, functional limitations), mental (e.g.fear of recurrence, scanxiety, emotional distress), and social health (e.g.loss of independence, financial burden, emotional impact).Conclusions: Patients face a multifaceted burden of living with EHE, from uncertainty, isolation, and lack of understanding, to physical complaints such as fatigue and pain.Further research needs to explore different disease courses and how it affects patients' HRQoL.Beyond development of personalised supportive care, results will help with creating an item list to complement the EORTC QLQ-C30 questionnaire for trials among EHE patients.