Systemic treatments and outcomes in CIC-rearranged Sarcoma: A national multi-centre clinicopathological series and literature review.

CIC-rearranged sarcoma is a recently established, ultra-rare, molecularly defined sarcoma subtype. We aimed to further characterise clinical features of CIC-rearranged sarcomas and explore clinical management including systemic treatments and outcomes. METHODS: A multi-centre retrospective cohort study of patients diagnosed between 2014-2019. RESULTS: Eighteen patients were identified. The median age was 27 years (range 13-56), 10 patients were male (56%), 11 patients (61%) had localised disease and 7 patients had advanced (metastatic or unresectable) disease at diagnosis. Of 11 patients with localised disease at diagnosis, median overall survival (OS) was 40.6 months and the 1-, 2- and 5-year OS estimates were 82%, 64% and 34% respectively. Nine patients (82%) underwent surgery (all had R0 resections), 8 (73%) patients received radiotherapy to the primary site (median dose 57Gy in 28 fractions), and 8 (73%) patients received chemotherapy (predominantly Ewing-based regimens). Metastases developed in 55% with a median time to recurrence of 10.5 months. In patients with advanced disease at diagnosis, median OS was 12.6 months (95% CI 5.1-20.1), 1-year OS was 57%. Median progression-free survival was 5.8 months (95% CI 4.5-7.2). Durable systemic therapy responses occurred infrequently with a median duration of systemic treatment response of 2.1 months. One durable complete response of metastatic disease to VDC/IE chemotherapy was seen. Responses to pazopanib (n = 1) and pembrolizumab (n = 1) were not seen. CONCLUSION: In this series, CIC-rearranged sarcomas affected young adults and had a high incidence of presenting with, or developing, metastatic disease. The prognosis overall was poor. In advanced disease, durable systemic therapy responses were infrequent.

Predictors of distress and poorer quality of life in High Grade Glioma patients.

OBJECTIVE: To determine High Grade Glioma (HGG) patients' levels of distress and QOL during combined chemoradiotherapy, explore predictors of distress and QOL and prioritize patients' supportive care needs. METHODS: Patients diagnosed with HGG who were referred for combined chemoradiotherapy were recruited. Participants completed demographics and questionnaires assessing distress, function, and supportive care needs. Descriptive statistics, correlation coefficients, t-tests and linear and logistic regression analyses were performed. RESULTS: 116 participants completed the questionnaire. Participants scored lower for QOL in physical, functional and emotional domains than the general Australian population. Poor physical function, lower education levels, loss of employment and financial impact associated with diagnosis were consistently linked with multiple domains of distress, poor QOL and high unmet needs. Having a carer who was their partner predicted lower emotional well-being. CONCLUSION: Patients with HGG experience a poor QOL, increased levels of distress and high unmet needs when commencing chemoradiotherapy. Patients who experience a financial impact and those with lower education levels may report higher levels of distress and increased unmet needs. PRACTICE IMPLICATIONS: Poor function, lower education and limited financial resources may help identify those who require additional screening and may benefit from additional information and psychological support at this time.