Background Systemic treatments for angiosarcoma remains an area of unmet clinical need. The authors conducted this retrospective study to assess the clinical activity of checkpoint inhibitors in patients with angiosarcoma. The primary objective was to assess the objective response rate, and the secondary objective was to assess the progression‐free and overall survival durations and disease control rate. Methods Patient data were obtained using The University of Texas MD Anderson Cancer Center Tumor Registry database. The final study population was refined to only include patients who had undergone pembrolizumab monotherapy. The objective response rate was evaluated using RECIST/irRECIST version 1.1. Progression‐free survival and overall survival were defined as the time from the initiation of immunotherapy to disease progression or recurrence, death, or last follow‐up and to death or last follow‐up, respectively. Results The final cohort comprised 25 patients. Most patients had metastatic disease (72%) and had undergone at least two lines of systemic therapy (80%) before starting pembrolizumab. The objective response rate was 18%, whereas the disease control rate was 59%. The median progression‐free survival duration was 6.2 months and was not significantly different between the cutaneous (4.7 months) and visceral angiosarcoma (6.2 months) groups (p = .42). The median overall survival duration was 72.6 months. Toxicities were recorded for eight patients, with fatigue, anemia, constipation, and rash being the most common. Conclusions Pembrolizumab shows durable clinical activity in angiosarcoma. These findings suggest that checkpoint inhibition as monotherapy or combination therapy is likely to have a high probability of success.© 2022 American Cancer Society. Lay summary This is the largest retrospective study to assess the clinical activity of checkpoint inhibitor monotherapy in angiosarcomas. The study includes an adequate number of patients with visceral angiosarcoma that enabled to obtain meaningful clinical insights that were previously unavailable. Our findings indicate an improvement in progression‐free survival with pembrolizumab that is comparable to other active agents in angiosarcoma. Pembrolizumab monotherapy in angiosarcomas also has a favorable tolerability profile. Our findings emphasize the need for prospective studies to evaluate the activity of pembrolizumab monotherapy and combination therapy.
e23527 Background: EHE is a rare soft tissue tumor of endothelial origin. It is distinguished by the pathognomonic WWTR1-CAMTA1 fusion (WWTR1 is the gene symbol for TAZ) seen in 90% of the tumors. YAP1-TFE3 fusion is less common and seen in 10% of the tumors. YAP and TAZ are critical downstream effectors of the Hippo pathway that regulate tumor development, progression, invasion and metastasis by modulating the expression of many Hippo pathway targets. Recent studies have shown that inhibition of HMG-CoA reductase, a key enzyme of the mevalonate pathway, can regulate YAP/ TAZ by preventing their nuclear accumulation and inhibiting their transcriptional activity. This has led to interest in the role of statins, which inhibit HMG-CoA reductase, as a modulator of YAP/ TAZ that could benefit patients with sarcoma, particularly EHE. Methods: A retrospective analysis was performed on patients with a diagnosis of EHE at M D Anderson Cancer Center. Patients were identified using the electronic database system and screened for statin use using EMRs. Demographic and clinical characteristics were tabulated. KM method was used to assess overall survival and log rank test was used to test survival differences between the statin use and non- statin use groups. All statistical analysis was performed using STATA 14. Results: 226 patients with EHE were identified. 27 of them had recorded statin use during the course of their disease. The median OS for the statin use group was not reached and the mean OS was 221 months. The median OS for the non- statin use group was 123.9 months, while the mean OS was 160 months. The difference in OS was not statistically significant between the two groups. The median follow-up time for our cohort was 36.6 months. Conclusions: Our findings indicate a trend towards improved survival for patients with EHE who have received statins over the course of their disease. Our study is limited by a small number of patients who received statins. Prospective studies are required to assess the therapeutic benefit of statins in EHE. [Table: see text]
e18095 Background: Utilizing AI and ML is an emerging method to improve risk identification, characterization and stratification for clinical outcomes such as HAPIs. The Jvion Cognitive Clinical Success Machine (CCSM) utilizes the Eigen Sphere technique to factor in clinical, socioeconomic, and behavioral covariates at the individual patient level to maximize accuracy of risk prediction and provide insights on prevention of HAPIs. Methods: A retrospective analysis was performed utilizing claims and EHR data on 63,476 inpatient admissions between June 2016 and June 2018 at M D Anderson Cancer Center, a 660-bed oncology facility (Table). All risk assessment indicators in the data were removed prior to analysis by the CCSM to compute unbiased risk probabilities for HAPIs. The performance of the CCSM risk prediction for all new stage 2 and above HAPIs ( > = Stage 2 HAPIs) was compared with the Braden scale using AUC. Results: The Jvion CCSM had an AUC of 0.84 compared to the AUC of 0.72 for the Braden scale in the prediction of > = Stage 2 HAPIs. Conclusions: The AUC value indicates that the Jvion CCSM has better predictive accuracy than the Braden scale. It also has better discrimination in risk identification. Thus, Jvion CCSM can be a valuable tool in risk screening for HAPIs which can lead to early preventative interventions. [Table: see text]
Introduction We investigated outcomes and prognostic factors for patients treated for cutaneous angiosarcoma (CA). Methods We conducted a retrospective review of patients treated for CA of the face and scalp from 1962 to 2019. All received definitive treatment with surgery, radiation (RT), or a combination (S‐XRT). The Kaplan–Meier method was used to estimate outcomes. Multivariable analyses were conducted using the Cox proportional hazards model. Results For the 143 patients evaluated median follow‐up was 33 months. Five‐year LC was 51% and worse in patients with tumors >5 cm, multifocal tumors, those treated pre‐2000, and with single modality therapy (SMT). These remained associated with worse LC on multivariable analysis. The 5‐year disease‐specific survival (DSS) for the cohort was 56%. Tumor size >5 cm, non‐scalp primary site, treatment pre‐2000, and SMT were associated with worse DSS. Conclusion Large or multifocal tumors are negative prognostic factors in patients with head and neck CA. S‐XRT improved outcomes.
Purpose: We evaluated a cohort of patients with cardiac angiosarcomas (CA) who developed brain metastases (BM) to define outcomes and intracranial hemorrhage (IH) risk. Methods: We reviewed 26 consecutive patients with BM treated between 1988 and 2020 identified from a departmental CA (n=103) database. Causes of death were recorded, and a terminal hemorrhage (TH) was defined as an IH that caused death or prompted a transfer to hospice. Results: The prevalence of BM was 25% (n=26/103). A total of 23 patients (88%) had IH, including 21 (81%) at initial BM diagnosis, of which 18 (86%) required hospitalization. The median platelet count at the time of IH was 235k (interquartile range, 108 to 338k). Nearly all patients died of disease (n=23, 88%) and most patients died from TH (n=13, 57%). TH occurred at BM presentation in 6 (46%) patients, whereas 3 (23%) had TH from known but untreated lesions, 2 (15%) had continued uncontrolled IH during radiation therapy, and 2 (15%) from new BM. Platelet count <50k was not associated with TH (P=0.25). Subsequent IH occurred in 9 patients (35%), and importantly, no patients who completed radiation therapy (n=10) for BM died from TH. Conclusion: Patients with CA frequently develop BM, and the risk of IH is high, resulting in an alarming rate of TH despite normal platelet counts. Therefore, early diagnosis and intervention are warranted. We recommend surveillance brain imaging, and importantly, once BM is detected, prompt local therapy is warranted to try and mitigate the risk of TH.
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