Background: Bone and soft-tissue tumor patients experience long-lasting physical and psychological challenges. It is unknown to what extent Health-Related Quality of Life (HRQoL) is already... Show moreBackground: Bone and soft-tissue tumor patients experience long-lasting physical and psychological challenges. It is unknown to what extent Health-Related Quality of Life (HRQoL) is already affected during the diagnostic process. This study assesses the HRQoL of bone and soft-tissue tumor patients around time of diagnosis and explores which patient or tumor characteristics are associated with a reduced HRQoL. Methods: All patients with a suspected benign/malignant bone tumor (BT), benign soft-tissue tumor (STT), or malignant soft-tissue sarcoma (STS) visiting the Leiden University Medical Center between 2016 and 2020 were invited to complete the Patient-Reported Outcomes Measurement Information System (PROMIS) 29-item profile questionnaire. Mean scores of all included patients and per diagnosis group were compared to mean scores of the general population using one-sample t-tests. Results: Overall, patients (n = 637) reported statistically significantly worse HRQoL-scores on anxiety (51.3 ± 9.6), pain (55.3 ± 10.1), physical functioning (46.0 ± 9.7), and social functioning (48.1 ± 10.8) with the difference in pain and physical functioning being clinically relevant (based on a 3-point difference on t-metric). HRQoL-scores differed between diagnosis subgroups, i.e., patients with malignant tumors had higher anxiety levels and experienced more pain, where patients with bone tumors had worse physical functioning. Conclusion: The HRQoL of patients with suspected bone and soft-tissue tumors is already affected during the diagnostic process. Show less
Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade... Show moreEpithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication. Show less
