Supportive care needs of patients living with an extremely rare and unpredictable cancer: The Epithelioid Haemangioendothelioma patient experience.

OBJECTIVE: Epithelioid haemangioendothelioma (EHE) is an ultrarare vascular sarcoma with an incidence of <1/million/year and a large clinical heterogeneity. Data on supportive care needs of rare cancer patients are scarce. This study aimed to investigate the level of supportive care needs of EHE patients and its association with sociodemographic, clinical and symptom burden characteristics. METHODS: We present secondary data of a cross-sectional questionnaire study involving EHE patients recruited from the international EHE Facebook group. Data were collected using the web-based PROFILES registry. Unmet needs were measured with Supportive Care Needs Survey Short Form (SCNS-SF34). RESULTS: 115 EHE patients from 20 countries completed the online questionnaire. Mean level of supportive care needs was 68.4 (range 34-170), with the highest mean score on the psychological domain. Supportive care needs were associated with age, disease stage, years since diagnosis and number of tumour locations. Highly symptomatic patients (33%) reported more supportive care needs than patients with low or intermediate symptom burden. CONCLUSION: Supportive care needs were found in all domains, highest in the psychological domain, and were associated with sociodemographic, clinical and symptom burden characteristics. Adequate and tailored supportive care should be offered especially to highly symptomatic EHE patients.

Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy.

Purpose: Epithelioid hemangioendothelioma (EHE) is an ultra-rare vascular sarcoma with unique clinical features. EHE is characterized by an unpredictable, often protracted, clinical course and highly variable clinical presentation. Due to difficulty recruiting ultra-rare cancer patients, health-related quality of life (HRQoL) of EHE patients has not yet been studied. The aim of this study was to assess EHE symptom burden and its impact on HRQoL and psychological distress.Methods: The study was initiated after EHE patients' foundations approached our research group to study HRQoL. Patients were recruited from the international EHE Facebook group from May through October 2018. Data were collected using the online PROFILES registry. Latent class cluster analysis was performed to identify groups based on frequently reported symptoms. Differences in HRQoL (EORTC-QLQ-C30) and psychological distress (Hospital Anxiety and Depression Scale) between symptom-based clusters were examined.Results: Among 115 EHE patients from 20 countries, three clusters were identified, with low-, intermediate- and high-symptom burden, respectively. Highly symptomatic patients (33%) had clinically relevantly lower scores on HRQoL compared to the other two groups (p < 0.001). These patients suffered mostly from pain, insomnia and fatigue. Symptom burden significantly correlated with reduced daily functioning and high levels of psychological distress. Only for highly symptomatic patients, HRQoL and symptom levels were worse compared to healthy individuals.Conclusion: For the first time, we studied HRQoL in a large international cohort of ultra-rare cancer patients with distinct clinical characteristics, enabled by collaboration with patients and use of social media. We showed a considerable number of EHE patients were highly symptomatic, with a significant impact on HRQoL and psychological distress.