Marije E Weidema1, Olga Husson2,3, Winette T A van der Graaf1,4, Hugh Leonard5, Belle H de Rooij6,7, Lisa Hartle DeYoung8, Ingrid M E Desar1, Lonneke V van de Poll-Franse2,6,7. 1. Department of Medical Oncology, Radboud University Medical Center, Nijmegen, The Netherlands. 2. Division of Psychosocial Research and Epidemiology, Netherlands Cancer Institute, Amsterdam, The Netherlands. 3. Division of Clinical Studies, Institute of Cancer Research/Royal Marsden Hospital, London, UK. 4. Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. 5. EHE Rare Cancer Charity, Surrey, UK. 6. Netherlands Comprehensive Cancer Organization, Utrecht, The Netherlands. 7. CoRPS - Center of Research on Psychology in Somatic diseases/Department of Medical and Clinical Psychology, Tilburg University, Tilburg, The Netherlands. 8. EHE Foundation, Anchorage, Alaska, USA.
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.
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 cancerpatients, 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 cancerpatients 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.
Authors: Anna M Frezza; Vinod Ravi; Salvatore Lo Vullo; Bruno Vincenzi; Francesco Tolomeo; Tom Wei-Wu Chen; Pawel Teterycz; Giacomo G Baldi; Antoine Italiano; Nicolas Penel; Antonella Brunello; Florance Duffaud; Nadia Hindi; Shintaro Iwata; Alannah Smrke; Alexander Fedenko; Hans Gelderblom; Winette Van Der Graaf; Aurore Vozy; Elizabeth Connolly; Massimiliano Grassi; Robert S Benjamin; Javier-Martin Broto; Giovanni Grignani; Robin L Jones; Akira Kawai; Andrzej Tysarowski; Luigi Mariani; Paolo G Casali; Silvia Stacchiotti Journal: Cancer Med Date: 2021-03-13 Impact factor: 4.452
Authors: Lonneke V van de Poll-Franse; Nicole Horevoorts; Dounya Schoormans; Sandra Beijer; Nicole P M Ezendam; Olga Husson; Simone Oerlemans; Sanne B Schagen; Geja J Hageman; Katrijn Van Deun; Corina van den Hurk; Mies van Eenbergen; Floortje Mols Journal: J Natl Cancer Inst Date: 2022-06-13 Impact factor: 11.816
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