Qualitative study to characterize patient experience and relevance of patient-reported outcome measures for patients with metastatic synovial sarcoma

Abstract

Background The outlook for patients with metastatic synovial sarcoma (mSS) is poor. Better understanding of patient experience in this setting, beyond clinical measures, may guide improvements in management. Validated patient-reported outcome (PRO) instruments specific to many types of cancer exist, but for rare cancers this is often not the case. Methods This study aimed to characterize patient experiences of symptoms and impacts of mSS and evaluate the content validity and relevance of the novel European Organization for Research and Treatment of Cancer Item Library 31 (EORTC IL31) Disease Symptoms PRO tool assessing synovial sarcoma symptoms. This tool comprises items from preexisting, validated cancer-specific PRO instruments from the EORTC Item Library. It was developed as an mSS-specific add-on to the EORTC Quality of Life Questionnaire Core 30 (QLQ-C30), which evaluates general cancer and treatment-related symptoms and functioning. This was a non-interventional, qualitative interview study involving semi-structured, concept elicitation (CE) and cognitive debriefing (CD) telephone interviews in adults with mSS. CE explored symptoms and their impact on functioning and quality of life; CD assessed participant understanding and relevance of the PRO tools. Results Among the 8 participants, the most common disease-related symptoms reported during CE were fatigue and pain, while shortness of breath was one of the most bothersome. The greatest negative impacts of mSS occurred in domains of physical functioning and sleep. Key treatment priorities for patients were to improve disrupted sleep and ability to undertake strenuous activities. Conclusions The interviews showed that, when used together, the EORTC IL31 and EORTC QLQ-C30 covered symptoms and impacts of most relevance and importance to patients with mSS, with no notable gaps and good conceptual coverage. This study therefore supports the content validity of 2 tools in mSS, advocating their use in clinical trials to assess treatment impact on PRO measures of importance to these patients.

Ambivalent and Conjectural-Synovial Sarcoma

“…Characteristic morphological features emerge as monotonous spindle-shaped cells permeated with vesicular, plump and overlapping nuclei. Cellular component is intermingled with haemangiopericytoma-like vascular articulations [1,2].…”

“…Around ~18% instances occur within children and adolescents. A mild male predominance is observed with male to female proportion of ~1.2:1 [1,2]. Synovial sarcoma preponderantly incriminates extremities, trunk, head and neck, intrathoracic or intra-abdominal region or may arise within diverse locations although no site of tumour emergence is exempt.…”

“…Soft tissue sarcomas depicting chromosomal translocation t (X;18; p11; q11) are contemplated to be synovial sarcomas. Synovial sarcoma is posited to occur due to chromosomal translocation t(X;18) (p11; q11) incriminating genes SS18 along with SSX1, SSX2 or SSX4 [1,2]. Majority (~90%) of synovial sarcomas manifest characteristic chromosomal translocation t(X;18) (p11.2; q11) along with SYT-SSX1 genetic fusion.…”

“…Majority (~90%) of synovial sarcomas manifest characteristic chromosomal translocation t(X;18) (p11.2; q11) along with SYT-SSX1 genetic fusion. Besides, chromosomal translocation t(X;18) (p11.21; q11) and SYT-SSX2 genetic fusion may occur [1,2]. Synovial sarcoma frequently depicts p16INK4A genetic deletion.…”

“…Synovial sarcoma frequently depicts p16INK4A genetic deletion. Enhanced expression of EZH2 differentiates poorly differentiated neoplasms from monophasic or biphasic synovial sarcoma [1,2]. Aforesaid chromosomal translocation variably influences diverse oncogenetic pathways as initiation of SWI/SNF chromatin remodelling complex, polycomb repressor complex or canonical Wnt pathway.…”