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

Abstract

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.

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

“…EHE patients do not only face the challenges of living with an ultrarare cancer, such as difficulties finding expert healthcare professionals and lack of evidence with regard to treatment strategies, but also face a highly unpredictable disease course. Our previous findings stress the importance of tailoring the health care offered to highly symptomatic EHE patients (Weidema et al, 2020). In order to be able to provide EHE patients with adequate supportive care, we aimed to investigate the (1) level of supportive care needs of EHE patients; (2) association of the level of supportive care needs with sociodemographic and clinical characteristics; (3) association of the level of supportive care needs with symptom level; and (4) top 10 unmet needs for highly symptomatic patients.…”

“…This cross-sectional questionnaire study was initiated upon request of the international EHE patient community and was previously described (Weidema et al, 2020). In short, EHE patients (age ≥18 years) were invited to participate through posts in the Facebook group by one of the researchers (MW).…”

“…Although reported five-year relative survival of all EHE patients is about 70% (Lau et al, 2011;Shiba et al, 2018), a recent study showed that patients with pleural disease (<20%) or lymph node metastases (~30%) had a more aggressive clinical course with poor overall five-year survival rates (Rosenbaum et al, 2020). We previously demonstrated that about one third of EHE patients have a high symptom burden and that these patients mostly suffer from pain and fatigue (Weidema et al, 2020). Moreover, EHE patients with a high symptom burden experienced significantly lower functioning in daily life and reduced HRQoL.…”

Health-related Quality of Life and Health Literacy in Patients With Systemic Mastocytosis and Mast Cell Activation Syndrome

“…In systemic mastocytosis and MCAS, both classi ed as hematologic neoplasms, fatigue, pain and insomnia are among the most frequent or predominant complaints, similar to a variety of different cancers such as ovarian cancer, epithelioid hemangioendothelioma or neuroendocrine tumors. [33][34][35] In our fourth Hypothesis we assumed that fatigue as a particular debilitating symptom is signi cantly higher in SM compared to MCAS. However, the comparison of the two mast cell diseases showed signi cant differences in the psychosocial domain in the form of fatigue and limitations in daily life and work (role function), with a more prominent expression in the MCAS group.…”

Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature

“…A rapidly evolving third variant does exist, which behaves as a high‐grade sarcoma. Similarly, the symptoms burden in this disease can be variable, but overall, a significant impact on quality of life and psychological distress have been recently reported in EHE patients 3 …”